[Granulomatosis with polyangiitis - clinical picture and review of current treatment options].

[Granulomatosis with polyangiitis - clinical picture and review of current treatment options]. Pneumonol Alergol Pol. 2014;82(1):61-73 Authors: Lembicz M, Batura-Gabryel H, Nowicka A Abstract Granulomatosis with polyangiitis (GPA) is a rare disease of unknown aetiology, characterized by necrotizing inflammation of small and medium-sized vessels and formation of granulomas. The disease typically involves the upper and lower respiratory tracts and the kidneys, but it can occur in many other locations, often giving a surprising clinical picture. The majority of patients have autoantibodies against cytoplasm of neutrophils (ANCA), of which a pathogenetic role is postulated. The disease affects mostly middle-aged and elderly people, with a predominance of Caucasians. Before the era of immunosuppressive therapy GPA was associated with high early mortality. Nowadays, we have a treatment that gives remission in the majority of patients. The decision about the method of treatment should be made depending on the clinical form of the disease (local or generalized, refractory). Standard remission induction therapy in the generalized disease is cyclophosphamide in combination with steroids. This therapy, however effective, is associated with a number of side effects. In addition, some patients are resistant to standard therapy, and half of them experience relapses. Therefore, there is need for further research for alternative treatment and maintenance ther...
Source: Pneumonologia i Alergologia Polska - Category: Respiratory Medicine Authors: Tags: Pneumonol Alergol Pol Source Type: research