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)

This review concentrates on those disorders in which superficial thrombophlebitis can be a significant or presenting clinical sign. Primary hypercoagulable states are those conditions associated with an increased risk of thrombosis caused by a specific measurable defect in the proteins of coagulation and/or fibrinolytic systems. These disorders are frequently inherited and include deficiencies of antithrombin III, heparin cofactor 2, protein C, protein S, abnormal fibrinolytic activity, dysfibrinogenemia, and Hageman trait. Patients with a lupus anticoagulant and anticardiolipin antibody syndrome with thrombotic episodes are also considered to have a primary hypercoagulable state. The physiology, pathophysiology, clinical characteristics, and treatment of primary hypercoagulable states are reviewed.
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PMID:Superficial thrombophlebitis. I. Primary hypercoagulable states. 219 91

More than a dozen primary hematologic disorders have been associated with ischemic stroke. Inherited deficiencies of antithrombin III, protein C, and protein S have been linked with stroke in case reports; optimal screening requires functional as well as antigenic assays. Antiphospholipid antibodies and lupus anticoagulants are the most frequently identified acquired states associated with ischemic stroke. Polycythemia vera, sickle cell anemia, sickle-C disease, and essential thrombocythemia are the major disorders of formed blood elements causing stroke. Special, step-wise screening for occult prothrombotic entities in stroke patients is recommended for young persons with stroke of uncertain cause, for those with prior venous thrombosis, for those with a family history of unusual thrombosis, and for those with no other explanation for recurrent stroke. Acquired, perhaps transient, abnormalities of platelets, coagulation inhibition, and fibrinolysis may contribute importantly to brain ischemia in synergy with other mechanisms, but at present these remain ill-defined. The contribution of prothrombotic diatheses to stroke is probably underrecognized and warrants further investigation.
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PMID:Hematologic disorders and ischemic stroke. A selective review. 186 63

The prevalence and importance of hypercoagulable states in the general vascular surgical population is unknown. Antithrombin III, protein C, protein S, plasminogen, lupus-like anticoagulant, and heparin-induced platelet activation were determined prospectively in 158 patients with aneurysmal (27), renovascular (1), cerebrovascular (28), aortoiliac (31), or infrainguinal (71) disease. Sixteen abnormal test results were obtained in 15 patients (9.5%) as follows: deficiencies of antithrombin III (2), protein C (4), and protein S (1) and presence of lupus-like anticoagulant activity (5) and heparin-induced platelet activation (4). Reconstructive surgery was performed in 137 of the study patients. Five reconstructions, all infrainguinal bypass grafts, suffered thrombosis within 30 days. Early graft thrombosis occurred in three (27%) of 14 patients with abnormal preoperative test results compared to two (1.6%) of 123 patients with normal testing (p less than 0.01). Of the three patients with abnormal test results and graft thrombosis, lupus-like anticoagulant was detected in two and heparin-induced platelet activation in one. This preliminary study supports routine preoperative screening for lupus-like anticoagulant and heparin-induced platelet activation in patients undergoing infrainguinal reconstruction. Hypercoagulable states appear to be sufficiently common and important in the general vascular surgical population to warrant further investigation.
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PMID:Screening for hypercoagulable states in vascular surgical practice: a preliminary study. 235 97

The coagulation system can be considered as a balance in which clotting and fibrinolysis have to be in a state of equilibrium. Increased fibrin formation or decreased fibrinolysis can predispose to thromboembolic diseases. Derailments in the clotting system leading to thrombosis center around the regulatory mechanisms, antithrombin III, protein C, protein S and possibly heparin cofactor II. Many cases of congenital or acquired deficiencies or abnormalities or antithrombin III, protein C and S have been described, all predisposing to thrombotic events. Alterations of the fibrinolytic system can also be associated with thromboembolisms. In particular, abnormalities of plasminogen, tissue plasminogen activator release and elevated tissue plasminogen activator inhibitor levels seem to be associated with thromboses. Conceivably also factor XIIa (Hageman factor) and prekallikrein deficiencies, when associated with thrombosis, exert their mechanism through the fibrinolytic system. Finally, about 50% of patients with lupus anticoagulant seem to suffer from thromboembolic disorders. The pathophysiology of this particular association is not known with certainty. Undoubtedly, there will be more disturbances discovered in the hemostasis system that are associated with increased intravascular fibrin formation. The understanding of these derailments is at this time only in its earliest stages of development.
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PMID:Pathophysiology of thrombophilic states. 246 10

In a family of four the whole spectrum of antiphospholipid and associated antibodies was present but without evidence of connective tissue disease. All four members had anticardiolipin antibodies; two had a confirmed lupus anticoagulant. Thrombocytopenia was severe in one and associated with a high titre of antiplatelet antibody, while another member was found to have a positive antiglobulin test. One member also had a low protein C concentration while two had decreased concentration of protein S. Factors that predispose to these antibodies may be environmental as well as genetic. In view of the well known association of spontaneous thrombotic events with some of these antibodies the prognosis for the family members must be guarded.
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PMID:Familial occurrence of the antiphospholipid syndrome. 211 Jan 95

Extensive cutaneous necrosis of the limbs with sudden onset was observed in 3 women suffering from benign systemic lupus erythematosus. All 3 had a circulating anticoagulant and a positive venereal diseases reference laboratory test without anticardiolipin antibodies. They were successfully treated with pulse methylprednisolone therapy and plasmapheresis. The necrosis-lupus anticoagulant relationship is discussed due to the absence of another possible etiology for cutaneous necrosis and the latter's histological aspect, i.e., thromboses of the dermal and hypodermal vessels without vasculitis. This cutaneous necrosis resembles that observed in congenital protein C deficiencies and favors the hypothesis of interaction between the lupus anticoagulant and the protein C-S complex.
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PMID:Cutaneous necrosis associated with the lupus anticoagulant. 250 25

