Primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH) are enigmatic chronic inflammatory diseases of the liver, which are frequently associated with chronic inflammatory bowel diseases. Both types of liver disease share various distinct autoantibodies such as atypical perinuclear antineutrophil cytoplasmic antibodies (p-ANCA), and thus are considered autoimmune disorders with atyp...

AIMS An association between inflammatory bowel disease (IBD) and spondyloarthropathies (SpA) has repeatedly been reported. The aim of the present study was to investigate whether serologic markers of IBD, e.g. antibodies against Saccharomyces cerevisiae (ASCA), antibodies against exocrine pancreas (PAB) and perinuclear antineutrophil cytoplasmic antibodies (pANCA) are present in HLA-B27-associa...

Antineutrophil cytoplasmic autoantibodies (ANCAs) recognizing human proteinase 3 of neutrophil granules are a diagnostic hallmark of Wegener granulomatosis, an autoimmune systemic vasculitis with predilection for the respiratory tract and kidneys. In vitro experiments have implicated several mechanisms by which ANCAs may lead to tissue injury. However, little is known about the pathogenic signi...

Antineutrophil cytoplasmic autoantibodies (ANCA) have been described in sera of patients with several forms of systemic vasculitis, including Wegener‘s granulomatosis and microscopic polyarteritis. The two main targets of ANCA in vasculitis are proteinase 3 (PR31 and myeloperoxidase (MW). ANCA are capable of activating neutrophils primed by tumor necrosis factor-a (TNF-a) in vitro, which...

INCE THE ORIGINAL description of the clinical releS vance of antineutrophil cytoplasmic antibodies (ANCA) testing for patients with vasculitis and glomerulonephritis, antibodies directed against different enzymes in granulocyte granules have been the subject of many ongoing studies.' Although the state of the art has been reviewed previously,2" new findings over the last 2 years reflect the wid...

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...

In recent years there have been substantial developments in the understanding of the aetiology and pathogenesis of ANCA-associated vasculitides, including myeloperoxidase (MPO) associated autoimmunity. This review will describe genetic and environmental factors that may increase the risk for the disease and will summarise findings demonstrating that T-cells, B-cells and ANCA themselves are of p...