Do Enzymes Called Matrix Metalloproteinases Play a Role in GCA? - Alicia Rodriguez-Pla, M.D., Ph.D.

Phillip Seo, M.D., MHS

Assistant Professor of Medicine

The Johns Hopkins University School of Medicine

Division of Rheumatology

Co-Director, The Johns Hopkins Vasculitis Center

Vasculitis Clinical Research Consortium Co-Investigator

It is a great pleasure to introduce Dr. Rodriguez-Pla to the members of the VF community. Dr. Rodriguez-Pla was recruited to the Johns Hopkins Division of Rheumatology by Dr. John Stone, who was the Director of the Vasculitis Center at that time. Her research interests have focused on the etiopathogenesis of giant cell arteritis, which is the most common form of primary systemic vasculitis in the United States.                       

In collaboration with Wills Eye Hospital in Philadelphia, she has been given access to multiple preserved temporal artery specimens obtained from patients suspected of having giant cell arteritis. These specimens served as the basis of an early project looking for evidence of an infectious etiology for this disease.                       

Dr. Rodriguez-Pla’s more recent research has examined the role played by matrix metalloproteinases in the development of giant cell arteritis.  This work was presented at the American College of Rheumatology Annual Scientific Meeting in Washington, D.C. last year. Her future research efforts will undoubtedly do much to further our understanding of these complex and challenging diseases.

By Alicia Rodriguez-Pla, M.D., Ph.D., March 2007  

I grew up in the southeast of Spain, between Caudete, a small town in which I was born, and Alicante, a nearby Mediterranean tourist city. I am married and have a four-month-old baby son called Mark. My husband, José, is also a doctor who specializes in Preventive Medicine and Public Health. 

I spend as much of my free time as possible with my husband and playing with my son. I like travelling and meeting people from different cultures. I love reading all kind of books, especially novels, walking, swimming, going to the theater and getting together with friends.  Although it may sound weird, studying and learning are my main hobbies.

I realized I wanted to become a doctor in high school. During those years I started to develop a great interest in health related issues, primarily for helping people and for research, which I consider the basic tool for medical advances.   And last but not least, decreasing the negative impact of diseases in people. 

I received a scholarship which allowed me to attend medical school in a very prestigious private university called Universidad de Navarra, in Pamplona, a city in the north of Spain. After medical school I moved to Barcelona and enrolled in a fellowship program in Rheumatology. Once completed, I worked on my PhD thesis, where I studied several pathogenic and epidemiologic aspects of giant cell arteritis. It was shortly after defending my thesis in 2003 that I moved to the United States.

Very early in my career I became fascinated by vasculitis disorders. My first clinical rotation was in the Department of Cardiovascular Surgery in 1991, during my fourth year in  medical school. I remember the first patient I visited in the hospital was a woman affected by Takayasu’s arteritis who had also developed retroperitoneal fibrosis. Never before had I heard that blood vessels could become inflamed. The faculty in charge asked me to review the literature to find out if a similar association between Takayasu’s arteritis and retroperitoneal fibrosis had previously been reported. I found only one case published in a Japanese journal, which was impossible for me to get at the time. The faculty then asked me to write a short review about Takayasu’s arteritis as an exercise. I remember how my interest and curiosity progressively increased while reviewing the literature and learning about this vasculitis. I typed a thirteen-page manuscript, which I still keep, on a classical typewriter. It was because of the interest that this patient woke up in me that I decided to become a Rheumatologist.  

I did not come across any other patients with vasculitis until January 1995, when I saw a second patient with Takayasu’s arteritis during my first month as a fellow in Rheumatology. This was striking, taking into consideration that this type of vasculitis is quite rare in Spain. During the following months I saw patients with other vasculitic syndromes, including Wegener’s granulomatosis, Churg-Strauss Syndrome, polyarteritis nodosa, and giant cell arteritis among others.  I was amazed that a patient with giant cell arteritis, who had been completely blind for two days, recovered her vision right after starting prednisone treatment. Fortunately, she had been diagnosed and treated early.  

As a curiosity, my father was the third patient I saw with giant cell arteritis and I participated actively in his diagnosis. In May 1996, at the age of 53, he started complaining of intense headaches, fatigue, and pain and weakness in the girdles. He was initially diagnosed with depression, and treated for several months with antidepressant medication. He did not improve, and I suggested to his doctor the possibility of giant cell arteritis. Initially, he did not believe me because my father was too young to have this vasculitis, and I was too young to suggest anything. After almost six months, he finally decided to draw some blood, and the diagnosis then became quite obvious.   

I would like to point out that my interest comprises all kinds of vasculitic syndromes and all vasculitis patients. I chose giant cell arteritis for my Ph.D. thesis simply because it is the most frequent type of vasculitis worldwide including Spain.   I am convinced that the pathogenic mechanisms underlying the vascular and organ damage have certain similarities among the different vasculitic disorders, and that advances in knowledge of one of them could be quite easily extrapolated to others. 

My Ph.D. thesis included a project to study the potential role of different infectious agents in giant cell arteritis. At the American College of Rheumatology Annual Scientific Meeting in San Francisco in 2001, I saw that the group of physicians, including Dr. Stone from the Johns Hopkins Vasculitis Center were also interested in this field. Shortly after the meeting, I contacted Dr. Stone to ask about the possibility of joining his team to continue working on this research.  Dr. Stone offered me a two-year fellowship at Johns Hopkins. Towards the end of these two years, my research interest had shifted and focused on understanding the molecular mechanisms underlying the vascular damage in the arterial wall in giant cell arteritis. It was at this point when the VCRC fellowship program was starting, and Dr. Stone mentored me to get into it.

My research is currently focused on better understanding the role of matrix metalloproteinases in giant cell arteritis. Matrix metalloproteinases are a group of enzymes capable of degrading varying components of the extracellular matrix in both normal and diseased tissue. They have been found to be associated with several vasculitic disorders. In concrete, I am working on two different laboratory projects. The first consists on characterizing the specific matrix metalloproteinases involved in vascular damage in giant cell arteritis. The second is a genetic study to determine if certain polymorphisms, that is, mutations, of metalloproteinase-9, a enzyme known to be involved in giant cell arteritis, are associated with this vasculitis.

One of the main things I have learned from my patients is to listen to them.  Listening carefully to patients’ symptoms helps us to suspect a serious condition or relapse. Therefore we may act quickly avoiding delays in treatment and, subsequently, preventing or diminishing complications.

