ANCA-Associated Vasculitides: Future Therapeutic Strategies

Are ANCA-associated vasculitides inherited?

Therapeutic Complement Targeting in ANCA-Associated Vasculitides and Thrombotic Microangiopathy

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) are a group of systemic autoimmune disorders characterized by necrotizing inflammation of medium-tosmall vessels, a relative paucity of immune deposits, and an association with detectable circulating ANCAs. AAVs include granulomatosis with polyangiitis (renamed from Wegener’s granulomatosis), microscopic polyangiitis, an...

ANCA-Associated Vasculitides—An Update

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides are characterized by destruction of small vessels, granulomatous inflammation of the respiratory tract and necrotizing glomerulonephritis. This review describes the clinical diagnosis and therapy as well as the pathophysiology of ANCA-associated vasculitides with a specific focus on the interplay of ANCAs with activated neutrop...

ANCA-associated vasculitides in childhood

Methods and result 14-year-old patient diagnosed with TGA at birth, at the age of 14 she presented a sudden hepatosplenomegaly. The systematic examination confirmed WG. According to our knowledge this is the first case of association of these two infrequent diseases. Furthermore hepatosplenomegaly as initial symptom of WG is especially rare feature. The second patient suffering from asthma for ...

Pathophysiology of ANCA-associated vasculitides: are ANCA really pathogenic?

The strong relation between antineutrophil cytoplasmic autoantibodies (ANCA) and primary vasculitic syndromes suggests a pathophysiological role for ANCA. Experimental evidence for the pathogenic potential of ANCA has been derived from in vitro studies that demonstrate that ANCA can activate tumour necrosis factor alpha primed neutrophils, monocytes and/or endothelial cells. The binding of ANCA...