Background Granulomatosis with Polyangiitis or Wegener’s granulomatosis (WG) is a necrotizing vasculitis of unknown etiology that involves small and medium calibre vessels. It is associated to anti-neutrophil cytoplasm antibodies (ANCA). It most often affects the respiratory tract and the kidneys and its most important pathologic feature is the presence of necrotizing granulomas.
Objectives To detail the features of 21 patients with WG diagnosed in a university department of Internal Medicine.
Methods Retrospective study: between 2000 and 2012, 21 patients with WG were diagnosed in our centre. The WG diagnosis was retained according to ACR criteria (1990). Epidemiological, clinical, laboratory tests as well as pathologic studies and treatment were retrospectively analyzed.
Results Twenty-one patients were diagnosed: 14 men and 7 women. Mean age at diagnosis: 45.5 years (16–70). Mean delay to diagnosis: 28 months. Fourteen patients had general symptoms (66%), 18 ear-nose-throat symptoms (85.7%), 16 pulmonary involvement (76%), 12 renal involvement (57%), 2 ureteral stenosis (9.5%), 11 neurological involvement (52.4%), 4 arthromyalgia (19%), 12 mucocutaneous manifestations (57%), 9 ocular involvement (43%), 5 cardiac involvement (24%), 4 vascular involvement (19%), 2 gastrointestinal involvement (9.5%). In one case, we report an association to a retroperitoneal fibrosis. ANCA were positive in 16 cases (76%), all had a cytoplasmic c-ANCA pattern. Anti-protease-3 specificity was detected in 5 cases and anti-myeloperoxydase in 3 cases. All patients received intravenous glucocorticoids and cyclophosphamide as induction therapy. During the disease progression 3 patients died, 9 patients progressed to chronic renal failure and hemodialysis. The remaining patients presented several relapses.
Conclusions The clinical features of this series do not differ from those described by other authors, except the younger age at diagnosis. Frequently used drugs were glucocorticoids and cyclophosphamide. The course was usually unfavourable, with outbreaks or complications due to immunosuppression, except for those with limited forms. Immunosuppressive therapy should be maintained indefinitely in most cases.
Disclosure of Interest None Declared
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