I think that to be a better patient is essential to follow the physician’s indications. Of course, this should not be done “blindly” but rather in a “educated” manner. By this I mean that if a patient finds, for example, that a treatment is not working,  call the doctor to discuss it with him/her. Another aspect would be by understanding that research is very important for advancing in the knowledge of the disease, and for developing new therapeutic options. An important challenge for doing investigation on vasculitis is the rarity of these disorders, which makes it difficult to get clinical samples for research purposes. By agreeing to participate in research projects whenever possible, patients can help to get better outcomes for themselves and others.Because most of the patients with vasculitis have chronic conditions, which require long-term follow-up, it is extremely important for them to engage in a good doctor-patient relationship. It is basic for them to find a physician they truly trust, and with whom they can communicate in a direct, open and honest way. They should take good care of themselves, attend regular follow-ups, and learn as much as they can about their diseases. The latter will help them to recognize promptly when they can be suffering a relapse, and then it will lead them to look for the best medical care in every situation.

My main job duty is to do research in the laboratory setting. I am also a student in the part-time Master of Public Health program at the Johns Hopkins Bloomberg School of Public Health.  So far, I have taken a series of courses in statistics and epidemiology, along with other required courses. I am currently taking a third series of courses on Genetic Epidemiology.  I plan to graduate next May.

In addition, I have also been allowed to observe patients in The Johns Hopkins Vasculitis Center. During my stay at Johns Hopkins I have seen more patients affected by Wegener’s granulomatosis, Churg-Strauss Syndrome, polyarteritis nodosa, and Takayasu’s arteritis than ever before.  Unfortunately, for the last few months my lab work and my classes in Public Health have made it impossible for me to attend the clinic. 

I am very grateful to the VCRC for the opportunity it has provided me with through this fellowship. My weekly visits to the Johns Hopkins Vasculitis Center clinic have offered me a unique opportunity of seeing a great variety of vasculitis patients, and some of them while going through challenging medical situations. This has greatly enhanced my clinical skills to manage patients with vasculitis. This fellowship has also helped me to increase my laboratory skills, and it has provided me with the opportunity of enrolling in an academic program in the form of a Master of Public Health in the worldwide prestigious Johns Hopkins Bloomberg School of Public Health. In addition, from the personal point of view, this experience has been also very fulfilling.

During my time at Johns Hopkins I would like to increase both my skills as a translational researcher and as a clinician. I think that the tridimensional approach of the training program I have been involved in during my stay here will help me to accomplish this goal. My bench work and the courses of the Master of Public Health program are providing me with essential tools to design and perform translational and epidemiologic investigations. In addition, the time I have spent in clinic has helped me to be a better and more skilled physician for my patients. By combining this training program, the detailed “picture” offered by the laboratory bench research, the patients observation in the clinical setting and the big “picture” provided by the epidemiology and public health perspective, I believe I can integrate a broad range of knowledge, that will overall and help me to be a better scientist.

Once I leave Johns Hopkins, I would like to continue working on vasculitis, and probably on other autoimmune and inflammatory disorders in Spain. I hope the skills I have acquired during my stay here will help me to earn a faculty position in Spain in where I can combine both, patient care and research. I would also like to participate in international projects in the future, which include collaboration among different countries. 

Francisco Silva, M.D. - Involved in Numerous Research Projects Hones Skills at Mayo

Ulrich Specks, M.D.

Professor of Medicine

Division of Pulmonary & Critical Care Medicine

Mayo Clinic

Rochester, MN 55905

Dr. Silva came to the Mayo Clinic in August of 2006 as a Vasculitis Clinical Research Fellow.  His fellowship is supported by the NIH-Rare Diseases Network and the multicenter Vasculitis Clinical Research Consortium.  The fellowship is designed as a post graduate training program that provides two additional years of post-graduate training in vasculitis research to rheumtologists, nephrologists, pulmonologists or other sub-specialists.  During this time he will achieve a Masters Degree in Clinical Research and complete several translational and clinical research projects.

Dr. Silva’s work at Mayo Clinic focuses on the identification of specific PR3-ANCA subsets that recognize specific epitopes, and he is investigating their utility as biomarkers of disease activity using samples from large trial cohorts.  In addition, he is involved in the VCRC longitudinal cohort studies and in a follow-up study of the Wegener’s granulomatosis etanercept trial.  Furthermore, he is investigating the clinical outcome of patients with orbital involvement of Wegener’s granulomatosis and the efficacy of mycophenolate mofetil in MPO-ANCA positive microscopic polyangiitis with mild renal involvement.

After completion of the VCRC fellowship and master’s degree program, Dr. Silva will return to Santiago, Chile, where he plans to establish a vasculitis center and conduct clinical research studies.

Since his arrival here last fall, I have gotten to know Dr. Silva’s enthusiasm and dedication to vasculitis and research.  He has picked up laboratory methods as quickly as the important theoretic pathogenetic concepts.  He is very inquisitive and extremely productive, and his work has already generated data for several abstract presentations and research papers.  But most importantly, through his caring personality he has enriched all of us who have had the privilege to meet Dr. Silva and work with him.  I have no doubt that Dr. Silva will succeed with his Master’s Degree program here at Mayo Clinic, but also with his long-term quest to become an independent clinician-investigator upon his return to Santiago, and I am looking forward to his continued long term collaborations with Mayo Clinic and the VCRC.

 

Dr. Francisco Silvaby Francisco Silva, M.D.

I grew up in Santiago, Chile where I completed both my undergraduate and medical degrees at the Catholic University of Chile.  I am married to Alejandra Araya who is a nurse and is completing her Ph.D. in Nursing Science at the University of Illinois in Chicago.  I enjoy reading Astronomy and observing the night sky.  I have especially enjoyed seeing the constellations from the northern hemisphere.

I became interested in becoming a physician in high school where I had the opportunity to participate in a scientific group organized by the Biology teacher.  It was a fantastic period in which I discovered the wonderful world of the microscope, fossils and experiments.  Since that time my interests in biology and the human body led me to medicine as the vocation that fulfills both.

Patients with vasculitis have fascinated me because of the complex nature of their disease manifestation. They are the type of patients you never forget.  To improve their treatment and care, we need to answer many open questions about etiology, pathophysiology and their relationship to other autoimmune diseases.

My interest in vasculitis was sparked by one most memorable patient I took care of during my early days of training.  She was a delightful woman in a public hospital in Chile who presented with skin ulcers on her legs.  This woman had histologically confirmed microscopic polyangiitis and a positive P-ANCA.  What initially seemed to be a minor ulcer progressed rapidly to compromise the entire foot, and new ulcers appeared on her upper extremities.  She also developed renal insufficiency.  I was impressed that a disease that initially presented itself as mild could progress so rapidly to extensive multi-system involvement.   After months of therapy she recovered physically and emotionally, more so than I ever expected. I was extremely impressed by her level of resilience.

My patients teach me about their lives and their disease – this is important, as it is a useful guide for the research I am involved in.  I try to teach vasculitis patients that they need to face their disease head-on and need to adhere to therapy.  They need to listen to what their bodies tell them so that they can work to keep their disease under control.

