On rare occasions, patients with Goodpasture’s syndrome (GPS) present both anti-glomerular basement membrane (GBM) and antineutrophil cytoplasmic antibody (ANCA). Concurrent ANCA anti-GBM disease is a high mortality rate. Here we case of (ANCA) complicated hyponatremia.

While antineutrophil cytoplasmic antibody (ANCA) is often used as a diagnostic marker for certain vasculitides, ANCA induction in the setting of infection is much less common. In the case of infective endocarditis, patients may present with multisystem disturbances resembling an autoimmune process, cases that may be rendered even trickier to diagnose in the face of a positive ANCA. Though not a...

In this issue of the American Journal of Clinical Pathology, Gaudin and colleagues report on their investigation of the spectrum of pulmonary lesions associated with anti-neutrophil cytoplasmic autoantibodies (ANCA). As various investigations have previously detailed, ANCAs are actually a heterogeneous collection of autoantibodies, which are for the most part directed at neutrophil granule enzy...

Atypical, cytoplasmic and perinuclear anti-neutrophil cytoplasmic antibodies (x-, c- and pANCA, respectively) are associated with a variety of inflammatory diseases, including inflammatory bowel disease (IBD). Anti-neutrophil cytoplasmic antibodies are more common in patients with ulcerative colitis (UC) than in patients with Crohn's disease (CD). Most publications only refer to p- and cANCA in...

Anti-neutrophil cytoplasmic autoantibodies (ANCA) associated with active Wegener's granulomatosis are directed against a soluble 29-Kd protein present in human neutrophils and monocytes. Affinity labeling with tritiated diisopropylfluorophosphate (3H-DFP) suggested that ANCA-antigen is a serine protease. We used immunoelectron microscopy to study the in situ localization of the ANCA-antigen in ...

proteinase 3(pr3) is a human polymorphonuclear leukocyte serine proteinase and is the main target antigen for antineutrophil cytoplasmic antibodies (anca) found in wegener's granulomatosis (wg). we developed a stable expression system for conformationally intact recombinant pr3 (rpr3) in chinese hamster ovary cells (cho-cells). the part of pr3 cdna that encoded the active form of pr3 was s...

We investigated the presence of anti-neutrophil cytoplasmic antibodies (ANCA) in the serum and bronchoalveolar lavage fluid (BALF) of 21 cystic fibrosis (CF), 7 idiopathic bronchiectasis (IBR), and 11 control children and the relation between ANCA and any bacteria grown in BALF. Six of the CFs, but none of the IBRs or controls had positive serum cytoplasmic or perinuclear-ANCA (c-ANCA, p-ANCA)....

There is evidence that there is an association between anti-glomerular basement membrane (anti-GBM) antibodies and anti-neutrophil cytoplasmic antibodies (ANCA) in the development of pulmonary renal syndrome (PRS) [1–7]. Whether these two distinct circulating auto-antibodies have some pathological interaction remains unclear. Studies have shown that 30% of patients with anti-GBM disease will te...

Antineutrophil cytoplasmic autoantibody (ANCA) causes vascular injury that leads to small-vessel vasculitis. Patients with ANCA aberrantly express neutrophil granule-encoding genes, including 2 that encode autoantigens: proteinase 3 (PR3) and myeloperoxidase (MPO). To uncover a potential transcriptional regulatory mechanism for PR3 and MPO disrupted in patients with ANCA vasculitis, we examined...

Background: Previous studies have shown considerable variation in diagnostic performance of enzyme linked immunosorbent assays (ELISAs) for measuring antineutrophil cytoplasmic antibodies (ANCA) specific for proteinase 3 (PR3) and myeloperoxidase (MPO). Objective: To analyse the performance characteristics of different commercially available direct ANCA ELISA kits. Methods: ELISA kits for detec...