Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0039483 (giant cell arteritis)
 3,204 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Autoantibodies against neutrophil cytoplasmic antigens (ANCA) produce two major immunofluorescence (IF) patterns on ethanol-fixed granulocytes: the "classical" (centrally accentuated) C-ANCA, associated with Wegener's granulomatosis (WG), and P-ANCA (perinuclear), which mainly occur in renal vasculitis. Rheumatic manifestations are an important clinical finding in systemic vasculitis, often preceding a fulminant course and sometimes imitating various rheumatic disorders. We analyzed the incidence of ANCA in rheumatic patients and looked for the frequency of rheumatic symptoms in systemic vasculitis. In WG (n = 186), we found rheumatic symptoms in 55% (myalgia, 45%; arthritis, 21%); in 90%, rheumatic complaints were associated with active vasculitis. In 730 patients with various rheumatic conditions (eg, 268 rheumatoid arthritis, 130 systemic lupus erythematosis [SLE], 32 sharp-S, 50 ankylosing spondylitis, 43 systemic sclerosis) no C-ANCA were found. On the contrary, the P-ANCA pattern was seen in seven of 62 giant cell arteritis, five of 27 Felty's/Still's syndrome, and four of 130 SLE patients in addition to renal vasculitis (21/74). We demonstrated that 95% of C-ANCA-positive sera react with proteinase 3 (PR3 or myeloblastin). Using monoclonal antibodies, we showed that PR3 is expressed on the plasma membrane of neutrophil granulocytes and monocytes; thus, PR3 autoantigens are accessible for circulating antibodies. The detection of ANCA in sera from vasculitis and other rheumatic diseases is of immunodiagnostic value and provides new insight in the pathogenesis of systemic vasculitides.
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PMID:Antineutrophil cytoplasmic autoantibody-associated diseases: a rheumatologist's perspective. 186 75

Antineutrophil cytoplasmic antibodies (ANCA) were demonstrated in all sera from 11 patients with active giant cell arteritis (GCA) using indirect immunofluorescence on 9% paraformaldehyde(PF)-fixed neutrophils according to Pryzwanski (median titer 1:256, range 1:64 to 1:512). After treatment during inactive disease titers decreased in all sera. Eight out of 9 sera from patients with active polymyalgia rheumatica (PMR) produced a cytoplasmic staining on Pryzwanski-fixed neutrophils in low titers (median titer 1:16, range 0 to 1:32), as did 8 out of 25 sera from healthy blood donors. None of the sera were positive for antibodies to defined antigens, i.e. proteinase-3, human leucocyte elastase, myeloperoxidase and lactoferrin as detected by ELISA. GCA seems to be associated with ANCA of as yet unknown specificity.
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PMID:Anti-neutrophil cytoplasmic antibodies in giant cell arteritis and polymyalgia rheumatica. 829 36

Pulmonary involvement is rare in giant cell arteritis (GCA). Only a few cases have been reported, manifested by interstitial infiltration, pulmonary nodules, pulmonary artery vasculitis, and granuloma formation. Moreover, only 3 previous cases of pleural effusion associated with GCA have been described. Herein we report a 67-year-old woman with biopsy-proven temporal arteritis, presented with prolonged fever, weight loss, cough and pleural effusion. ELISA test for the presence of anti- proteinase-3 antibodies was negative. The importance of the anti-neutrophil cytoplasmic-antibodies (ANCA) examination in the differential diagnosis from other vasculitides with pulmonary involvement is discussed.
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PMID:Pleural effusion as a presenting manifestation of giant cell arteritis. 877 58

Establishing a diagnosis of systemic rheumatic disease requires an integration of a patient's symptoms, physical examination findings, and the results of diagnostic testing. There is often a temptation by clinicians to rely heavily on objective measures such as the presence or absence of an autoantibody. Medical textbooks and the medical literature may overestimate the diagnostic utility of many commonly ordered tests for rheumatic disease because the tests are usually analyzed among patients with established rheumatic disease rather than among patients with an uncertain cause of symptoms as is common in practice. Few diagnostic tests are highly sensitive, though the antinuclear antibody in systemic lupus erythematosus (SLE) and the erythrocyte sedimentation rate in temporal arteritis are notable exceptions. Conversely, few diagnostic tests are highly specific; anti-proteinase-3 and antimyeloperoxidase antibodies (types of antineutrophilic cytoplasmic antibodies) among patients with Wegener granulomatosis (and related vasculitides) and anti-double-stranded and anti-Smith antibodies among patients with SLE may be particularly helpful in the proper clinical settings due to their high specificity. Anticitrullinated cyclic protein (anti-CCP), a newly described autoantibody that may be highly specific for rheumatoid arthritis, requires additional study as its utility in clinical practice is uncertain.
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PMID:Diagnostic tests for rheumatic disease: clinical utility revisited. 1610 39

Based on studies comparing the prevalence of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) between Japan and Europe, we have learned that the difference may be due to genetic background and environmental factors, but not to diagnosis or ELISA system for myeloperoxidase and proteinase-3 ANCA. In Japan, microscopic polyangiitis is the most common among AAV, but Wegener's granulomatosis was present in less than 2 per million patients. Also, one study from Hokkaido reported only 16 patients in a 27-year time frame. A recent retrospective study of renal vasculitis between 2000 and 2004 from Miyazaki prefecture in Japan reported an incidence of microscopic polyangiitis of 14.8 per million, but no patients with Wegener's granulomatosis or Churg-Strauss syndrome. In the present review, we focus on ANCA-related vasculitis in Japan: (1) AAV and large vessel vasculitis - Takayasu's arteritis and giant cell arteritis; (2) primary renal vasculitis; (3) epitopes of myeloperoxidase-ANCA in vasculitis in the Japanese population and comparison of ANCA-ELISA systems in Japan and Europe, and finally (4) children with vasculitis in Japan involving Kawasaki disease - a systemic vasculitis.
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PMID:Anti-neutrophil cytoplasmic antibody-associated vasculitis, large vessel vasculitis and Kawasaki disease in Japan. 2107 54

BACKGROUND Systemic vasculitis can present with a multitude of symptoms involving multiple organ systems. Clinicians should avoid anchoring bias and be cognizant that different types of vasculitides can be present in the same patient and that the diagnosis of one should not preclude the subsequent diagnosis of another. CASE REPORT A 67-year-old woman was referred for evaluation of episodes of epistaxis and recurrent severe sinusitis. Her physical examination showed nasal congestion and purpuric rash on the lower extremities. CT of the sinuses showed severe mucosal thickening. ANCA serologies were positive with a c-ANCA titer of 1: 5120 and anti-proteinase-3 (anti-PR3) antibodies of 1061 units. Serum creatinine was elevated at 1.32 mg/dL (GFR of 40.62 ml/min). Urine analysis showed proteinuria and hematuria. The patient declined treatment initially, but while awaiting kidney biopsy she developed episodes of headache and blurry vision. She underwent right temporal artery biopsy 4 days later, which confirmed the diagnosis of GCA. The biopsy showed characteristic histopathology findings and she was started on 60 mg of prednisone daily. The kidney biopsy showed pauci-immune crescentic glomerulonephritis (PICGN) consistent with ANCA-associated vasculitis. We identified all the cases of co-presentation of GCA and GPA in the literature and summarized their clinical features in this report. CONCLUSIONS Astute clinicians should be cognizant of overlapping and atypical presentations of vasculitides to avoid delayed diagnosis and errors in management.
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PMID:Co-Presentation of Giant Cell Arteritis and Granulomatosis with Polyangiitis: A Case Report and Review of Literature. 2987 33