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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The clinical and biological features of a series of 27 patients with the recently described primary antiphospholipid syndrome are reported. Most of them belonged to a cohort of 90 patients who were carriers of lupus anticoagulant, which had been detected in the systematic evaluation of prolonged activated partial thromboplastin times in our hospital. Since the diagnosis they underwent a prospective protocol of follow up, with a peak follow up period of 9 years. The mean age of the 27 patients was 40.8 years and there were virtually no differences between sexes. Venous thrombosis was the most common clinical finding (16 episodes in 14 of the 27 patients). The most prevalent laboratory findings were lupus anticoagulant and IgG anticardiolipin antibodies.
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PMID:[Primary antiphospholipid syndrome: study of 27 patients]. 210 12

Both lupus anticoagulant and anticardiolipin antibody are groups of antiphospholipid antibodies associated with high frequency of thrombosis, fetal loss and thrombocytopenia. The hall marks of their identification is the prolongation of phospholipid-dependant coagulation tests. Much is written in literature about the successful management of lupus anticoagulant during pregnancy, via corticosteroid and acetyl salicylic acid (Aspirin) therapy; however, up to now only little has been mentioned about maternal and fetal complications associating lupus anticoagulant and its management. Here we present three cases with significant complications among patients with lupus anticoagulant managed in Sint Augustinus Hospital over the last 3 years. These complications were secondary to antiphospholipid syndrome or to therapy. Maternal complications included gastritis, atrophy of quadriceps muscle, resistant premature contractions and pre-eclampsia. One of our patients developed small lymphocytic lymphoma 1 year after her last labour. Fetal complications included: prematurity, suprarenal insufficiency (temporary) and delayed neuromuscular development found at the 2 year follow-up. As far as we know, some of these complications have never been mentioned in literature.
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PMID:Maternal and fetal complications associating lupus anticoagulant and its management; three case reports. 211 27

The term antiphospholipid syndrome is used to characterize a complex of clinical and pathologic findings mediated by a group of antibodies formed against a family of antiphospholipids. These antiphospholipid antibodies were originally found in patients with lupus erythematosus in whom the partial thromboplastin time was prolonged and in patients with other autoimmune diseases; subsequently, they have been observed in association with a variety of other conditions, including infections, reactions to drugs, malignant neoplasms, human immunodeficiency virus disease, and as an isolated finding. In recent years, there has been some clarification of the significance of the various tests for antiphospholipid antibodies, including the lupus anticoagulant test and the anticardiolipin antibody tests, in predicting the antiphospholipid syndrome. The mechanism of disease, however, has not been well defined. The most common cutaneous lesion seen in seven patients with lupus anticoagulant and anticardiolipin antibody who have the antiphospholipid syndrome was ulceration due to thrombosis of dermal veins and arteries. Often there is a reactive vascular proliferation around the thrombosed vessels. The presence of primary thrombosis of both veins and arteries in thrombotic disorders is unusual and may provide insight into the mechanism of thrombosis in antiphospholipid syndrome.
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PMID:Cutaneous histopathologic findings in 'antiphospholipid syndrome'. Correlation with disease, including human immunodeficiency virus disease. 211 49

Patients with systemic lupus erythematosus often develop thrombosis, recurrent abortion, thrombocytopenia and neurological disease, in association with serum antibodies against phospholipids, termed the secondary antiphospholipid syndrome. In our search for an animal model for the human antiphospholipid syndrome, we examined the MRL-1pr/pr mouse, which develops a lupus-like illness. These mice were found to have significantly elevated levels of serum antiphospholipid antibodies (aPL) and thrombocytopenia compared to normal mice. By immunodiffusion, these antibodies are similar to human aPL. We observed histologic evidence of central nervous system thromboses as well as perivascular infiltrates of the choroid plexus. The high titers of serum aPL, thrombocytopenia, and thrombosis establish this strain as an animal model for the secondary antiphospholipid syndrome.
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PMID:Autoimmune MRL-1 pr/1pr mice are an animal model for the secondary antiphospholipid syndrome. 212 Apr 36

The clinical and serological findings on 24 patients with pulmonary hypertension (PHT) seen at the Lupus Clinic of St. Thomas' Hospital, London are presented. Twenty-two patients had systemic lupus erythematosus (SLE), one other a primary antiphospholipid syndrome and another an SLE/progressive systemic sclerosis (PSS) overlap syndrome. In 21 of the 24 patients, the disease resembled the primary idiopathic variety with clear lung fields and no clinical evidence of pulmonary thromboembolism, although angiography and nuclear perfusion scans were not performed. Two patients clearly suffered from thromboembolic PHT, one with SLE and one with an antiphospholipid syndrome. One patient with SLE/PSS overlap syndrome developed pulmonary fibrosis. The frequency of antiphospholipid antibodies (lupus anticoagulant and antibodies to cardiolipin was 68% which appears to be higher than generally found in patients with SLE, and the clinical significance of this finding is unknown. Other associated features of the antiphospholipid syndrome in this group were uncommon. Death occurred in 13 of the 24 patients, 4 were lost to followup and 7 are known to be alive. The cause of death was circulatory failure in the majority; sudden death once this complication occurred was particularly common. One patient died from adult respiratory distress syndrome and one from hemorrhagic shock while undergoing heart/lung transplantation. Two patients underwent successful heart/lung transplantation. One, however, died of a mesenteric occlusion and bowel infarction following a second lung transplantation because of rejection of the first heart/lung transplantation after one year. The other patient is alive and well 2 years later.
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PMID:Pulmonary hypertension in a lupus clinic: experience with twenty-four patients. 212 32

