What is the difference between limited and severe granulomatosis with polyangiitis (GPA, formerly Wegener’s)?

Not all patients with granulomatosis with polyangiitis (GPA) have similar disease. The term “limited” GPA was introduced in 1966 and was originally defined as GPA that did not involve the kidneys. Unfortunately, this term has often been misinterpreted. “Limited” GPA is not equivalent to non-severe GPA as there are people who meet the definition of “limited” disease who may have very severe disease and people who do not meet the definition of limited GPA whose disease may be quite mild. Because of this, the term “limited” GPA is best avoided. It is far more helpful to view this disease in terms of the location of organ involvement and the degree of severity of the disease in each of these locations.

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