Wegener's granulomatosis, ANCA, and microscopic polyarteritis
Article Abstract:
Wegener's granulomatosis (WG) is a rare and lethal disease that usually occurs in people who are in their 40s or 50s. It causes ulcers in the lungs, excess mucous production in the nasal passages, obstruction of the nasal passages, bleeding in the lungs, coughing up of blood, and kidney damage (glomerulonephritis). Inflammation and damage to blood vessels (vasculitis) in the kidneys and lungs also occurs. The vasculitis is believed to be caused by an immune system disorder in which antibodies that attack proteins in white blood cells (neutrophils) are produced. These antibodies are called anti-neutrophil cytoplasmic antibodies (ANCA). Two different types of ANCA have been identified, based on their location inside the cell. The first type is found in the cytoplasm (the fluid inside the cell), and is called C-ANCA; the second is found in the nucleus (the structure that contains the genetic material), and is called P-ANCA. These two types of ANCA can be used to diagnose the severity of the disease. For example, when the disease occurs only in the kidneys, then the patient will have P-ANCA in their blood. Microscopic polyarteritis is a condition that causes damage to many small blood vessels, and patients with this condition have P-ANCA. When the patient has all of the symptoms of WG, then C-ANCA are present in the blood. In less severe forms of WG, C-ANCA may not be present, indicating that C-ANCA might play a role in causing blood vessel damage. (Consumer Summary produced by Reliance Medical Information, Inc.)
Publication Name: Journal of Pathology
Subject: Health
ISSN: 0022-3417
Year: 1991
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The clinical utility of ANCA positivity
Article Abstract:
Antineutrophil cytoplasmic antibody (ANCA) testing appears to be useful as a secondary test when evaluating patients with confirmed or suspected inflammatory vessel diseases. ANCAs are antibodies directed against normal white blood cells. Increased ANCA levels have been associated with Wegener's granulomatosis, ulcerative colitis, and Henoch-Schonlein purpura in addition to several other non-vessel diseases. This non-specificity makes ANCA testing unsuitable as a screening test. The evidence is inconclusive whether ANCA levels can reliably reflect disease progress in patients with Wegener's granulomatosis.
Publication Name: Annals of the Rheumatic Diseases
Subject: Health
ISSN: 0003-4967
Year: 1996
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Clinical spectrum associated with positive ANCA titres in 94 consecutive patients: is there a relation with PR-3 negative c-ANCA and hypergammaglobulinaemia?
Article Abstract:
Detection of antineutrophil cytoplasmic antibodies (ANCAs) in the blood does not appear strongly associated with Wegener's granulomatosis (WG), and may be indicative of other chronic diseases. WG is a systemic inflammatory vascular condition. Researchers evaluated 94 patients with positive ANCA tests. WG was present in only 40% of patients with cytoplasmic ANCA. WG was present in 85% of patients with anti-proteinase 3 antibodies. Elevated ANCA may be related to hypergammaglobulinemia, an elevation of a blood immune factor seen in some chronic diseases, such as liver cirrhosis and colitis.
Publication Name: Annals of the Rheumatic Diseases
Subject: Health
ISSN: 0003-4967
Year: 1998
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