Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Ten patients with circulating lupus anticoagulant who presented with cutaneous vascular disease and cerebrovascular disease are presented. Cutaneous manifestations were gangrene, thrombophlebitis, ulcers, and livedo reticularis. All 10 patients had cerebral infarction. The relationship between the cerebral and cutaneous vascular changes and the presence of lupus anticoagulant is supported by a common noninflammatory vascular thrombosis histologically in these patients and by the presence of similar pathologic and clinical findings in patients with the lupus anticoagulant syndrome.
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PMID:Cutaneous thrombosis, cerebrovascular thrombosis, and lupus anticoagulant--the Sneddon syndrome. Report of 10 cases. 210 36

Most patients with lupus anticoagulant (LA) activity have coincident antibodies to a group of negatively charged phospholipids, and its is suggested that LA and anticardiolipin tests detect antibodies with overlapping specificities. Some discordance between the two assays has been described, however. One patient presenting with severe thrombotic disease (recurrent deep vein thrombosis, pulmonary embolism, inferior venocaval obstruction, myocardial infarction, and digital gangrene) showed strong LA activity in February 1987. An enzyme linked immunosorbent assay (ELISA) showed no binding to the negatively charged phospholipids cardiolipin, phosphatidylserine, and phosphatidic acid, but binding to zwitterionic phosphatidylethanolamine (PE) was demonstrated. Inhibition studies and affinity purification confirmed this finding. Interestingly, the serum did not bind to the kaolin cephalin clotting time reagent when used as antigen in an ELISA. The pathogenic significance of anti-PE antibodies and their relation to LA remains to be clarified. Further studies of the occurrence of anti-PE antibodies in patients with LA activity who have negative anticardiolipin tests are suggested.
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PMID:Antibody to phosphatidylethanolamine in a patient with lupus anticoagulant and thrombosis. 249 57

This retrospective study of 295 patients extends the earlier findings of an association between lupus anticoagulation and thrombosis by demonstrating the occurrence of cutaneous lesions related to the coagulation defect in 70 patients. The most frequent cutaneous associations were thrombophlebitis, skin ulcer, gangrene, haemorrhage, and cutaneous necrosis. Of the patients with skin lesions, 41% had the skin lesion as the first sign of the disease. It is important to recognize this association because nearly 40% of the patients with skin lesions have multisystem thrombotic phenomena in the course of the disease.
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PMID:Skin lesions associated with circulating lupus anticoagulant. 249 41

Two patients with livedo vasculitis and microthrombosis of the dermal vessels and one patient with symmetric gangrene of both hands are presented. Their only biologic abnormality was the presence of a lupus-type circulating anticoagulant and an increased level of anticardiolipin antibodies. These observations and the few previously reported cases of cutaneous disorders associated with abnormal antiphospholipid activity suggest that the common pathologic process is thrombosis.
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PMID:Thrombotic skin disease as a marker of the anticardiolipin syndrome. Livedo vasculitis and distal gangrene associated with abnormal serum antiphospholipid activity. 250 66

A 77 year old man with digital gangrene of his left hand had anti-phospholipid antibodies, the lupus anticoagulant and antcardiolipin antibodies. This patient, as far as we are aware, is the first elderly person to present in such fashion with this uncommon syndrome.
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PMID:The anti-phospholipid antibody syndrome in a 77 year old man with digital gangrene. 251 30

