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Query: UMLS:C0409974 (lupus)
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Two classes of antiphospholipid antibodies (APA) are associated with adverse pregnancy outcomes. Those APA identified by immunoassays using phospholipid-coated surfaces (e.g., anticardiolipin antibodies) seem to bind to the 57 kD anticoagulant protein, beta 2-glycoprotein-I, when complexed with anionic phospholipid bilayers. Such APA may or may not prolong phospholipid-dependent clotting assays. A second class of APA are identified by their interference with phospholipid-dependent clotting assays (i.e., lupus anticoagulants). The latter bind to phospholipids present in a unique hexagonal phase either alone or complexed with prothrombin or beta 2-glycoprotein-I. There is evidence that both classes of APA are directly responsible for adverse pregnancy outcomes including spontaneous abortions, stillbirths, fetal growth retardation, thrombosis, thrombocytopenia, and preeclampsia. Putative APA-mediated pathogenic mechanisms include intervillous thrombosis, intravillous infarctions and decidual vasculopathy. The thrombogenicity of APA may result from their interference with endothelial phospholipids required for antithrombin III and protein C and S anticoagulant activity and prostacyclin synthesis and/or increased endothelial expression of the procoagulants: tissue factor, von Willebrand factor, platelet-activating factor, and plasminogen activator inhibitor type-1. Other prothrombotic properties seem to include: increased platelet aggregation, and reduced beta 2-glycoprotein-1 and annexin V anticoagulant activity. Rigorous diagnostic criteria must be applied to the detection of both classes of APA because the prevention of adverse pregnancy outcomes requires potentially hazardous anticoagulant therapy.
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PMID:The immunobiology and obstetrical consequences of antiphospholipid antibodies. 752 11

Autoimmune diseases are relatively common in women, and tend to occur in the childbearing years. These disorders fall broadly into two groups: (i) Multisystem diseases such as systemic lupus erythematosus (SLE) and related connective tissue disorders (CTD). This group includes the 'pre-clinical' antiphospholipid or lupus obstetric syndrome which may first manifest itself as a pregnancy disorder causing recurrent abortion, fetal death, fetal growth retardation and early onset severe pre-eclampsia. (ii) Tissue- or organ-specific disorders such as autoimmune thrombocytopaenic purpura (ATP), autoimmune thyroid disease (Graves' disease, Hashimoto's autoimmune thyroiditis, and post-postum thyroiditis), autoimmune haemolytic anaemia, and the very rare myasthenia gravis. The study of autoimmune diseases against the background of pregnancy as an experimental system of nature has provided important insights into the nature of the disease processes and the relevance or otherwise of circulating autoantibodies to pathological effects. Thus, for example, if neonatal manifestations of adult disease are causally related to the transfer of autoantibodies across the placenta, they will disappear over a time course consistent with the catabolism of IgG, providing no permanent damage is produced. Conversely, if autoantibodies are demonstrable in the neonate, in the absence of clinical effects, they may only be an epiphenomenon of the maternal disease. In addition, on occasions, disease manifestations may be seen in the baby when the mother shows none. This may occur when the mother is in remission, but still has circulating antibodies, or when she has an occult form of the disease.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Autoimmune disease and pregnancy. 784 94

The objective of this work was to analyse the course of maternal disease and fetal outcome in pregnant patients with systemic lupus erythematosus (SLE) counselled and followed according to a protocol intended to optimize maternal and fetal outcome. The prospective study included all pregnancies between 1987 and 1993 in SLE patients known at least 6 months before pregnancy at the Lupus Clinic of our hospital. In 25 patients there were 35 pregnancies. Thirty-four (97%) started at sustained remission of disease; 11 (31%) were in women with antiphospholipid antibodies (aPL); 14 (40%) in women with a history of biopsy-proven lupus nephritis; one (3% in a woman with a serum creatinine above 125 mumol/l. In 29 pregnancies (82%) maternal disease remained inactive during gestation. In three pregnancies (9%) active disease was treated with prednisone. There were no serious post-partum flares of disease. Pregnancy resulted in 25 (71%) live births, 8 (23%) first trimester abortions, and one intrauterine fetal death. One pregnancy was terminated because of hydrocephalus. Nine of 25 (36%) live births were delivered by caesarean section. For 6 of 9 (67%) caesarean sections the indication was fetal distress and pre-eclampsia. In the majority of patients with SLE who conceive at remission, the disease does not flare in pregnancy. With optimal obstetric care, close follow-up and treatment with low-dose aspirin if aPL are present, a high success rate (71%) can be achieved.
Lupus 1994 Jun
PMID:Pregnancy in systemic lupus erythematosus: a prospective study. 795 Dec 99

