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)

Two anti-cardiolipin antibody (ACA)-producing cell lines were established, using Epstein-Barr virus transformation followed by a repeated cluster-picking from the same individual with antiphospholipid syndrome who had a history of 8 consecutive fetal losses and deep venous thrombosis. Characterization of the two ACAs derived from these cell lines revealed that one (Ab-019, subclass IgM, kappa) reacted exclusively with cardiolipin and showed strong lupus anticoagulant activity, while the other (Ab-226, subclass IgM, lambda) reacted with negatively charged phospholipids such as phosphatidylserine and phosphatidylglycerol, as well as cardiolipin. Furthermore, Ab-226 showed reactivity with human umbilical vein endothelial cells whereas Ab-019 did not. It is suggested that ACA is heterogeneous even in the same individual, and that reactivity against negatively charged phospholipids corresponds to reactivity against endothelial cell.
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PMID:Establishment of two distinct anti-cardiolipin antibody-producing cell lines from the same individual by Epstein-Barr virus transformation. 802 10

ACAs and the lupus anticoagulant are strongly associated with thrombosis and appear to be the most common of the acquired blood protein defects causing thrombosis. Although the precise mechanism or mechanisms whereby antiphospholipid antibodies alter hemostasis to induce a hypercoagulable state remain unclear, several theories, as previously discussed, have been advanced. The most common thrombotic events associated with ACAs are DVT and PE (type I syndrome), coronary or peripheral artery thrombosis (type II syndrome), cerebrovascular or retinal vessel thrombosis (type III syndrome), and occasionally patients present with mixtures (type IV syndrome). The relative frequency of ACAs in association with arterial and venous thrombosis strongly suggests that these should be looked for in any patient with unexplained thrombosis; all three idiotypes (IgG, IgA, and IgM) should be assessed. Also, the type of syndrome (I through IV) should be defined, if possible, because this may dictate both type and duration of both immediate and long-term anticoagulant therapy. Unlike those with ACAs, patients with primary lupus anticoagulant thrombosis syndrome usually have venous thrombosis. Since the aPTT is unreliable in patients with lupus anticoagulant and is not usually prolonged in patients with ACAs, definitive tests (ELISA for ACA and the dRVVT for lupus anticoagulant) should be immediately ordered when suspecting antiphospholipid syndrome or in patients with otherwise unexplained thrombotic or thromboembolic events.
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PMID:Antiphospholipid and thrombosis syndromes. 805 32

Recurrent pulmonary emboli or microthromboses are hypothesized as possible causes of pulmonary hypertension in the antiphospholipid syndrome (APS), but thrombosis of the pulmonary vessels has been rarely documented. We describe the case of a 45-year-old Caucasian man affected by thrombocytopenia, recurrent deep venous thrombosis, recurrent pulmonary embolism and fatal chronic pulmonary hypertension (systolic pressure: 85 mm Hg). Anticardiolipin antibodies were highly positive, and the lupus anticoagulant was present. At autopsy, recent thromboses of small vessels were observed in the lung, with organized clots and recanalized channels. Furthermore, friable and firm vegetations and nodules were observed on the cusps of the mitral and tricuspid valves, intermingled with recent surface fibrinous thrombi. In the adrenals we found vascular thrombotic lesions similar to those in the lungs. The pathological lesions suggest pulmonary hypertension secondary to pulmonary arterial microthromboses. Moreover, this is the first documentation of tricuspid valve pathology in a patient with APS.
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PMID:Pulmonary hypertension secondary to thrombosis of the pulmonary vessels in a patient with the primary antiphospholipid syndrome. 806 40