Recent reviews have suggested a higher frequency of the lupus anticoagulant or related antiphospholipid antibodies in patients with systemic lupus erythematosus (21% to 65%) than was found in earlier studies (6% to 18%). In our study of 60 consecutive patients, we found the frequency of the lupus anticoagulant by Russell viper venom time was 6.7% (95% confidence interval, 16.2 to 1.8) and by anticardiolipin antibody assay was 25% (95% Cl, 37.0 to 15.7), compared with 0% (p = not significant) and 2.5% (p = 0.002), respectively, in the normal control population. The Russell viper venom time (p = 0.0001 by t-test) and anticardiolipin antibody levels (p = 0.01) were significantly associated with presumed thrombotic events (stroke, deep venous thrombosis, and digital gangrene). No association with miscarriage or pulmonary hypertension was detected. The Russell viper venom time was more specific than the anticardiolipin antibody level in the prediction of past presumed thrombotic events, miscarriage, or pulmonary hypertension (100% compared with 84%, p = 0.01).
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PMID:The frequency of lupus anticoagulant in systemic lupus erythematosus. A study of sixty consecutive patients by activated partial thromboplastin time, Russell viper venom time, and anticardiolipin antibody level. 310 10

We reviewed the histopathologic findings in 28 specimens from 25 patients who had skin lesions associated with lupus anticoagulant. The clinical lesions were ulcers, gangrene, thrombophlebitis, hemorrhage, and cutaneous necrosis. Noninflammatory thrombosis of small dermal vessels was observed in all 8 biopsy specimens from gangrene lesions, 10 of 13 specimens from ulcer lesions, and 2 of 5 specimens from thrombophlebitis lesions. Necrotizing vasculitis was not significant in these biopsy specimens. Immunofluorescence findings included a positive lupus band test in lupus erythematosus-associated disease and nonspecific deposits in occasional cases. Focal noninflammatory intravascular coagulation is responsible for the microscopic and clinical skin lesions in these patients.
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PMID:Histopathologic and immunofluorescence study of skin lesions associated with circulating lupus anticoagulant. 231 21

Critical conditions had been established in 21 (23.1%) of 91 patients with systemic connective tissue diseases for a 12 year period: renal failure (most often), sepsis, pericarditis with cardiac tamponade, hemorrhagic diathesis, terminal arteritis with gangrene, gastrointestinal perforations with peritonitis, etc. The corticosteroids applied in high doses and predominantly parenterally and the immunosuppressors are the main drugs used in the treatment of these conditions. Plasmapheresis when possible is a useful supplement. The prognosis of the acute critical conditions depends mainly on the affected organ (more favorable in pericarditis with tamponade and unfavorable in renal failure and gastrointestinal perforations with peritonitis (and on the basic disease) more optimistic in systemic lupus erythematodes and very pessimistic in nodal periarteritis and other allergic vasculitis).
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PMID:[The problems of treating acute critical states in diffuse connective tissue diseases]. 321 40

Anatomical studies have demonstrated the high incidence of vasculitis in SLE, the appearances of which are variable and non-specific, ranging from necrotizing angiitis which is undistinguishable from periarteritis nodosa, to scarring lesions. Micro-angiitis is easily demonstrated in skin lesions and is also encountered to varying degrees in CNS, renal, cardiac, pulmonary and gastrointestinal localisations. Disease of large vessels is more rare and sometimes causes gangrene of the limbs. In SLE, vasculitis should be distinguished from thrombosis related to lupus anticoagulant and from atherosclerosis favoured by chronic steroid therapy but perhaps initiated by vascular deposits of immune complexes during the acute inflammatory stage. The treatment of lupic angiitis is mainly based on steroid therapy. The results are variable, probably due to the fibrous nature of some of the vascular lesions.
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PMID:[Lupus vasculitis]. 332 46

Six patients are presented, all of whom had systemic lupus erythematosus or a "lupus-like" disease and who developed major thromboses with gangrene of the extremities. Four of the 6 patients had circulating antiphospholipid antibodies at some point during the course of their illness. These serological markers, which have been associated with a tendency to thrombosis, may have contributed to the development of gangrene in these patients. Histological examination of affected arteries in 4 patients did not show any evidence of vasculitis. One patient in whom antiphospholipid antibodies were negative showed healing vasculitis on histology.
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PMID:Large vessel occlusion and gangrene in systemic lupus erythematosus and "lupus-like" disease. A report of six cases. 377 22


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