041. ANCA ASSOCIATED VASCULITIS IN SYSTEMIC SCLEROSIS

ANCA positive vasculitis in systemic sclerosis

Treatment of ANCA-associated systemic vasculitis.

The antineutrophil cytoplasmic antibodies (ANCA)-associated small vessel vasculitides include Wegener's granulomatosis, Churg-Strauss syndrome, microscopic polyangiitis (MPA), and the renal limited form of MPA, also known as pauci-immune or idiopathic crescentic glomerulonephritis. ANCA are probably necessary but not sufficient for disease pathogenicity. Classical induction and maintenance ther...

ANCA vasculitis in a patient with systemic sclerosis.

The development of vasculitis in patients with systemic sclerosis (SS) is a rare event and association. However, because they share similar clinical manifestations but a different etiopathogenic basis, the need to distinguish between inflammatory vasculitic damage or scleroderma vasculopathy can sometimes be a real dilemma that determines the clinical prognosis and therapeutic regimen for these...

Myeloperoxidase-antineutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis developed from ANCA negative renal limited vasculitis

RATIONALE The relationship between antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) and ANCA-negative vasculitis has not been elucidated. PATIENT CONCERNS A 64-year-old female with edema and proteinuria was admitted. A kidney biopsy indicated focal proliferative nephritis with crescents in 25% of glomeruli. Serum ANCA was negative. Eighteen months later, systemic sym...

Decreased Neutrophil Apoptosis in Quiescent ANCA-Associated Systemic Vasculitis

BACKGROUND ANCA-Associated Systemic Vasculitis (AASV) is characterized by leukocytoclasis, accumulation of unscavenged apoptotic and necrotic neutrophils in perivascular tissues. Dysregulation of neutrophil cell death may contribute directly to the pathogenesis of AASV. METHODS Neutrophils from Healthy Blood Donors (HBD), patients with AASV most in complete remission, Polycythemia Vera (PV), ...