It is six years since a leader in the Annals reviewed the performance of antineutrophil cytoplasmic antibodies (ANCA) in clinical practice.' At that time there was increasing confidence in the value of ANCA testing in the diagnosis ofWegener's granulomatosis and related primary vasculitides. Sensitivity of cANCA for Wegener's granulo-matosis was reported as 500/o-96% and specificity was 900/o-1...

BACKGROUND Antineutrophil cytoplasmic antibody (ANCA)-associated crescentic glomerulonephritis (CGN) is a major cause of rapidly progressive glomerulonephritis (RPGN). ANCA-associated CGN is generally classified into pauci-immune RPGN, in which there are few or no immune complexes. CASE PRESENTATION A 78-year-old man presented with RPGN after a 7-year course of chronic proteinuria and hematur...

Antineutrophil cytoplasmic antibodies (ANCA) are the serological hallmark of some idiopathic systemic vasculitides. Besides the investigation of ANCA-associated vasculitis (AAV) and constant effort for a standardized nomenclature and classification of the AAV, a main focus of research during the last few years has been to constantly improve the performance of enzyme immunoassays. With the lates...

Background: Antineutrophil cytoplasmic autoantibodies (ANCA) are serological markers of ANCA associated systemic vasculitides (AASV), which is one of the most common multisystem autoimmune diseases. Features of Chinese patients with AASV have not been fully investigated. Objective: To analyse the clinical and pathological characteristics of Chinese patients with AASV. Methods: 426 Chinese patie...

Our previous study demonstrated that plasma levels of complement factor H (FH) were inversely associated with the disease activity of patients with anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV). In addition to serving as an inhibitor of the alternative complement pathway, there is increasing evidence demonstrating direct regulatory roles of FH on several cell types...

BACKGROUND Antineutrophil cytoplasmic autoantibodies (ANCA) are serological markers of ANCA associated systemic vasculitides (AASV), which is one of the most common multisystem autoimmune diseases. Features of Chinese patients with AASV have not been fully investigated. OBJECTIVE To analyse the clinical and pathological characteristics of Chinese patients with AASV. METHODS 426 Chinese pati...

BACKGROUND ANCA is detected in several vasculitic diseases, including drug-induced systemic vasculitis: propylthiouracil (PTU), hydralazine, minocycline, penicillamine, allopurinol, procainamide, carbimazole, thiamazole, clozapine and phenytoin. All have been known to induce ANCA positive vasculitis in adult patients. OBJECTIVE To study the clinical manifestation, renal pathology and outcome ...

Microscopic polyangiitis (MPA) is a rare systemic vasculitis with an incidence of about 1–3/100,000/year [1]. MPA is defined as a necrotizing vasculitis, with few or no immune deposits, primarily affecting small vessels including capillaries, venules or arterioles [2]. MPA mainly affects kidneys and lungs, nevertheless cutaneous vasculitis, musculoskeletal symptoms, gastrointestinal involvement...

Both the innate and the acquired immune system are involved in the pathophysiology of renal vasculitis. However, anti-neutrophil cytoplasmic antibody (ANCA)-associated renal vasculitis is characterized by a 'pauci-immune' pattern of immunofluorescence during kidney biopsy, indicating the relative lack of immunoglobulin and complement deposition within the kidney. On the other hand, evidence is ...

BACKGROUND IgG4-related disease (IgG4-RD) is a fibroinflammatory condition characterized by serum IgG4 elevation and tissue infiltration of IgG4-positive plasma cells. Substantial overlap between IgG4-RD and antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAV) exists in terms of organ involvement and histopathological features. A positive ANCA assay is regarded as a highly...