Anticardiolipin antibodies and the lupus anticoagulant are strongly associated with thrombosis and appear to be the most common of the acquired blood protein defects causing thrombosis. Although the precise mechanism(s) whereby antiphospholipid antibodies alter hemostasis to induce a hypercoagulable state remain unclear, several theories have been advanced. The commonest thrombotic events associated with anticardiolipin antibodies are deep vein thrombosis and pulmonary embolus (type I syndrome), coronary or peripheral artery thrombosis (type II syndrome), or cerebrovascular/retinal vessel thrombosis (type III syndrome), and occasionally patients present with mixtures (type IV syndrome). The relative frequency of anticardiolipin antibodies in association with arterial and venous thrombosis strongly suggests that these should be looked for in any individual with unexplained thrombosis; all three idiotypes (IgG, IgA, and IgM) should be assessed. Also, the type of syndrome (I through IV) should be defined if possible because this may dictate both type and duration of immediate and long-term anticoagulant therapy. In contrast to those with anticardiolipin antibodies, patients with primary lupus anticoagulant thrombosis syndrome usually suffer venous thrombosis. Because the aPTT is unreliable in patients with lupus anticoagulant (prolonged in only about 40% to 50% of patients) and is not usually prolonged in patients with anticardiolipin antibodies, definitive tests (ELISA for anticardiolipin antibody and the dRVVT for lupus anticoagulant) should be immediately ordered when suspecting antiphospholipid syndrome or in individuals with otherwise unexplained thrombotic or thromboembolic events.
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PMID:The antiphospholipid and thrombosis syndromes. 817 Feb 64

A young patient with severe pulmonary hypertension of thromboembolic origin due to recurrent deep vein thrombosis is described. Although he presented no signs of systemic lupus erythematous, a high quantity of cardiolipin antibodies was found in his serum. Therefore, we discuss a possible association between the primary antiphospholipid antibodies syndrome and pulmonary embolic events.
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PMID:[Severe pulmonary arterial hypertension and primary antiphospholipid antibodies syndrome]. 817 54

The specificity and immunoglobulin isotype distribution of antiphospholipid (aPL) antibodies have been evaluated in 68 patients with systemic lupus erythematosus (SLE) by ELISA which employed a panel of 7 different PL antigens. A total of 49 patients (72%) were positive for aPL antibodies of different isotypes and directed to one or more PL epitopes. Prevalence of IgG anticardiolipin (aCL, 37%) was similar to that of the other negatively charged PLs phosphatidylserine (PS, 35%), phosphatidylinositol (PI, 35%), phosphatidylglycerol (PG, 35%) and phosphatidic acid (PA, 40%); prevalence reduced to 9-12% and 7-16% respectively for IgM and IgA isotypes to the same antigens. aPL antibodies to the zwitterionic PLs phosphatidylcholine (PC) and phosphatidylethanolamine (PE) were also observed, though their prevalence was lower than that demonstrated for negatively charged PLs. Of the 36 SLE patients who were aCL negative (53%), 17 (25% of all patients and 47% of aCL-negative patients) were positive for aPL antibodies of different isotypes to one or more non-CL epitopes. During a mean follow-up period of 30 months, 10 patients had deep vein thrombosis (DVT) with a total of 21 events. By chi 2 test, a significant correlation was found between DVT and IgG aCL (p = 0.03) and between this event and the presence of lupus anticoagulant (LA) antibody (p = 0.04). However, stronger correlations were demonstrated between DVT and IgA aCL (p = 0.007), IgG anti-PS (p = 0.02), and IgA anti-PC, -PI and -PG (p = 0.02, 0.003 and 0.02, respectively), whereas no correlation was found between thrombotic events and aPL antibodies with PE and PA specificities.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Prevalence and clinical significance of antiphospholipid antibodies to noncardiolipin antigens in systemic lupus erythematosus. 817 49

The authors describe the case of a female patient with a history of simultaneous abortion and deep vein thrombosis as well as moderate bleeding disturbances. Investigations revealed the presence of lupus anticoagulant while a thrombocyte "storage pool" disease was confirmed. This case demonstrates the association of these two haemostatic disturbances, and points to a possible relationship between them. Since detailed analyses failed to demonstrate the presence of antiplatelet antibodies, the authors suggest a possible damaging effect of lupus anticoagulant to the endothelium leading to thrombocyte activation.
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PMID:[Simultaneous occurrence of lupus anticoagulant and acquired "storage pool" disease of thrombocytes]. 818 44

