Primary systemic vasculitis: clinical features and mortality

Primary systemic vasculitis: clinical features and mortality.

BACKGROUND Wegener's granulomatosis (WG), Churg Strauss syndrome (CSS) and microscopic polyangiitis (MPA) are primary systemic vasculitides (PSV), the clinical features of which have been described from tertiary centres. AIM To provide the first clinical description of MPA from a general hospital and compare clinical features with WG and CSS. DESIGN Retrospective analysis of patient records...

Clinical and immunological features of retinal vasculitis in systemic diseases.

BACKGROUND/AIM Vasculitis is a clinical, pathologic process characterized by inflammation and necrosis of blood vessel occuring anywhere in the body. The aim of the study was to present some clinical and immunologic features of retinal vasculitis in systemic diseases: systemic lupus erythematosus, multiple sclerosis, sarcoidosis, Behcet's diseases, and others. METHODS A total of 1 254 peatien...

Mortality in systemic vasculitis: a systematic review.

There has been a considerable improvement in the survival of patients with systemic vasculitis since the introduction of immunosuppressive therapy and improved diagnostic tools to allow earlier diagnosis. We review the published literature on current risk of mortality in patients with small vessel antineutrophil cytoplasm antibody- (ANCA) associated vasculitis including Wegener's granulomatosis...

Studies on the Epidemiology and Outcome of Primary Systemic Vasculitis

Clinical features of anti-neutrophil cytoplasmic antibody-associated systemic vasculitis in French children

Results Forty-seven children were included, 15 (32%) with WG and 32 (68%) with MPA, 77% children were pANCA +/MPO. Female predominated (85%), the median age at diagnosis was 10 years (2-16), and average time to diagnosis was 8 months (0-58). 53% children presented with fever, 77% with deterioration of general condition. 91% had renal involvement. Among them 91% had proteinuria, 44% had hyperten...