Antiphospholipid antibodies (APA) comprise a family of immunoglobulins characterized by their pattern of reactivity in a number of laboratory tests. Included in this family are lupus anticoagulant (LA) anticardiolipin antibodies (ACA) and antibodies causing biologic false positive serologic tests for syphilis (BFP-STS). LA and ACA occur in a variety of conditions, including other autoimmunes disorders, infectious diseases, neoplasic disorders, in association with certain drugs and in otherwise healthy individuals. Clinical interest in LA and ACA is increasing. Antiphospholipid antibody syndrome is characterized by a triad of clinical features which include fetal loss, thromboembolic disease and thrombocytopenia. Other clinical manifestations related with APA are livedo reticularis, cutaneous necrosis, hemolytic anemia, heart valve disease, chorea, migraine and obstetric problems as fetal growth retardation, pre-eclampsia, post-partum serositis or neonatal thrombosis or catastrophic antiphospholipid syndrome. Therapy is mainly directed against the widespread and diverse manifestations associated with the obstruction of small and large vessels. Long-term treatment with oral anticoagulation therapy is advised, even if the venous or arterial occlusion occurred many years previously. In patients with primary antiphospholipid syndrome there is no evidence that the prophylactic administration of steroids or immunosuppression will prevent thromboembolic events. Although the administration of more energetic immunosuppression with cyclophosphamide in pulse form is effective in reducing elevated antibody levels, there is usually a rapid rebound to pretreatment levels shortly after discontinuation of the therapy. A history of recurrent fetal loss requires mandatory treatment during pregnancy. Although the actual prospective risk of pregnancy loss in women with antiphospholipid syndrome and prior pregnancy loss is unknown, it may exceed 60%. Because of this many investigators have treated women with antiphospholipid syndrome with either antiplatelet agents, immunosuppressive agents, or anticoagulants in an attempt to improve pregnancy outcome. Unfortunately, there is no unequivocal proof that any of these therapies are fully efficacious. Despite varying treatment protocols, the live birth rate with treatment was 70%, similar to that reported in the recent randomized clinical trial. Thrombocytopenia and autoimmune hemolytic anemia in patients with APA are treated similarly as patients without APA. Treatment of asymptomatic patients isn't indicated, because only approximately 10-15% of patients with APA developed complications.
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PMID:Clinical and therapeutic aspects associated to phospholipid binding antibodies (lupus anticoagulant and anticardiolipin antibodies). 798 46

Microalbuminuria in the general population is associated with recognized risk factors for cardiovascular disease such as hypertension, hyperglycemia, hyperinsulinemia, and hyperlipidemia; and it is an independent predictor of subsequent cardiovascular mortality in hypertensive, diabetic, and elderly populations. Although different methods have been used for measuring and expressing urinary albumin excretion and a variety of cutoff levels have been used for defining microalbuminuria, prevalence of microalbuminuria appears to be higher in non-Europeans (8%-28%) than in Europeans (2%-10%). However, because of the large within-individual variability of urinary albumin excretion and the relatively low prevalence of microalbuminuria, large studies are required to detect statistically significant associations between albuminuria and cardiovascular risk factors. Evidence presented here supports the proposition that microalbuminuria represents a marker of cardiovascular disease risk in nondiabetic individuals as well as diabetic individuals. Moreover, because of a high sensitivity of the test and because albuminuria is a concomitant of many forms of renal disease, microalbuminuria also has a role in detecting patients with renal involvement associated with essential hypertension, lupus erythematosus, women with pre-eclampsia, and subjects with unsuspected primary and secondary nephropathies.
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PMID:Epidemiology of microalbuminuria in the general population. 808 51

