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)

We report the case of a patient with the unusual combination of migraine, chorea, and retinal arterial thrombosis along with laboratory evidence of autoimmunity. In the absence of systemic lupus erythematosus, the clinical manifestations suggest the presence of the primary antiphospholipid antibody syndrome.
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PMID:Retinal migraine, chorea, and retinal artery thrombosis in a patient with primary antiphospholipid antibody syndrome. 203 Mar 76

Antiphospholipid antibodies (aPL) interfere with the coagulation system and can cause thrombosis and other clotting disorders. The combination of recurrent venous thrombosis, arterial embolism and recurrent fetal loss is nowadays considered to be primary antiphospholipid syndrome (PAPS), provided an underlying systemic lupus erythematosus (SLE) has been excluded and aPL have been detected. We report on two patients with PAPs, and show the course of their IgG- and IgM-anticardiolipin antibody (aCL) titers during immunosuppressive therapy with prednisone and azathioprine or cyclophosphamide. Over a period of 18 months this therapy was effective in preventing relapses of thrombo-embolism and other complications. Therapy with cyclophosphamide resulted in normalization of the aCL titers in one of the two reported cases. Azathioprine treatment reduced the aCL titer in the other patient, without fully normalizing it. Based on our observation, we propose to treat PAPS-associated severe and recurrent thrombo-embolic complications by aggressive immunosuppression, including azathioprine and cyclophosphamide.
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PMID:Primary antiphospholipid syndrome (PAPS). Two case reports and therapeutic implications. 204 85

In a 2-year prospective study of 146 patients with cerebral ischemia, we compared vascular risk factors for stroke with clinical and laboratory findings, particularly antiphospholipid antibodies. Ten patients (6.8%) were positive for at least one antiphospholipid antibody; one patient had systemic lupus erythematosus, one had rheumatoid arthritis, and the remaining eight fulfilled criteria for the diagnosis of primary antiphospholipid syndrome. These patients were predominantly male, not necessarily young, and 50% of them did not have any other vascular risk factors; there were no significant clinical or paraclinical differences between these patients and those without antiphospholipid antibodies. Outcome in the 10 patients was good, and platelet antiaggregating drugs proved to be useful in preventing further cerebrovascular ischemic events in our patients.
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PMID:Antiphospholipid antibodies in cerebral ischemia. 205 74

Habitual abortion is sometimes an incurable pathogenetic state. Even more serious to both the patient and gynecologist is that the incidence of repeated reproductive wastage is higher in subsequent pregnancies. We registered more than 1,000 women with repeated spontaneous abortions in this clinic for 5 years and analyzed their pathogenesis for the wastages by several different approaches. One hundred twenty-four women were diagnosed as having a congenital uterine abnormality. Metroplasty was performed in 50 patients, resulting in 82% successful subsequent pregnancies, whereas more than 98% of the pregnancies had terminated in spontaneous abortion before the operation. By measuring anticardiolipin antibody (ACA) and lupus anticoagulant (LA), the present study diagnosed several cases as antiphospholipid syndrome, and successfully pregnant patients were observed and treated with appropriate medications. Fifty-five partners of 54 couples had either a chromosomal abnormality or normal variants, showing a high incidence of spontaneous abortion in each type of abnormality. In the other 311 cases, female partners were treated by the immunotherapy of the husbands' lymphocytes in pre- and postgestational periods. Among them, 200 women became pregnant, and 147 cases successfully maintained the pregnancy. These studies indicate that early diagnosis through comprehensive examinations is essential for effective treatment of curable fetal loss in human reproduction.
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PMID:Current comprehensive therapy of habitual abortion. 205 75

The authors provide disease histories of 4 patients afflicted with genuine systemic lupus erythematosus (SLE). There were 2 men and 2 women who developed acute transverse myelitis (ATM) at an age of 24 to 31 years. Before ATM developed, SLE standing was from 3 to 10 years. Based on the authors' and reported data, the problems of the pathogenesis, clinical picture, diagnosis and prognosis in that grave complication of SLE are discussed. As for the mechanisms of ATM development, the role of antiphospholipid antibodies is reviewed. The data on familial aggravation of the patients are presented in terms of the development of the antiphospholipid syndrome in their relatives. Emphasis is laid on the necessity of early intensive care with glucocorticoid hormones and cyclophosphamide of patients with associated SLE and ATM in order to improve their prognosis.
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PMID:[Acute transverse myelitis in patients with systemic lupus erythematosus]. 206 59

