ANCA-Associated Vasculitis in Patient with CREST-Syndrome - Case Report

Background: ANCA-associated vasculitis is a small vessel necrotizing vasculitis with few or no immune deposits, necrotizing glomerulonephritis is very common in the microscopic poliangiitis subset. Systemic scleroderma renal involvement is included neither in the current classification criteria, nor in the definition of CREST-syndrome. The presence of ANCA in patients with SS was first described in 1996, and since that has been reported to be rare and not presenting clinical significance. However during next decade about 50 cases with association of SS and AAV were described, and more recently, additional cases were reported and the clinical features of such association were recognized.