I am most grateful to the VCRC for providing me with the opportunity to improve my translational and clinical research skills through the fellowship at Mayo Clinic.  I am pursuing a Masters Degree in Clinical Research, and I am involved in several translational research studies.  These focus on the utility of PR3-ANCA subsets as biomarkers for disease activity, organ manifestations and prognosis in Wegener’s granulomatosis, and on the long term outcome of different therapies in the ANCA-associated vasculitides.

During my time at the Mayo Clinic I would like to acquire the knowledge and skills needed to become an independent translational clinician investigator. I think the Mayo Clinic is an ideal place to achieve this goal.  When I have completed my work here I want to return to my country, to become an expert in the care of vasculitis patients and to develop translational research projects in vasculitis.

  

 

Irish CCF Fellow Specializles in Patient Care and Vasculitic Investigation - Eamonn Molloy, M.D.

Carol A. Langford, M.D., MHS

Director, Center for Vasculitis Care and Research

Cleveland Clinic

I am pleased to introduce Dr. Eamonn Molloy who has been with us at the Cleveland Clinic since August, 2005 and who formally began his vasculitis fellowship in July, 2006. Thanks to the leadership of Dr. Gary Hoffman, the training of physicians interested in vasculitis has had a long history in the Cleveland Clinic Center for Vasculitis Care and Research. During their training, these physicians have contributed meaningful scientific work that has enhanced our understanding of vasculitis. After completing their fellowships, our graduates have provided expert care to vasculitis patients in Mexico, Asia, South America, Canada and the USA.

Even though our own Institution's enthusiasm and resources have been very important to our fellowship program, the training of a vasculitis expert is not possible without funding. The Vasculitis Clinical Research Consortium (VCRC) Fellowship that was discussed in an earlier Vasculitis Foundation newsletter is an important landmark in funding vasculitis education. However, it will be a long time before we have adequate numbers of vasculitis experts. Patient needs are often unmet and we lack a critical number of talented physician investigators who can lead future research directed towards finding cures. It is for this reason that we are so deeply appreciative of the Vasculitis Foundation's support of fellowship training.

Dr. Molloy is an excellent example of the promise of such training. As seen in his comments, Dr. Molloy is engaging and sincere in his commitment to vasculitis. His excellent education to date earned him funding from the R.J. Fasenmyer Center for Clinical Immunology to partially support his quest to acquire advanced knowledge and skills. However, if it was not for the generous support of the Vasculitis Foundation, completion of his vasculitis fellowship and Masters Degree studies would not be possible. It has been a great pleasure for all of us in Cleveland to work with Dr. Molloy and we are tremendously grateful for the support of the Vasculitis Foundation which has invested in our common goal to help people with vasculitis. 

  

By Eamonn Molloy, M.D., September 2006Dr. Eamonn Molloy 

I was born and raised in the western part of Ireland. As a family we were heavily involved with Irish culture – language, music and sports. There is no medical background in my family; my boyhood dreams focused more on winning the All-Ireland Gaelic Football championship with my county team, but the likelihood of those dreams coming to fruition was critically undermined by a lack of sporting prowess!

I first considered a career in medicine at age 17. The Irish system of medical training differs substantially from that in the United States, requiring the completion of a six year course, which is generally pursued directly after finishing secondary school (the equivalent of high school). The system for college entry is based on the ‘Leaving Certificate’, an examination taken at the end of secondary school. This is the most onerous examination that Irish students face at any time during their academic career, with approximately 10 days of examinations, typically consisting of 6 hours of written/essay papers per day across at least 6-7 subjects.

My decision to apply for medical school was a late one, coming after completion of the Leaving Certificate, just before the deadline for revised submission of college course choices. I had not been sure as to what I wanted to do in college because I enjoyed studying a broad range of subjects at secondary level. I had not really considered medicine until my mother suggested it while I was discussing with her some of the latest medical news from Time magazine. The more I thought about it the more it appealed to me so I applied about one week later, started medical school at University College Dublin in October 1991 and I have not regretted it since.

The six year medicine course is followed by a one year internship (six months of internal medicine and six months of surgery). At this point, doctors are fully registered practitioners. Subsequent to this, I entered a senior house officer scheme (analogous to a residency scheme) in internal medicine at the Mater Misericordiae University Hospital (MMUH) in Dublin. This provided a number of key experiences that influenced my subsequent career choices. A 3-month rotation on the Rheumatology service convinced me that my future lay within this specialty. The patient-centered approach and the potential to build a rapport with patients over the course of their chronic illness was an important factor in this decision. Many of the rheumatic conditions are multi-system diseases that provide a diagnostic and therapeutic challenge for the clinician. Even at that early stage, I was particularly stimulated by the challenge provided by the vasculitis patients that I cared for during this rotation.

As part of the MMUH Senior House Officer scheme, I then spent a six month period as an internal medicine resident at the Mayo Clinic, Rochester, Minnesota, USA. This was another important experience, as it emphasized the potential benefits to my career of seeking specialty training in a top-level academic center in the USA.

I commenced the Specialist Registrar (SpR) program in Rheumatology and Internal Medicine in July 2000. This is a 5-year program, overseen by the Royal College of Physicians in Ireland, which ultimately leads to Certification of Satisfactory Completion of Specialist Training (CSCST; analogous to specialty board certification in the USA). The first three years of my SpR program were spent in internal medicine and rheumatology at three different teaching hospitals in Ireland, including one year at MMUH, where one of my mentors was Dr. Geraldine McCarthy. I subsequently began a two-year research experience under the supervision of Dr. McCarthy. This was undertaken at the Department of Molecular and Cellular Therapeutics at the Royal College of Surgeons of Ireland Medical School in Dublin and MMUH. This laboratory-based research explored the role of prostaglandins in calcium crystal-associated osteoarthritis. Based on this work I submitted a thesis for a Doctorate of Medicine degree through the National University of Ireland, which was accepted in January 2006.

Health care in Ireland is provided mainly by a public health care system, supplemented by a number of private hospitals. The government-funded public health care system provides acute and elective care for the entire population. The private hospitals mainly engage in elective clinic services and non-acute inpatient care. Because of the shortcomings in the public system, approximately half of the population purchase private health insurance so that they can access these services in the private sector in a more timely fashion.

Rheumatology has historically been underserved in Ireland, with long waiting times for clinic appointments in many centers. Vasculitis has a relatively low profile in Ireland, with no established vasculitis center. Because of the shortage of rheumatology posts, demand for positions and quality of applicants has been high. Therefore, virtually all rheumatologists have trained both in Ireland and in international academic centers, mainly in the United Kingdom and North America, and have thereby gained significant experience in management of patients with complex rheumatic diseases. However, this scarce resource is further diminished by the fact that the vast majority practice as dual internists-rheumatologists. A new strategy aims to considerably increase the number of rheumatologists, and critically, also the number of appointments in the allied health professions. Hopefully, if implemented, this strategy will significantly increase the resources available to Irish patients with vasculitis and other rheumatic diseases.