Sneddon's syndrome consists of livedo reticularis and cerebral vascular accidents with no evidence of systemic disease responsible for the livedo. The syndrome has been assimilated to a subgroup of systemic lupus erythematosus (SLE) with presence of antibodies directed against phospholipids. Recently, a significant increase in the frequency of cardiac valve diseases has been demonstrated in some SLE patients with livedo reticularis, cerebral vascular accidents and antiphospholipid antibodies. We report the case of a 26-year old woman who had been presenting for 6 years with idiopathic livedo reticularis. Her history was remarkable for the occurrence of 2 cerebral ischaemic accidents at the ages of 23 and 26 years, generalized convulsive seizures at 22 years, and hypertension of pregnancy with 2 miscarriages. Biopsy of the livedo showed normal histological patterns, but electron microscopy detected an obliterating endothelial proliferation and endothelial cells with numerous Weibel-Palade bodies. Laboratory signs of SLE, as well as antiphospholipid antibodies were absent. At the age of 26 years, cardiac abnormalities were heard at auscultation for the first time, and echocardiography showed that they were due to a fairly loose mitral stenosis. According to Burton's criteria our patient had all the typical features of Sneddon's syndrome. The finding of mitral stenosis--an emboligenic cardiopathy that is potentially responsible for cerebral vascular accidents--raises the problem of its relationship with Sneddon's syndrome. The association does not seem to be fortuitous, since our case is very similar to the cases of SLE or antiphospholipid antibody syndrome associated with cardiac valve lesions. However, this case is particular in that 6 years after the onset of the disease there was still no sign of SLE and of antiphospholipid antibodies.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Livedo reticularis, cerebrovascular disorders and mitral disease: a new cause of Sneddon's syndrome?]. 208 86

Both normal and autoimmune mice have IgM natural autoantibodies to bromelain-treated erythrocytes in which phosphatidylcholine (PTC) becomes exposed. At one stage this antibody may participate in the genesis of autoimmune hemolytic anemia in the NZB mouse. We have recently studied a patient with hemolytic anemia who had persistently high serum titers of IgM anticardiolipin antibodies (aCL) that were also demonstrated in a hemolysate of his erythrocytes obtained at the time of the anemia. We affinity-purified the antibody and sought its binding to normal human bromelain-treated erythrocytes (BrE) because of the IgM isotype of the antibody, since cardiolipin is not a constituent of the erythrocyte wall, and because the anionic phospholipids, with which aCL are known to cross-react, are not located at the outer leaflet of the erythrocyte membrane. We found binding of the antibody to HBrE in their hemolysates and by flow cytometry. We also demonstrated that the aCL cross-reacted extensively with PTC, as well as with other anionic or zwitterionic phospholipids. The purified IgM antibody lysed BrE in the presence of complement and also bound to in vitro-aged erythrocytes. Because this patient had no other evidence of systemic lupus erythematosus or any other autoimmune condition, his disease may represent a variant of the recently described primary antiphospholipid syndrome.
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PMID:Hemolytic anemia related to an IgM autoantibody to phosphatidylcholine that binds in vitro to stored and to bromelain-treated human erythrocytes. 208 93

Recurrent thrombosis, abortion and thrombocytopenia are the most frequent manifestation of antiphospholipid syndrome, which usually presents antibodies against some anionic phospholipids. A few years ago, this syndrome was considered as a manifestation of systemic lupus erythematosus; nowadays it is classified as an isolated systemic lupus erythematosus; nowadays it is classified as an isolated autoimmune disease. Hypotheses have been proposed to explain the origin of antiphospholipid antibodies, including infectious and autoimmune diseases with high titles of anticardiolipin antibodies. Genetic factors have also been involved. Laboratory tests of choice for the detection of anticardiolipin antibodies are RIA and ELISA tests; there are some structural differences among them depending on the underlying disease. Hypercoagulability and recurrent thrombosis are the main features of this entity; ischemic heart disease is in this context of outmost importance. Despite the lack of any clinically demonstrated association between antiphospholipid syndrome and ischemic heart disease, there are many "in vitro" studies that support this possibility.
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PMID:[Antiphospholipid syndrome and cardiovascular disease]. 210 94

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


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