[Diagnosis and management of small vessel vasculitides]

The spectrum of small vessel vasculitides includes Wegener's granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome, Henoch-Schönlein purpura, cutaneous leukocytoclastic vasculitis and essential cryoglobulinemic vasculitis. The first three are characterized by the presence of circulating anti-neutrophil cytoplasmic antibodies (ANCA). The symptoms of vasculitis range from stable or slowly progressive to rapidly progressive glomerulonephritis or alveolar haemorrhage. The diagnosis of small vessel vasculitis should preferably rely on both clinical findings and histopathological examination of the organ involved. Cyclophosphamide combined with glucocorticoids is still standard therapy for remission induction in generalized ANCA-associated vasculitis. In severe cases the use of plasmapheresis treatment has been advocated. Medications suitable for remission maintenance include azathioprine, methotrexate, leflunomide and mycophenolate mofetil. Early experience with biologic drugs, particularly with rituximab, for refractory disease has been quite promising.