We describe a patient with previous venous thrombosis while using oral contraceptives and recurrent pregnancy loss, who presented with massive hepatic infarction in the last trimester of the fourth gestation. Thrombocytopenia, the lupus anticoagulant (LA) and the anticardiolipin antibody (aCL) were detected and a diagnosis of a 'primary' antiphospholipid syndrome (APS) was made. The clinical and histological manifestations and the differential diagnosis, especially with DIC and pre-eclampsia, are discussed.
Lupus 1993 Aug
PMID:Hepatic infarction in a pregnant patient with the 'primary' antiphospholipid syndrome. 826 78

Since 1989, 22 patients with persistent antiphospholipid syndrome (PAPS) associated with recurrent miscarriage (defined as three or more miscarriages) were treated with fish oil, equivalent to 5.1 g eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA) at a ratio of 1.5 EPA to DHA. Twenty-two patients had 23 pregnancies (one patient had two pregnancies) over a period of 3 years. There was only one intrauterine fetal death at the 27th week associated with pre-eclampsia. Twenty-one pregnancies, 19 of which ended after the 37th week, produced a baby. Two pregnancies ended with cesarean section for pre-eclampsia at 30th and 35th week of gestation and one is ongoing at 32nd week. All babies are well. The weight at birth of babies delivered at term was always > 2500 g. These encouraging results favour a therapeutic role, without any adverse reaction, of fish oil to prevent recurrent miscarriage in PAPS.
Lupus 1993 Oct
PMID:Fish oil derivatives as a prophylaxis of recurrent miscarriage associated with antiphospholipid antibodies (APL): a pilot study. 830 26

Four women with the antiphospholipid syndrome associated with lupus anticoagulant and a poor obstetric history were treated with a combination of glucocorticosteroids, anticoagulants and platelet inhibitor therapy. All patients had at least one previous miscarriage while receiving prednisone and low-dose aspirin. The treatment regimen included: aspirin, dipyridamole, prednisone, and warfarin or heparin. This treatment resulted in a successful pregnancy outcome in all cases, without preeclampsia or recurrence of thrombosis. One patient developed a vertebral compression fracture while receiving heparin and prednisone. Two pregnancies required cesarean delivery for fetal distress at 32 and 34 weeks. All four infant birth weights were appropriate for the gestational age. This regimen may be a therapeutic option for patients with the antiphospholipid antibody syndrome, especially if they have failed other commonly used treatments.
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PMID:Successful pregnancy outcome with combination therapy in women with the antiphospholipid antibody syndrome. 841 Aug 69

The case of a 25-year-old pregnant woman with systemic lupus erythematosus and severe pulmonary hypertension is presented. The pregnancy was complicated by worsening right heart failure and pre-eclampsia, requiring a caesarian section at 29 weeks' gestation. On the fourth day postpartum, the patient's respiratory status worsened and she was transferred to the coronary care unit where she soon died from combined right heart failure and respiratory arrest. The presumed pathogenesis and etiology of lupus-related pulmonary hypertension are discussed, in addition to noninvasive tests and proposed management. Given that the mortality rate is very high during pregnancy and therapy is of limited value, women with lupus-associated pulmonary hypertension should avoid conceiving. Those who choose to become pregnant must be carefully managed by a multidisciplinary team.
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PMID:Systemic lupus erythematosus and pulmonary hypertension during pregnancy: report of a case fatality. 879 79

Recurrent fetal loss and pregnancy complications, especially severe early-onset preeclampsia, are frequently associated with anti-phospholipid antibodies. We report a case of post-partum cardiac involvement leading to dilated cardiomyopathy in a woman with a persistent positivity for anti-cardiolipin and anti-nuclear antibodies. Her clinical and obstetric record reported two previous fetal losses but no other signs characteristic of the anti-phospholipid syndrome or diagnostic for a systemic lupus erythematosus. Post-partum cardiomyopathy might be another cardiac presentation of the anti-phospholipid syndrome, in addition to the well known valvular involvement. In patients with persistent positivities for anti-phospholipid antibodies, a prompt identification of such a complication in the post-partum period should be taken into account by physicians. Adequate cardiologic treatment associated with antiaggregant and steroid therapy might be useful to prevent further complications in these patients.
Lupus 1996 Jun
PMID:Post-partum dilated cardiomyopathy in anti-phospholipid positive woman. 880 99


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