Lupus-like anticoagulants (LLA), lupus anticoagulant and/or anticardiolipin antibody, are increasingly recognized in association with venous and arterial thrombotic events. We recently reviewed our experience with patients undergoing revascularization for lower-limb ischemia who were found to have LLA. Nine patients had LLA based on a prolongation of the partial thromboplastin time or by anticardiolipin assay by an enzyme-linked immunosorbent assay system. The ages of the patients ranged from 23 to 57 years. There were seven (78%) men, six (67%) blacks, two (22%) diabetic patients, and three (33%) hypertensive patients. One patient had systemic lupus erythematosus. All patients except one were cigarette smokers. Four patients had concurrent regulatory protein abnormalities: three protein C deficiencies, one protein S deficiency, and one plasminogen deficiency. The nine patients had 10 lower-extremity arterial reconstructions with two postoperative failures within 30 days. Patients were anticoagulated with heparin or aspirin after all but one operation. Patients at risk were identified on the basis of age (less than 51 years), unexplained early graft thrombosis, or history of venous or arterial thrombotic events. This group of patients is believed to be at risk for early postoperative thrombosis. Postoperative anticoagulation after revascularization for patients with LLA may be beneficial.
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PMID:Lupus-like anticoagulants and lower extremity arterial occlusive disease. 250 7

Lupus anticoagulant, concentrations of anticardiolipin antibodies, antithrombin III, plasminogen, (free) protein S, protein C, prothrombin, platelet counts, and bleeding times were determined in 74 lupus patients (58 with systemic lupus erythematosus; 16 with lupus-like disease) to establish the presence of risk factors for thrombosis in these patients. Of the variables evaluated, lupus anticoagulant had the strongest association with a history of thrombosis. Both positive anticardiolipin antibody concentrations and the presence of (mild) thrombocytopenia were significantly associated with a history of thrombosis and the presence of lupus anticoagulant. Reduced concentrations of antithrombin III, plasminogen, (free) protein S, and protein C were found in some patients but were not associated with either thrombosis or lupus anticoagulant. Mean concentrations of total protein S were significantly lower in patients with thrombosis than in those without and in patients with lupus anticoagulant than in those without. The antigenic concentration of prothrombin was reduced in 3/74 (4%) lupus patients. These three patients had lupus anticoagulant but no history of thrombosis, which suggests that a low prothrombin concentration protects patients with lupus anticoagulant from the development of thrombosis. A prolonged bleeding time was associated with the presence of lupus anticoagulant but not with a history of thrombosis. Analysis by stepwise logistic regression did not disclose additional risk factors for thrombosis in lupus patients with lupus anticoagulant. Increased antithrombin III concentrations and decreased free protein S concentrations are often found in lupus patients, unrelated to lupus anticoagulant or thrombosis.
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PMID:Risk factors for thrombosis in lupus patients. 251 63

The clinical and serological findings in 13 patients with myocardial infarction and antiphospholipid antibodies (the 'lupus anticoagulant', antibodies to cardiolipin, antibodies to phosphatidylethanolamine (one patient] seen by our unit and other units from 1984 to 1989, are presented (eight males and five females, ages ranging from 20 to 52 years). Five suffered myocardial infarction before the age of 30; four of these five were in their early 20s. Other risk factors such as excessive smoking (greater than 20 cigarettes a day) (two patients), long-term treatment with steroid (one) and use of oral contraceptives (one) were present. One patient had demonstrated a plasminogen activator deficiency and one a deficiency of protein C. Two patients developed myocardial infarction six to eight weeks after warfarin was discontinued for recurrent deep vein thrombosis. Six patients had SLE as defined by the revised 1982 criteria, three suffered from 'lupus-like' disease, while four patients conformed to a 'primary' antiphospholipid syndrome.
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PMID:Myocardial infarction and antiphospholipid antibodies in SLE and related disorders. 251 55

This study prospectively evaluates hypercoagulable states in patients under 51 years of age undergoing lower extremity revascularization for ischemia and assesses early outcome after operation. Twenty patients whose ages range from 23 to 50 years (mean 40.8 years) were identified prospectively who underwent lower extremity revascularization and evaluation of hypercoagulability. Fifteen patients were male (75%), 10 were black (50%), six had hypertension (30%), and four were diabetic (20%). All but two were cigarette smokers (90%). Seven aortoiliac procedures and 13 infrainguinal procedures were performed. Six patients had one or more abnormalities of regulatory proteins (protein S deficiency, four; protein C deficiency, three; presence of lupus-like anticoagulant, three; plasminogen deficiency, two). Eight of 17 patients in whom platelet aggregation profiles were obtained showed increased reactivity (47%). Only 4 of 17 patients (24%) were normal when tested for all parameters. Arterial or graft thrombosis developed in four of the 20 patients within 30 days after operation. Hypercoagulability was found in all four patients whose revascularizations failed. A high incidence of hypercoagulable states was found in patients under 51 years of age with lower limb ischemia requiring revascularization. Hypercoagulability may have contributed to early postoperative thrombosis of the vascular procedure.
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PMID:Hypercoagulable states and lower limb ischemia in young adults. 252 8


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