Granulomatosis with polyangiitis in pregnancy - clinical implications and treatment possibilities.

Granulomatosis with polyangiitis (GPA) is an autoimmune disease which has a variable clinical presentation and usually progresses from a localized to a generalized form over the course of weeks to years. Histopathologically, it is a necrotizing systematic vasculitis that can cause sino-nasal, pulmonary, renal, ocular, and cutaneous manifestations. Diagnostic workup should include serologic, radiologic, endoscopic and histopathological examination. Autoantibody c-ANCA may be used as a marker of disease activity and individual follow-up. An appropriate local and systemic treatment should be implemented, which is particularly important in pregnancy. Comprehensive management should be planned, including the needs of both mother and fetus (particularly if vasculitis is diagnosed de novo during pregnancy). Pregnancy in patients with GPA is burdened with the risk of possible complications and increased mortality and the conception should be delayed until remission of the disease. A flare-up of GPA may be life threatening for both mother and fetus. The immunosuppressants, which are used during pregnancy include glucocorticosteroids (GCS) and azathioprine. Studies of GPA in pregnancy are scarce, and this calls for individualized management. Thus, the approach to care for pregnant women with GPA is interdisciplinary, and firmly places the rheumatologist, gynecologist, pulmonologist, otorhinolaryngologist and nephrologist on the management team.