As mentioned above, I have had a specific interest in vasculitis from an early point in my career. To further my goal of sub-specialty training in vasculitis, I sought out the opportunity to participate in the Vasculitis Fellowship Program at the Center for Vasculitis Care and Research at the Department of Rheumatic and Immunologic Diseases, Cleveland Clinic Foundation. This commenced in August 2005. Since then I have gained invaluable experience in the care of vasculitis patients under the supervision of Dr. Carol Langford (Program Director), Dr. Gary Hoffman (Department Chairman) and Dr. Leonard Calabrese.  I am planning to spend a further two years as a vasculitis fellow at the Cleveland Clinic Foundation, to gain further experience in the care of patients with these complex diseases. In addition, I have enrolled in the two-year Clinical Research Scholars Program at Case Western Reserve University. This program is specifically aimed at teaching clinical investigative skills, culminating in the award of a Master of Science in Clinical Research degree.

The support provided by the Vasculitis Foundation Fellowship will enable me to achieve these goals. My ultimate objective is to develop the skills necessary to provide expert care to vasculitis patients and to become an effective vasculitis investigator, preferably in Ireland. The potential exists for participation in international vasculitis collaborative research networks, which could benefit vasculitis patients in Ireland and elsewhere.

    

Otolaryngologist Performs Reconstructive Surgery for WG Patients - Robert S. Lebovics, M.D., F.A.C.S.

Dr. Lebovics 

 

 

 

 

 

Robert S. Lebovics, M.D., F.A.C.S. is a nationally known Otolaryngologist with expertise in infectious and inflammatory disorders for both adults and children, nasal and sinus surgery, and disorders of the special senses. Dr. Lebovics is a New York native who received his medical degree from the State University of New York in Brooklyn and trained at the Albert Einstein/Montefiore Hospital Center in otolaryngology. For nearly nine years, Dr. Lebovics was Chief of the Clinical Otolaryngology Service at the National Institutes of Health in Bethesda, Maryland.

In 1997, Dr. Lebovics joined the Head and Neck Surgical Group, affiliated with the St. Lukes-Roosevelt Hospital Center. In 1999, Dr. Lebovics was appointed Chief of Otolaryngology/Head and Neck Surgery at Cabrini Medical Center in New York City.

Among his accomplishments, Dr. Lebovics has authored over 25 articles and text book chapters in the field of otolaryngology head and neck surgery. Dr. Lebovics continues as a surgical consultant to the National Institutes of Health in Bethesda, MD for the Department of Health and Human Services.

Dr. Lebovics has seen approximately 200 WG patients including patients from Central America, Mexico, Canada and Ireland. He receives calls from physicians in Australia and Europe seeking advice and tapping into his expertise.

Most of the surgery that Dr. Lebovics performs is in the reconstructive realm. He has performed many surgeries on WG patients in need of reconstruction for saddlenose deformity. His goal for this type of surgery is a fine balance between cosmetic and reconstructive procedures for the best possible outcome.

Dr. Lebovics also performs tracheal repairs on WG patients, a serious operation with no room for mistakes. Few situations in medicine are as black and white as the need for an open airway. Endoscopic dilation is the preferred method but not always sufficient for the repair. Each repair is an individual condition with its’ own problems and peculiarities.

Dr. Lebovics enjoys the work he is able to accomplish in the operating room. With the belief that we need to live for today and do all we can to make each day brighter, he sees his work as a method to achieve this goal.

Dr. Lebovics has learned a great deal from his WG patients - medically and philosophically. There is no limit to the power of the human spirit and no greater lesson than that of redemption. There is always hope, there is a future, do not give up. Dr. Lebovics emphasizes that he can and wants to help his WG patients with the pain, discomfort and deformity that WG so often brings. He loves his WG patients and says they are a fun loving group - some of the nicest people you could ever meet.

During his time at NIH, Dr. Lebovics worked with Dr. Fauci, Dr. Hoffman and Dr. Langford on WG research. There was a tremendous need for the research at the time along with an interest among these doctors that resulted in a “match made in heaven” team. The combination of brilliant physicians and a multidisciplinary approach to the research resulted in great strides within the knowledge and treatment of WG.

Gail Schultz, cosponsor of the NYC support group, says that Dr. Lebovics has been the primary promoter of the NYC support group. He has graciously offered the conference room of his practice for meetings which otherwise would have no place to gather. He attends every meeting and is always available to answer questions. He is dedicated to his patients and once took a patient aside to provide a consultation after one of the support group meetings.

Dr. Lebovics is not only a wonderful and knowledgeable physician, but a great human being as well, says Gail. He truly cares about his patients and in return, he has the admiration of every WG patient in the tri state area (New York, New Jersey and Connecticut).

*Some material excerpted from www.entsurg.com with Dr. Lebovic's permission.

Search Begins for a Genetic Link to Vasculitis - Sharon A. Chung, M.D.

By Sharon A. Chung, M.D., April 2007

Dr. Chung

My story begins in Raleigh, NC, where I was born and raised.  My father is a retired a physics professor from North Carolina State University (NC State) and my mother is an artist.  My sister is 5 years older than me and a pediatric ER doctor in Boston. 

I must say that I was extremely lucky growing up—the schools I went to had a strong emphasis on science, and everyone around me was tremendously supportive of my interests.  One of my most influential experiences was attending the North Carolina School of Science and Mathematics (NCSSM) for my last 2 years of high school.  While at NCSSM, I took a class called “Research in Biology”.  Although it had a very simple name, I cannot underestimate the impact of this class on me.  It introduced me to molecular biology, and allowed me to develop an independent research project on yeast RNA viruses.  The results of the project were definitely not earth shattering—but the project itself jump-started my desire to conduct research studying human diseases. 

After high school, I went to NC State and obtained a degree in Chemical Engineering.  I had an inkling at that time that I wanted to go to medical school, and to continue research exploring medical diseases.  However, I knew that I wasn’t a “pure science” person—that is, that I enjoyed applying science towards something more than the just discovering the science itself.  Therefore, I obtained an applied science degree—engineering.  While at NC State, I became interested in genetics, and began taking classes in this area, with the idea that I may do research in this field in the future.

