Wegener's granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome are small- to medium-vessel vasculitides linked by overlapping pathology and the presence of antineutrophil cytoplasmic antibodies (ANCA). Commonly referred to as the ANCA-associated vasculitides, these diseases are challenging to diagnose and to treat. Distinguishing the ANCA-associated vasculitides from other forms...

PURPOSE OF REVIEW The primary idiopathic small-vessel vasculitis syndromes include granulomatosis with polyangiitis, Churg-Strauss syndrome, and microscopic polyangiitis. These disorders are commonly referred to as antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides and prominently affect the pulmonary vasculature. Although significant progress has been made in the management of ...

Antibodies to neutrophil cytoplasmic antigens (ANCA) are good serological markers for patients with mainly vasculitic conditions. Two main types of ANCAs have been detected, the first termed cytoplasmic antineutrophil cytoplasmic antibody (cANCA) are mainly associated with patients with Wegener's granulomatosis, the other termed perinuclear antineutrophil cytoplasmic antibody (pANCA) are mainly...

FOR RELATED ARTICLE, SEE PAGE 2334Ethnically diverse case series and cohort studies have reported the association of interstitial lung disease (ILD) microscopic polyangiitis (MPA), an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis.1Tzelepis G.E. Kokosi M. Tzioufas A. et al.Prevalence outcome pulmonary fibrosis in polyangiitis.Eur Respir J. 2010; 36: 116-121Crossref PubMed Scop...