Despite the considerable interest in antiphospholipid antibodies during the last 10 years, the clinical disorders with which they are associated are still not well understood (Love and Santoro, 1990). The concept of the antiphospholipid syndrome (APS) and a means by which this can be defined (Harris, 1987) may aid us in better classifying these patients, evaluating their clinical features, and in conducting prospective studies. Standard methods for measurement of anticardiolipin antibodies (Harris et al, 1987b; Harris and the Kingston Anticardiolipin Standardization Group, 1990) and more attention to measurement of the lupus anticoagulant (Triplett and Brandt, 1988) will ultimately play an important role in improving the diagnosis of these patients. The initiation of the Kingston AntiPhospholipid Study (KAPS) group in January 1988 paves the way for future multicentre treatment trials of these patients (Harris, 1988; Triplett, 1989; Recker and Leff, 1989). For the practising physician managing a pregnant woman with the APS, there are few clear guidelines for treatment. An appropriate approach would be to assemble a multidisciplinary team for pregnancy management, exclude other causes of pregnancy loss and select the drug regimen which will cause the least harm and has some reported efficacy. Most importantly, the patient should be followed carefully throughout her pregnancy.
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PMID:Maternal autoantibodies and pregnancy--I: The antiphospholipid antibody syndrome. 212 86

In recent years an association has been described between, on the one hand, an in vitro prolongation of phospholipid-dependent coagulation tests (the 'lupus anticoagulant') or the demonstration of antiphospholipid antibodies and, on the other, clinical events, particularly recurrent thrombosis (usually venous but sometimes arterial), thrombocytopenia, and also recurrent mid-term fetal loss. Other less well-documented associations with haemolytic anaemia, livedo reticularis, strokes and other neurological syndromes have been suggested. The antibodies are present temporarily in many infections, are usually of IgM isotype and thrombosis does not occur. However, they are persistently present and mainly of IgG isotype in a number of auto-immune disorders associated with thrombosis, in particular systemic lupus erythematosus, in which 50% of patients will show antibody of one isotype or another. The strongest association is with antinuclear factor-negative lupus and 'lupus-like' disorders in which a full diagnosis of classical lupus cannot be made. The clotting test abnormality and antiphospholipid antibodies may be found also in otherwise normal individuals suffering thrombosis or fetal loss--the so-called primary antiphospholipid syndrome. These data raise important questions for management, but many details are controversial despite a decade's work; this review examines the present position and outlines some of the difficulties, particularly from the point of view of nephrology and paediatrics.
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PMID:The 'antiphospholipid syndrome' and the 'lupus anticoagulant'. 212 60

The clinical manifestations of Sneddon's syndrome (cerebrovascular disorder, livedo reticularis, peripheral venous thrombosis++, cardiac pathology, obstetric pathology--fetal loss and intrauterine fetal death) are characteristic of the antiphospholipid syndrome. A lupus anticoagulant (LA), one of the types of antiphospholipid antibodies, was detected in the blood plasma of 18 out of 30 patients with Sneddon's syndrome. The negative results of LA-examination in 12 patients don't exclude the presence of other antiphospholipid antibodies. So 16 of 30 patients had anticardiolipin antibodies, among them being 6 LA-negative patients. The authors discuss the significance of antiphospholipid antibodies in the genesis of vascular abnormalities in patients with Sneddon's syndrome.
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PMID:[Cerebrovascular disorders associated with livedo (Sneddon's syndrome): its relation to lupus anticoagulant]. 217 72

The antiphospholipid antibody syndrome is characterized by venous and/or arterial thrombosis, repeated miscarriages and the presence of anticardiolipin antibodies and/or a circulating lupus anticoagulant. Antibodies directed against phospholipids constitute a family of immunoglobulins with different specificities. The mechanism through which lesions are induced by these antibodies has not yet been fully determined. Treatment includes immunosuppressive measures and prevention of recurrent thrombosis.
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PMID:[Antiphospholipid syndrome]. 223 84

Antiphospholipid antibody is associated with a clinical syndrome of vascular thrombosis, thrombocytopenia, recurrent fetal loss, and livedo reticularis, whether or not a clinical diagnosis of systemic lupus erythematosus (SLE) coexists. A positive antiphospholipid antibody test is defined by enzyme-linked immunosorbent assay (ELISA) (antiphospholipid antibody itself) or by coagulation assay (lupus anticoagulant). These are similar but not identical antibodies. The test for syphilis is less closely related to the preceding two and is less regularly associated with clinical complications. The mechanism of action of either antiphospholipid antibody or lupus anticoagulant is as yet unknown. SLE-induced but not infection-induced antiphospholipid antibody has immunoglobulin G2 (IgG2) and IgG4 predominance. It recognizes all negatively charged phospholipids, but various physical characteristics of the phospholipids alter the recognition patterns. Treatment for the antiphospholipid antibody syndrome has not been clearly defined. Anticoagulation with aspirin, heparin, or warfarin is currently favored. A role for corticosteroid remains to be demonstrated.
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PMID:Antiphospholipid antibody syndrome: immunologic and clinical aspects. 225 9


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