After graduating from NC State, I went to medical school at Johns Hopkins University in Baltimore, MD, and stayed at Hopkins for my residency in internal medicine.  I realized 2 extremely important things while at Hopkins—First, that I enjoyed seeing patients far more than I enjoyed growing yeast or working a pipette in a lab.  Second, I was introduced to rheumatology, and became fascinated with this area of medicine.  I remember the vasculitis clinics I attended with John Stone and David Hellmann.  I saw such a range of diseases during that time—from Wegener’s granulomatosis to Takayasu’s arteritis to Behçet’s to CNS vasculitis.  I remember asking myself—“How do these diseases start?  We believe that the immune system loses the ability to distinguish itself from foreign cells—but why?  Is there anything that makes it more likely to happen—genetics, environmental exposures, infections?  Why does it affect some, and not others?” Given my previous interest in genetics, I realized that I had found my calling—to study the genetics of autoimmune diseases, and to use genetics to learn how these diseases develop.   

I came to University of California, San Francisco (UCSF) for a rheumatology fellowship, in part, because of the well-developed genetics research program in rheumatology at UCSF.  Dr. Lindsey Criswell, my mentor, has significant experience studying the genetic epidemiology of both rheumatoid arthritis and lupus.  With her, I have developed a research project to identify genes related to autoantibody production in lupus.  These autoantibodies are believed to play a role in starting lupus.  By understanding which genes are involved in producing these autoantibodies, we may gain insight into the genetic causes of lupus.

My research goal is to expand this work in lupus genetics to other autoimmune diseases, such as vasculitis.  In the future, I would like to study how genes, and which genes, influence the risk of developing vasculitis.   This knowledge may help us predict who is at risk for disease, develop potential treatments, identify which treatments may be more effective for a particular patient, or perhaps even prevent disease.  These are definitely long-term goals—we are not close to achieving them currently—but the potential for reaching these goals is growing! 

I don’t want to give the impression that the only thing I think about is research—quite the opposite!  My clinical practice at UCSF is an important and valuable aspect of my rheumatology career.  I currently see a wide variety of patients with different autoimmune diseases, including vasculitis.  However, due to my interests, I am interested in seeing more vasculitis patients in my clinic and developing an expertise in this field.  In addition, I am spending a month this year at the Vasculitis Center at the Cleveland Clinic to expand my experience caring for these patients.

I find it extremely rewarding to care for vasculitis patients.  Often times, I first meet them when they are quite ill—for example, wheelchair bound due to nerve damage, or in the intensive care unit (ICU) due to organ failure.  These patients have shown me the body’s amazing capacity to heal.  My patient who was wheelchair bound is now walking on her own; the patient who was in the ICU is now a college student.  It is uplifting to see my patients make such significant strides in their quality of life.  I’d like to think that I have some impact with medications and a caring ear, but I know that my patients have a significant role on their own recovery.  Their positive outlook, dedication to medical care, attention to physical therapy, self-education, determination to improve—all of these aspects have helped them progress and I am proud of their accomplishments. 

This July, I will finish my rheumatology fellowship and join the faculty at UCSF.  I look forward entering a new stage of my career and developing a stronger relationship with the vasculitis community!

       

Subglottal Stenosis in Wegener's Patients - Robert Lorenz, M.D., F.A.C.S.

Dr. LorenzDr. Robert Lorenz is an ENT physician and surgeon at the Cleveland Clinic Foundation (CCF) in the Department of Otolaryngology, where he is Section Head of Head and Neck surgery. His area of special interest for Wegener’s patients is the larynx. WG is unique in its ability to involve the larynx while the other vasculitides do not. When subglottal stenosis affects the larynx, the cause can be either WG or idiopathic (of unknown origin), or sometimes due to trauma such as breathing tubes.

Almost all WG patients have, to some extent, involvement with their nose and ears. However, few WG patients have problems with their larynx. Only 20% of adult WG patients have subglottal stenosis. This jumps to 50% in patients under 20 years of age, the reason for this unknown. In children, the SS can remain as an issue for years. Dr. Lorenz finds that as long as the WG is not active, the SS stays quiescent . Once the patient has an exacerbation, the SS can flare and then the body’s natural ability to create a stenosis from the scar occurs and the physician and patient struggle to get the airway widely patent while preventing further scaring. SS is a disease that you either have or you do not - there does not seem to be “a little subglottal stenosis”.

Dr. Lorenz has a basic philosophy when it comes to treating subglottal stenosis. If a person is not limited in their activities by their pathology then there should be no intervention. This is an extremely important point. Some physicians may tell a patient that they have a little subglottal stenosis and that something should be done. Unless a patient says they are having problems, Dr. Lorenz will not perform surgery. The only exceptions to this are in patients who are anticipating problems, such as someone needing to undergo general anesthesia and their airway is too tight to get a breathing tube down, or in a pregnant woman who will need a widely open airway to go through the added stress of labor without a restricted airway, but these scenarios are very rare.

This ties into the second part of his philosophy; the more you intervene, the more scarring that will result along with long-term problems. The larynx is an extremely delicate organ and the more you traumatize it, the less chance of recovery in the future. Dr. Lorenz says “Be gentle and do the least amount of trauma to the voice box“.

An important point to understand about subglottal stenosis is that it acts independently of generalized WG disease and systemic therapies. If somebody flares, it doesn’t mean the larynx will get worse. If the larynx is getting worse, it does not mean you are in a WG flare. Lastly, it means that systemic immunosuppression usually does not help reduce the severity of the subglottal stenosis.

Problems do not usually arise unless there is a 50% or greater reduction in the airway. At this point, one returns to the philosophy of “are you having problems?”. You may have a 50% reduction yet not have any significant problems and so you would not have any treatment. Older patients seem to be more subjectively affected with SS - patients over 50 tend to feel more confined by it while younger patients tend to do better.

Dr. Lorenz tries to perform as few surgeries as possible and when he does, the goal is to be as atraumatic as possible. Under general anesthesia, working through the mouth with no tracheotomy or neck incisions in a "minimally invasive", endoscopic manner, the first part of the treatment is the injection of localized steroids which seems to be the most important part of preventing the stenosis from returning. Next, the stenosis is lysed with a small endoscopic knife, and thereafter, the stenosis is lightly dilated. It is very important that the dilation part of the procedure is done very gently so that no further scar tissue is caused. Lastly, a topical application of mitomycin-C is used and the patient is awakened. So far, as long as the patient has not had previous airway surgery by a different surgeon prior to Dr. Lorenz, he has never had to perform a tracheotomy or other more invasive surgeries, and the minimally invasive approach has been able to maintain the airway open. Repeat procedures may be needed at intervals of 8-12 months, but usually are limited to 3 procedures.

Dr. Lorenz does not personally perform the radial incision in the larynx with the laser because there is some evidence that lasers are more traumatic to the larynx in animal studies, but the new generation of lasers may be able to avoid this problem.

Most ENT’s are surgeons, but are generalists who treat all things such as allergy, children, adult, cosmetics, ear, sinus, etc. There are very few with significant interest and experience with Wegener’s and other vasculitides. There are only a handful of ENT’s who have significant experience with subglottal stenosis.

