Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Anticardiolipin antibodies (aCL) were measured in the sera of patients with different connective tissue diseases and spondyloarthropathies. Elevated antibody binding was found in systemic lupus erythematosus (SLE), rheumatoid arthritis, scleroderma, primary sicca syndrome, dermatopolymyositis and psoriatic arthritis but not in ankylosing spondylitis. The 15 highest binding SLE patients included 10 with Raynaud's phenomenon, 5 with livedo reticularis, 7 with vasculitis, 3 with major thrombotic episodes and 3 with spontaneous abortions. aCL were also measured in patients with these clinical features in isolation. Seven of 18 patients with multiple thromboses and 3/22 with multiple spontaneous abortions had raised aCL binding. Normal or near normal levels were found in patients with idiopathic thrombocytopenia, livedo reticularis, a single cerebral thrombosis and uncomplicated myocardial infarction.
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PMID:Antiphospholipid antibodies in the connective tissue diseases: their relation to the antiphospholipid syndrome and forme fruste disease. 277 57

Two girls, 22 months and 12 years of age, presented with repeated cerebral infarctions in association with primary antiphospholipid syndrome. The younger patient also suffered from protein C deficiency, while the other one had protein S and complement C4 deficiencies. All other causes of cerebral infarction were excluded; however, vasculitis remains a possibility in one patient. Both girls developed spastic tetraparesis as a sequela of the previous infarctions. The two patients were treated with aspirin and prednisone, with remission of the infarctions during the next 8 months of observation. A primary deficiency of protein C or S is proposed which would produce cerebral thrombosis with exposure of phospholipids; this thrombosis then, like antigens, would generate antibodies acting on the thrombin-thrombomodulin complex, exacerbating the thrombotic process. The association of complement C4 deficiency is an additional risk factor.
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PMID:Childhood stroke associated with protein C or S deficiency and primary antiphospholipid syndrome. 845 4

Cerebral thrombotic disease is a rare and nearly always fatal complication of ulcerative colitis. It is associated with a necrotizing vasculitis. We report a fatal case with a confusing neurologic picture arising from this complication. Autopsy revealed necrosis and hemorrhages affecting both cortical grey and white matter. Microscopic examination showed thrombosis of small and medium size vessels associated with hemorrhages and a necrotizing angiitis. Ulcerations, hemorrhages, pseudopolyps, and cryptic abscesses were found in the rectosigmoid region of the colon compatible with active ulcerative colitis. A sudden neurologic deficit in a patient with ulcerative colitis should direct attention to the consideration of a cerebral thrombotic event and the possibility of an associated cerebral vasculitis. Diagnosis may be strongly suggested by MRI or arteriography, but it may require confirmation by biopsy of the brain parenchyma and leptomeninges. A hypercoagulable state has been associated with the thrombosis. Anticoagulation has yielded successful results in some patients with cerebral thrombosis but the risk of massive intracranial and gastrointestinal bleeding preclude to establish clear indications. Neurologic improvement has been obtained with the use of steroids and cyclophosphamide.
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PMID:Cerebral thrombosis and vasculitis: an uncommon complication of ulcerative colitis. 1084 81

Polyarteritis nodosa (PAN) is a vasculitis of small- and medium-sized vessels, characterized by vascular aneurysms that can lead to ischemia and infarction. We present the case of a patient with classic polyarteritis nodosa in abdominal organs who additionally demonstrated antitreponemal and antiphospholipid antibodies, resulting in a severe cerebral thrombosis. This case highlights: 1. The potential for false-positive syphilis serology in PAN patients, and 2. The rare coexistence of polyarteritis nodosa and the catastrophic antiphospholipid syndrome.
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PMID:Polyarteritis nodosa complicated by catastrophic antiphospholipid syndrome. 1704 11