Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss Syndrome, is a rare form of vasculitis affecting small-to-medium sized blood vessels. Although the number high-quality clinical trials have been increasing over the years, there remains a need for consensus recommendations to help with evaluation and management of the disease.
To address these concerns, a EGPA Consensus Task Force was formed. Comprised of 23 experts from Europe and the United States, they met several times between 2009 and 2013. They published their work in the September 2015 issue of European Journal of Internal Medicine.
“Among systemic vasculitis, EGPA has a very special place: It belongs to the group of anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitides, but its pathogenesis is more complex,” said Professor Loïc Guillevin, from Université Paris Descartes in France. “This complexity has an impact on patient care.”
Work Began With 40 Questions
The Task Force’s work began with a list of 40 questions forming the basis of discussions. An extensive search of journal articles and other research was compiled and used by the members. For each question, the member’s answers and comments were collected. From these a draft manuscript was prepared that was recirculated multiple times until a consensus of Task Force participants was reached.
The level of evidence supporting each recommendation was assessed using the Grading of Recommendations, Assessment, Development and Assessment (GRADE) criteria. These rate the research used in formulating the guidelines from grade A or high (where further research is thought to be unlikely to change confidence in the estimate of effect) to D or very low (any estimated effect is uncertain).
Task Force Recommendations
Among the recommendations:
- EGPA should be managed in, or in collaboration with centers with established expertise in the management of small- and medium- sized-vessel diseases.
- A minimal initial work up should include at least blood testing for various diseases and a computed-tomography (CT) scan of the chest to rule out other diseases that can mimic EGPA.
- Once diagnosed, physicians should evaluate possible involvement in the heart, lungs, kidney, gastrointestinal tract and peripheral nerves.
- Those with life- or organ-threatening disease should be prescribed a specific medication regimen that includes at least a glucocorticoid (steroid) and another medication that suppresses immunity.
- Certain medications can be considered for second- or third-line therapies if the original regimen did not work, the side effects are great, or pregnancy might be involved.
- Vaccinations with inactivated vaccines against influenza and pneumonia should be encouraged. Live vaccines are not indicated in patients taking immune system suppressants or high dose prednisone.
- Implementing patient education programs were encouraged to begin enhancing the patient’s knowledge of the disease, hopefully leading to earlier recognition of disease flares.
- Patients should be encouraged to avoid tobacco and other irritants as they can trigger asthma flares and further reduce lung function.
The authors stress that these are not definitive treatment guidelines but reflect consensus statements based on the best currently available knowledge. They only serve to give doctors tools to individualize patient management and as a starting point for further research.
“Now a days, treatment for ANCA-associated vasculitides is clear for most of them,” said Prof. Guillevin. “This is not true for EGPA, so clinicians have a responsibility to clarify approaches. We noted the necessity to codify investigations and clinical approaches to make sure all are speaking the same language.”
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management.
Groh M, Pagnoux C, Baldini C, Bel E, Bottero P, Cottin V, Dalhoff K, Dunogué B, Gross W, Holle J, Humbert M, Jayne D, Jennette JC, Lazor R, Mahr A, Merkel PA, Mouthon L, Sinico RA, Specks U, Vaglio A, Wechsler ME, Cordier JF, Guillevin L.
Eur J Intern Med. 2015 Sep;26(7):545-53. doi: 10.1016/j.ejim.2015.04.022.