Disease in the sinuses ranges from minor inflammation all the way to tissue destruction. At the least, there is usually some redness, swelling and some crusting. In the worst cases, the septum has completely eroded leaving a large nasal cavity with no real sinuses left.

The difference between disease in the nasal cavity and in the sinuses is as follows … Disease in the nasal cavity centers around crusting, bleeding and a destroyed septum. Disease in the air-filled spaces of the sinus cavity is primarily infection or sinusitis. Sinus surgery is rarely performed when a person is in a WG flare. Once a diagnosis comes back, organ systems are checked, treatment with meds is decided to bring the WG under control and with proper treatment, the nose tends to get better and surgery can be avoided.

It is extremely important to take good care of your nasal and sinus passages. Proper hygiene is the primary method of preventing problems in our WG patient population of approximately 200. It is important for all patients to know the complications that can arise from poor hygiene. They include acute sinusitis, mucoceles (walled off area of mucous which can eventually involve neighboring tissues like the eyes and brain), and ear problems.

Dr. Lorenz performs surgery only with the larynx in WG patients. If care is needed for the sinuses, he refers them to Dr. Citardi or Dr. Batra, both also at the CCF. Ear problems are referred to a specialist as well.

Dr. Lorenz is from New York, north of New York City in the Hudson Valley. His parents were immigrants from Europe after WWII. He currently lives with his wife, who is also a physician, in Shaker Heights, Ohio.

When Dr. Lorenz is not in Cleveland seeing patients and performing surgery, he spends much of his time lecturing. In May he was in Chicago followed by trips to Colombia, Panama, Turkey and then on to Baltimore for the Vasculitis Foundation Symposium in July. When he is not taking care of patients, he likes to relax at home with his wife, Aylin.

As a college student at Yale, Dr. Lorenz became involved with an international medical equipment aid group and visited hospitals in Central America. This is what really inspired him to become a doctor. He is the first member of his family to become a doctor. Dr. Lorenz has also been on medical mission trips to Africa.

Dr. Lorenz credits Drs. Isaac Eliachar and Gary Hoffman as fantastic teachers who taught him an incredible amount about vasculitis patients. Dr. Bob Lebovics and Dr. Carol Langford have also been excellent collaborators.

Dr. Lorenz states that the best part of his job is making a person who has a severe airway narrowing which affects their daily life, breathe normally again. He feels that WG patients are wonderful people - folks just like him, but were very unlucky to develop a disease that could affect anyone. His practice is limited to laryngotracheal reconstruction and Head and Neck tumors.

Vascular Surgery in Patients with Vasculitis - Ravi Veeraswamy, M.D.

By Ravi Veeraswamy, M.D.Dr. Ravi Veeraswamy

 

 

As a vascular surgeon, I am frequently asked to perform temporal artery biopsies to make a diagnosis of arteritis.   This is perhaps the most common referral I receive.

Other physicians not familiar with the disease process also often refer vasculitis patients to vascular surgeons.  The physician and the patient are concerned about arterial insufficiency or arteriosclerosis as the cause.  For example, I recently referred a gentleman in excellent health without any risk factors for arteriosclerosis who noted that his fingers became discolored on several occasions in cold temperatures.  The role of the vascular surgeon is to identify the underlying process and refer the patient to an appropriate specialist, usually a rheumatologist.  The most common type of referral in this category is for patients with Raynauds Phenomenon.  Often, the presentation can be confusing and we will perform a diagnostic angiogram to help define the problem and rule out other causes such as proximal aneurysms or other embolic sources.

The other end of the spectrum is when patients are referred to us for “end-stage” disease.  Patients have developed chronic stenosis of vascular beds such as the upper extremities or the carotid circulation and they need bypasses to restore arterial flow.  In this scenario, it is the rheumatologist who sends the patient to us.  Patients typically need bypass grafts placed distal to the areas involved in the inflammation.  For example they may need bypasses from the aorta to the carotid or from the carotid to the brachial and so on.   The decisions on specific bypasses are based on a given individual’s arteriograms and anatomy.

We typically are “repairing damage” rather than preventing it.  There are really no great surgical treatments currently to help prevent damage in the setting of vasculitis.  I first encountered patients with vasculitis in the surgical clinic as a medical student.  I was amazed at how little we knew about the root causes of the problem.  While we have learned a lot since then, there is a long way to go.  My research interest has always been in immunobiology and in managing patients with vasculitis, I am able to combine my clinical and research interests.  Currently, I am establishing research projects to study the role of dendritic cells and T cells in vascular biology and the arterial wall.  Hopefully this will help us understand the process that initiates the inflammatory response and give us insight into how it might be controlled.

On a personal note, I have wanted to be a doctor literally for as long as I can remember.  I used to dress up as a doctor all the time as a kid.  I still find the profession fascinating and consider myself very lucky to be in a field where I can directly help other people.  I think the best relationship is when the doctor and the patient work together with a common understanding of the possibilities of treatment and the limitations of treatment.  The best thing a vasculitis patient can do is find a good rheumatologist with an interest and experience in managing the vasculitis.   

Vasculitis Center to Open in Salt Lake City, Utah - Curry L. Koening, M.D.

Carol A. Langford, M.D., MHS

Director, Center for Vasculitis Care and ResearchDepartment of Rheumatic and Immunologic Diseases

Cleveland Clinic Foundation

 As Dr. Koening discusses in his interview, he is one of a group of talented young investigators who have been selected to receive training through the Vasculitis Clinical Research Consortium (VCRC) that is sponsored by the National Institutes of Health. The establishment of a vasculitis fellowship through the VCRC was vigorously pursued and supported by the VCRC’s Principal Investigator Dr. Peter Merkel and the entire VCRC steering committee. The education of physicians to become vasculitis investigators represents one of the most important contributions that we may bring to the care of people with vasculitis and to the future of our field.

Dr. Koening and all of the VCRC scholars exemplify the promise that this future holds. Upon completing his vasculitis fellowship, Dr. Koening will be taking the skills that he has gained back to the University of Utah where he will be establishing a Vasculitis Center. This Center will serve as a vital resource to people with vasculitis in the Western United States and as a valuable partner in ongoing research studies. It has been a great pleasure for all us in Cleveland to work with Dr. Koening and we look forward to the opportunity to collaborate with him for many years to come.

By Curry L. Koening M.D., February 2006  Dr. Koenig

I first realized that I wanted to be doctor when I was in high school. I grew up in a small town in West Texas. Farming and ranching were how most families made a living in this part of the country. Oil also played a huge part in the local economies. However, lack of primary medical care was a major issue in many of these small towns. Not many doctors were moving to these rural areas to start a practice, leaving many with limited access to medical care. I began medical school with the intent of moving back to rural Texas and starting a general medical practice.

