Antineutrophil cytoplasmic antibodies (ANCAs) constitute a new class of autoantibodies that seem to recognise myeloid lysosomal enzymes. The occurrence of ANCAs with specificity for human leucocyte elastase (HLE) was assessed in serum samples that were routinely submitted for ANCA determination. During a study period of more than six years anti-HLE was found in only six out of 1102 serum sample...

Teaching NeuroImages: Myeloperoxidase–anti-neutrophil cytoplasmic antibody–positive hypertrophic pachymeningitis A woman in her 40s presents with painless monocular vision loss and is found to have optic perineuritis and dural thickening. Comprehensive laboratory testing,MRI of the brain, and dural biopsy reveal a diagnosis of myeloperoxidase–antineutrophil cytoplasmic antibody–positive hypertr...

It was with much interest that we read the letter of Novikov et al on testing for antineutrophil cytoplasmic antibodies (ANCAs) in patients with ANCA-associated vasculitides (AAV) and other diseases. In their letter, the authors (1) share their experience with direct testing for proteinase-3 (PR3) ANCA and myeloperoxidase (MPO) ANCA and (2) raise some important issues regarding interpretation o...

PURPOSE OF REVIEW The primary idiopathic small-vessel vasculitis syndromes include granulomatosis with polyangiitis, Churg-Strauss syndrome, and microscopic polyangiitis. These disorders are commonly referred to as antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides and prominently affect the pulmonary vasculature. Although significant progress has been made in the management of ...

Aretrospective investigation was conducted by members of the Japanese Society for Pediatric Nephrology from 1990 to 1997 to define the clinical features and outcome of antineutrophil cytoplasmic autoantibody (ANCA)-associated glomerulonephritis in children. Thirty-four ANCA-seropositive Japanese pediatric patients with biopsy-proven pauci-immune necrotizing crescentic glomerulonephritis were id...

We determined the occurrence of antineutrophil cytoplasmic antibodies (ANCAs) and their specificities in 77 rheumatoid arthritis (RA) patients and compared them with 25 patients with psoriatic arthritis (Pso), 19 with drug-induced lupus erythematosus (DI-LE) and 11 with systemic lupus erythematosus (SLE). Thirty-two percent of RA patients had positive indirect immunofluorescence (IIF) stains (P...

Wegener's granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome are small- to medium-vessel vasculitides linked by overlapping pathology and the presence of antineutrophil cytoplasmic antibodies (ANCA). Commonly referred to as the ANCA-associated vasculitides, these diseases are challenging to diagnose and to treat. Distinguishing the ANCA-associated vasculitides from other forms...

It was with great interest that we read the correspondence of Mahler and Fritzler on our recent European Vasculitis Study Group (EUVAS) study describing the performance of immunoassays for antineutrophil cytoplasmic antibodies (ANCA) in patients with ANCA-associated vasculitides (AAV). In their letter, Mahler and Fritzler raise some interesting points, mainly related to (i) test result interpre...

The pathogenic role of antineutrophil cytoplasmic autoantibodies (ANCA) remains controversial because of the difficulty in explaining how extracellular ANCA can interact with intracellular primary granule constituents. It has been postulated that cytokine priming of neutrophils (PMN), as may occur during a prodromal infection, is an important trigger for mobilization of granules to the cell sur...

BACKGROUND Churg-Strauss syndrome (CSS), also known as allergic granulomatous angiitis, is a rare entity that is characterized by systemic vasculitis in patients with a history of asthma. Patients with CSS show a marked peripheral blood eosinophilia, but the pathogenesis remains unknown. OBSERVATIONS A retrospective review was performed in 9 cases of CSS in whom cutaneous findings were presen...