Pathogenesis and pathology of anti-neutrophil cytoplasmic antibody（ANCA）-associated vasculitis

Genetic aspects of anti-neutrophil cytoplasmic antibody-associated vasculitis.

The genetics of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a complex area of investigation because of the low frequency of AAVs, the rarity of familial cases and the complexity of disease phenotypes. However, recent studies have been able to gather significant numbers of patients, and multicentre collaborative efforts have allowed the performance of two genome-wi...

Anti-neutrophil cytoplasmic antibodies and vasculitis.

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis: where to go?

Enormous progress has been made during the last 25 years in our understanding of the aetiopathogenesis of the anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides (AAV). This has led to improvements in early diagnosis, treatment and secondary prevention of these diseases. Nevertheless, there are still unmet needs in the AAV. With respect to diagnosis and follow-up, sensitive ...

Renal involvement in anti-neutrophil cytoplasmic autoantibody associated vasculitis.

Renal involvement is a common and often severe complication of anti-neutrophil cytoplasmic autoantibody (ANCA) associated vasculitides (AAV). With the exception of Churg-Strauss syndrome (CSS), where kidney involvement is not a prominent feature, renal disease is present in about 70% of patients with Wegener's granulomatosis, now called granulomatosis with polyangiitis (GPA) and in almost 100% ...

Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, Large Vessel Vasculitis and Kawasaki Disease in Japan

Based on studies comparing the prevalence of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) between Japan and Europe, we have learned that the difference may be due to genetic background and environmental factors, but not to diagnosis or ELISA system for myeloperoxidase and proteinase-3 ANCA. In Japan, microscopic polyangiitis is the most common among AAV, but Wegener’s...