After college I went to medical school at Texas A&M System Health Science Center College of Medicine. I was first introduced to rheumatology during my third year clerkship. I decided to pursue it as a profession after spending some time on the rotation during my internal medicine residency at the University of Utah. Rheumatology was not unlike primary care. It too needed new, young doctors to help underserved areas, especially where wait times to see a rheumatologist may have been as long as six months. I completed my rheumatology fellowship at the University of Utah in June of 2005 and moved to Cleveland shortly thereafter to learn more about vasculitis at the Center for Vasculitis Care and Research at the Cleveland Clinic Foundation. Because few doctors have trained specifically in vasculitis, patients with these diseases are underserved. Research in this field is also lacking and clinical trials for new therapeutics have lagged behind.

I am fortunate to be receiving support for my vasculitis training from funds provided by the National Institutes of Health through the Vasculitis Clinical Research Consortium (VCRC). It is the mission of the VCRC to train physicians to serve patients with vasculitis and through research provide better therapies, diagnostic tools and identify the causes of these diseases. I am fortunate to be a part of this organization and I look forward to playing a greater role in the care of patients with vasculitis and leading research studies in the future.

The Vasculitis Clinical Research Consortium (VCRC) has four main clinical centers: Boston University School of Medicine, Cleveland Clinic Foundation, Johns Hopkins School of Medicine and Mayo Clinic College of Medicine. The goal of the VCRC is to conduct clinical research in hopes of developing new treatments for patients afflicted with vasculitis. It has been the goal of the VCRC to train new clinicians to care for patients with vasculitis, conduct clinical trials for new drugs in the treatment of vasculitis, and work with vasculitis patient support groups. My goal as a trainee through the VCRC is to maintain this tradition. My training will consist of two years of research, patient care and academic work. This includes not only learning how to better care for patients with vasculitis, but also graduate work in how to perform basic clinical research that will serve to ultimately improve our patient’s daily lives.

My current research interest is in disease monitoring. Little is known regarding how best to follow patients with vasculitis who are considered to be in “remission”. For most patients who are on immunosuppressant drugs, regular blood and urine monitoring are a requirement. How to follow patients who are in a low disease state or even disease remission and not on immunosuppressant drugs is much less clear. Do these patients need regular blood monitoring to detect an early disease relapse? Do these patients need regular CAT scans or chest x-rays to detect an asymptomatic lung relapse of their disease? If so, then how often do they need to be seen and is it cost effective to do regular lab monitoring? These are questions that still remain and that future research will address.

My first encounters with WG and other forms of vasculitis were during my residency. As you might guess, these experiences were infrequent. Nonetheless, I had the opportunity to care for a few patients with WG, Takayasu's arteritis, Churg-Strauss and other forms of vasculitis when they were hospitalized. I learned in my fellowship that patients with vasculitis often have life-threatening complications, but with prompt and proper therapy they can do very well.

The number of vasculitis patients that we see at the Cleveland Clinic varies from week to week.   About 50 patients with vasculitis will be seen on average per week. WG patients make up a large number of those seen and is one of the most common vasculitides that we take care of at the Center for Vasculitis Care and Research. Other vasculitides that we see commonly include Takayasu’s arteritis, giant cell arteritis, polymyalgia rheumatica, polyarteritis nodosa, Churg Strauss and Behçet’s. The Center for Vasculitis Care and Research has one of the largest populations of WG patients worldwide. Not only has the Center committed itself to the care of patients with vasculitis but also to increasing our knowledge about these diseases through clinical trials and basic research.

 

As a fellow in vasculitis my job entails many things:

1) Designing and participating in research.

2) Participating as a co investigator in clinical trials.

3) Seeing and treating new and established patients who have vasculitis.

4) Working closely with clinical staff members who have cared for vasculitis patients for many years and learning from their experiences.

5) Being available to other clinicians world wide to answer questions and to help better serve patients with Vasculitis.

6). Teaching medical students and residents basic approaches to diagnosing and treating Vasculitis.

7). Completing academic work in a pursuit for a master’s degree in the Clinical Research Scholars Program at Case Western Reserve University.

 

The importance of communication has been the most helpful thing that I have learned during my training. I have found that taking the time to discuss any concerns that patients have and helping them develop a plan to deal with their disease has greatly enhanced my relationship with my patients. In this day and age of an electronic medical record and e-mail there is no reason that patients should ever feel “left out of the loop.”

I think there are some things that we as physicians and patients can do to make things easier for both. First, I think it is very important for patients to have a vested interest in their disease. In this way, patients will be more apt to report changes that they may recognize as a disease flare. Patients will also be prompted to ask questions that are important to their own care. Patients should be optimistic about the progress that is being made that has led to better care for patients with vasculitis. Progress in the field of vasculitis research can not happen without the help of patients. I think it is important for patients to keep an open mind when it comes to participating in clinical trials. The more people that are willing to participate in trials, the better our understanding of these rare diseases will be. Patients should also be involved in their local Vasculitis support groups. It is through these groups where patients can learn about new therapies and how they can support future research in this area.

I think the best advice for any vasculitis patient is to recognize that recent medical advances have made not only long term survival but long term disease remission a reality. Even though disease remission is a reality for almost all patients, a cure is still many years away. Patients should continue to have frequent follow-up visits with their care providers, ask pertinent questions and be involved with their local and national Vasculitis organizations. This also includes patients who have been in disease remission for an extended period of time. These patients also have a high risk of relapse from their disease. Vigilance can prevent complications. Early detection of relapses can lead to early treatment, shorter exposure to immunosuppressant drugs, less morbidity and hopefully a faster return to normal daily living.

I have several goals that I would like to accomplish before my time is complete at the Cleveland Clinic. My ultimate goal is to learn how to design and implement clinical trails for the treatment of patients with Vasculitis. I would also like to be a part in developing new imaging and laboratory modalities that will help how we follow patients in the future. Through my course work at Case Western Reserve University and clinical time at the Cleveland Clinic, I hope also to forge collaborations worldwide with clinicians and researchers who are dedicated to better understanding Vasculitis and the patients we care for. Finally, as any young physician I want to provide the most advanced therapies for my patients in a compassionate clinical setting for which patients can feel a part of.

My plans at this time are to take an academic appointment at the University of Utah in Salt Lake City. Here, in conjunction with the Department of Medicine and Division of Rheumatology, we plan to create a vasculitis center. This will be the first Center in the Western United States. Our goal is to be a tertiary referral center for patients from all over the United States and the World who are afflicted with either WG or other rare vasculitides. We will also offer clinical trials of new therapeutics to treat patients with vasculitis that would not otherwise be able to participate. We also hope to build a strong relationship with local and national Vasculitis support groups such as the Vasculitis Foundation and maintain a strong collaborative network with the Centers for Vasculitis Care and Research and other Vasculitis centers around the world.

