BACKGROUND Microscopic polyangiitis is characterized by pauci-immune, necrotizing small-vessel vasculitis and an anti-neutrophil cytoplasmic antibody-associated vasculitis. Although gastrointestinal involvement in microscopic polyangiitis is not rare, endoscopic observation of it is extremely rare. To the best of our knowledge, this is the first case report of small intestinal involvement in mi...

We report a case of a 71-year-old man with rapidly progressive nephritic syndrome and dual positivity for anti-glomerular basement membrane antibody and myeloperoxidase-specific anti-neutrophil cytoplasmic antibody. Renal biopsy revealed crescentic, mainly cellular, glomerulonephritis with granulomatous lesions, and advanced membranous changes. Membranous nephropathy had apparently existed for ...

IgG4-related lung disease (IgG4-RLD) is a rare and chronic progressive autoimmune disease. We report a case of IgG4-related inflammatory pseudo-tumor of the lung that was seropositive for proteinase 3 anti-neutrophil cytoplasmic antibody (PR3-ANCA). A 61-year-old male had a mass lesion in the right lower lung field in chest X-ray. Transbronchial lung biopsy resulted in a pathological diagnosis ...

Correspondence to: Albert Young Abstract: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis are devastating diseases with high mortality if left untreated. Combined corticosteroid and cyclophosphamide is an effective treatment but it also carries high risks of adverse events and high relapse rates. Numerous studies endeavor to explore alternative agents. This review aims to exam...

Goodpasture's syndrome is a rare autoimmune disorder characterized by rapidly progressive glomerulonephritis (RPGN) and alveolar hemorrhage in the presence of antiglomerular basement membrane (anti-GBM) antibodies. Central nervous system involvement is highly unusual in the absence of anti-neutrophil cytoplasmic antibodies. We report the case of a 20-year-old man with RPGN accompanied by bloody...

We herein report a rare case of microscopic polyangiitis with primary biliary cirrhosis (PBC) and a literature review of six previously reported cases of PBC complicated by anti-neutrophil cytoplasmic antibody-associated vasculitis. Due to the scarcity of similar reports, it was not possible to establish a true overlap syndrome or casual association. When the biliary enzyme levels are elevated ...

A 58-year-old man with a 15-year history of idiopathic pulmonary fibrosis was hospitalized for rapid progression of muscle weakness to bilateral foot drop. Although laboratory data revealed high titers of myeloperoxidase anti-neutrophil cytoplasmic antibody (489 EU), the patient was diagnosed as polyarteritis nodosa limited to the lower portions of the legs. Despite of the treatment with large ...

Polyarteritis nodosa (PAN) is the systemic necrotizing vasculitis on small and median muscular vessels. Recent studies reveal that PAN consists of classical PAN and microscopic polyangitis (MPA). MPO-ANCA, which is one of anti-neutrophil cytoplasmic antibody (ANCA), is involved in the pathogenesis of MPA. In future, by the progress of research of ANCA, the definition or criteria of systemic vas...

Introduction Microscopic polyangitiis is characterized by anti-neutrophil cytoplasmic antibodies (ANCA) and small vessel vasculitis. The diseases usually respond to cyclophosphamide (Cyc) but some patients are therapy resistant. Rituximab (RIT) is a chimeric antibody to CD20 causing lysis of B-lymphocytes and used for treatment of lymphomas. RIT has also been successfully used in patients with ...

a. Serum anti-neutrophil cytoplasmic antibody (ANCA) measurement should not be used alone in the initial diagnosis of ANCA-associated vasculitis (AAV) but should be used in combination with the gold standard of tissue diagnosis. (Level 1 evidence) b. Measurement of ANCA by both ELISA and indirect immunofluorescence (IIF) in combination ensures optimal test sensitivity and specificity. (Level I ...