To evaluate the prognostic significance of lupus anticoagulant (LA), 37 SLE patients with LA and 37 age- and sex-matched SLE patients without LA were followed up for a median of 22 years, of which 16 years (median) after the initial LA-testing. Deep venous thrombosis was observed in 20 (54%) patients with LA. Of these patients, 90% had the first episode within 8 years after the onset of SLE symptoms, as compared to only one of the six LA-negative patients with deep venous thrombosis (P 0.0001). Cerebral artery occlusions were more common in patients with LA (P 0.016), but typically appeared as a late phenomenon. Nephritis or neuropsychiatric manifestations, previously associated with a poor outcome in SLE, did not correlate with the presence of LA. However, higher mortality was associated with both LA (P 0.021) and a history of deep venous thrombosis (P 0.004), as well as with nephritis (P 0.038). The most common cause of death in both LA positives and negatives was vascular occlusion. In conclusion, it appears that the first episode of deep venous thrombosis in patients with LA is typically seen early in the course of disease, and that LA and a history of deep venous thrombosis are both associated with increased mortality.
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PMID:Lupus anticoagulant as a prognostic marker in systemic lupus erythematosus. 833 26

Antiphospholipid antibodies (APL) are associated with venous and arterial thrombosis in SLE patients. Various thrombotic and non-thrombotic neurological manifestations have been reported in SLE but whether or not they are related to the presence of APL antibodies remains uncertain. To assess the possible association between neurological involvement in SLE and APL antibodies, IgG anticardiolipin antibodies (IgG ACL) were looked for using an ELISA technique in 92 consecutive SLE patients seen over a one-year period. Other APL determinations included VDRL and lupus anticoagulant (LAC) testing using APTT and the diluted thromboplastin time. Twenty-four SLE patients presented with neurological manifestations (40 episodes): 15/24 (62.5%) were found positive for APL antibodies (11 VDRL, 8 LAC, 7 ACL antibodies) versus 22/68 patients (32%) without neurological symptoms (p < 0.01). APL antibodies antedated neurological symptoms in 13/16 cases. Neurological manifestations were subsequently divided into 3 groups: thrombotic (n = 14), psychosis and convulsions (n = 15), miscellaneous (n = 10). No correlation was found between APL antibodies and any of the 3 subgroups. Among patients with neurological SLE, APL antibodies were present in two with valvular heart disease, as well as in seven with a history of either deep vein thrombosis, livedo reticularis or miscarriage. Among 7 patients with thrombocytopenia and neurological symptoms, 6 had APL antibodies. These data suggest that APL syndrome is associated with neuro-ophthalmological manifestations of SLE regardless of whether or not the mechanism of neurological involvement is thrombotic. SLE patients with APL antibodies may be at risk for future neurological manifestations. However, it is still questionable that APL positivity has definite therapeutic consequences.
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PMID:Neurological manifestations of systemic lupus erythematosus: role of antiphospholipid antibodies. 840 81

Since there are few data on the use of various birth control methods in systemic lupus erythematosus (SLE), the authors performed a cross-sectional study of the actual contraceptive practices in a group of 85 Finnish female SLE patients of reproductive age. They also recorded side effects experienced during the use of oral contraceptives (OCs) and IUDs. The use of contraception was lower in SLE patients than in healthy women of the same age (59% vs. 77%, p .001). Sexually active SLE patients requiring contraception used barrier and natural methods more often (p 0.001) and OCs less often (p 0.05) than did the corresponding group of healthy women. The risk of deep venous thrombosis in SLE patients while using estrogen-containing OCs was slightly increased (RR 2.3, 95% Ci 0-5-10.3). 25 (78%) of the 32 patients who had used progestagen-only contraceptives discontinued them because of side effects which were mainly gynecological. Major bleeding or pelvic infection did not occur during use of IUDs.
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PMID:Contraceptive practice in women with systemic lupus erythematosus. 844 13


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