 

Young Investigator Seeks Answers Through Epidemiological Studies - Alfred D. Mahr, M.D.

Peter A. Merkel, M.D., M.P.H.

Principal Investigator, Vasculitis Clinical Research Consortium  

Director, Boston University Vasculitis Center

Associate Professor of Medicine

Boston University School of Medicine

It is a great pleasure to introduce Dr. Alfred Mahr to members and friends of the Vasculitis Foundation.  Dr. Mahr has been a Research Fellow and Visiting Scholar at the Boston University Vasculitis Center since November 2005.  He is one of four current Fellows supported by the Vasculitis Clinical Research Consortium (VCRC) which receives its funding from the National Institutes of Health.  One of the core missions of the VCRC is to train new investigators in vasculitis and thus help expand the number of caregivers and researchers in vasculitis. It is hoped some of the Fellows will go on to establish new centers of excellence for vasculitis. 

Unlike the other current VCRC Fellows, Dr. Mahr was already an investigator in vasculitis when he joined us here in Boston.  As a member of the French Vasculitis Study Group in Paris, Dr. Mahr studied under Dr. Loïc Guillevin.  Dr. Mahr was a productive researcher in vasculitis and a clinical expert in the treatment of these difficult diseases.  We were thrilled that Dr. Mahr wished to come and study here at Boston University where he is pursuing advanced courses in clinical epidemiology and biostatistics, and has designed and is implementing novel research studies.  The research projects he has developed includes clinical trials, clinical epidemiology, and genetic studies; the diseases his research covers includes Wegener’s granulomatosis, microscopic polyangiitis, Churg Strauss vasculitis, and giant cell arteritis.  His first year as a VCRC Fellow has been a productive one and his second year will be even more exciting.

The support of clinical and research fellows in vasculitis is instrumental to not only continuing to provide care and conduct research, but also to expand the scope of care and research in vasculitis.  The continued support from the NIH and the Vasculitis Foundation makes this training possible.  The impact that young investigators have on the productivity and conduct of research should not be underestimated.   

Young Investigator Seeks Answers Through Epidemiological Studies

By Kathy Savickas, December 2006

Alfred, Laurence and Simon MahrDr. Alfred Mahr was born in Germany and at the age of four he moved to France for the next five years.  His whole family fell in love with France and though they returned to Germany when he was nine, they visited often for vacations.  France would become Dr. Mahr’s second home.

Dr. Mahr knew in high school that he wanted to attend medical school.  His father was an engineer and his mother a physical therapist but his grandmother, great-grandfather and great-great-grandfather were also doctors.  Dr. Mahr knew that he wanted to work with people and medicine was a perfect balance between an intellectual challenge and humanitarian work.  That mixture drove him to become a doctor and is what continues to motivate him today.

Once Dr. Mahr finished high school in Germany, he moved to Montpellier in the south of France to attend medical school, the oldest in the country, and complete his residency in internal medicine. He then moved to Paris to specialize and complete his fellowship.  Dr. Mahr’s wife, Laurence, is also a physician and they have a five-month-old baby named Simon.

While doing rounds in various specialties, he met his first vasculitis patient in 1995 when he was a fellow in internal medicine.  The patient initially presented with fevers and muscle aches.  It took quite a while to unravel the nature of the patient’s disease before he could be diagnosed with Polyarteritis nodosa. Soon thereafter, he saw an elderly lady with Microscopic polyangiitis involving the kidney. At that time, Dr. Mahr became intrigued by vasculitis.  He felt that, because they were rare and presenting so differently from patient to patient, vasculitides were more difficult to understand and recognize than many other diseases he has taken care of since then.

In 1997 he decided to complete rounds with Dr. Loïc Guillevin who is the head of the Department of Internal Medicine at Hôpital Cochin in Paris and a prominent expert in the field of vasculitis in Europe.  Folks come from many countries to seek his help.  During his rounds with Dr. Guillevin, Dr. Mahr came to see many patients with different forms of vasculitis. At that time, he decided to devote his medical thesis to Wegener’s granulomatosis. After completion of his fellowship in 1998 he accepted an offer to return to Dr. Guillevin’s department as a faculty member, a position that he has not left until now. Since 1998, he has continuously worked in the field of vasculitis and has learned to take care of various types of vasculitis including Wegener’s granulomatosis, Microscopic polyangiitis, Polyarteritis nodosa, Behçet’s disease, Churg Strauss syndrome, giant-cell arteritis, Takayasu’s arteritis, and others.

During this time, Dr. Mahr also completed a Master’s in Public Health and began to foster his personal research by combining his interest in vasculitis with epidemiology.  Epidemiology can be defined as a field of study that searches to understand the triggers and causes of diseases.  Dr. Mahr has accomplished different studies conducted on the prevalence of vasculitis in the Parisian region, which include an investigation of factors that predict the severity of vasculitis and a study of links between use of particular anti-asthma drugs and Churg Strauss Syndrome. Dr. Mahr is also working on his Ph.D. thesis in Public Health, which he plans to submit in 2007 on the theme of vasculitis.

While attending various meetings, Dr. Mahr met Dr. Peter Merkel who, in time, invited him to come to Boston and work on vasculitis research by enrolling in a fellowship program with the Vasculitis Clinical Research Consortium (VCRC).  Boston proved to be an excellent location for Dr. Mahr because the Vasculitis Center in Boston has both the skills in vasculitis and the tradition of doing epidemiological studies.  The position couldn’t be more perfect for the studies that he is currently working on.  He took a sabbatical from his position in Paris for two years and came to Boston in November 2005.

Dr. Mahr’s primary goal as he works in Boston is to become more skilled in research.  He is grateful he has the opportunity to study here and is learning a lot in the United States from a scientific and a personal standpoint. He is taking advanced classes in statistics, genetics, and research methods.  Dr. Mahr is also conducting new studies of Wegener's granulomatosis, Churg Strauss, giant cell arteritis, and other topics.  When his training in Boston is completed in November 2007, Dr. Mahr plans to return to Paris where he will continue to work with Dr. Guillevin. However, he hopes to continue collaborating with physicians from the United States as well as from other parts of the world. For rare diseases such as vasculitis, it is crucial that physicians and researchers from different countries combine their efforts to keep moving forward in the understanding and treatment of these illnesses.

Dr. Mahr enjoys taking care of his patients.  He feels privileged in his role as their doctor and of being able to assist them while they are going through a difficult patch. He feels that a good relationship between a patient and his doctor should be built on one of complete trust and confidence.  Dr. Mahr encourages his patients to tell or ask him everything they have on their mind. His motto is “There is nothing to hide” and “There are no stupid questions”!