Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report the observations of 4 young women suffering from SLE witha circulatig antiprothrombinase anticoagulant. Antiprothrombinase is the most frequent circulating anticoagulant found in SLE (5 to 10 p. 100). SLE is the main aetiology for antiprothrombinase (over 50 p. 100). It is called 'lupus anticoagulant'. Some symptoms seem to be more frequent in SLE with antiprothrombinase. Such are biological signs (false positive tests for syphilis. Coombs test, thrombopenia, prothrombin deficiency) and clinical signs (venous or arterial thrombosis particularly if oestroprogestative treatment is taken, bleeding if thrombocytopenia or deficiency of prothrombin; repetitive abortion and may be neuropsychiatric signs). Antiprothrombinase is an autoantibody (IgG or IgG + M) polyclonal in SLE, with antiphospholipid activity. It could decrease the production of prostacyclin (PGI2) from free arachidonic acid derived from membrane bound phospholipids. Immunological properties of antiprothrombinase could account for clinical and biological associated signs.
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PMID:[Antiprothrombinase type of circulating anticoagulants during acute disseminated lupus erythematosus]. 698 16

Eleven patients with 18 pregnancies occurring during the course of systemic lupus erythematosus (SLE) were reviewed. Ten had long-standing lupus glomerulonephritis and a single patient developed glomerulonephritis during pregnancy. Patients were divided into those without (Group A) and those with (Group B) clinical evidence of renal disease or active SLE at conception. In Group A there were 10 pregnancies in five patients; all pregnancies were uncomplicated, except for mild superimposed pre-eclampsia in two, and all resulted in term delivery. Eight pregnancies in six patients occurred in Group B; four pregnancies were complicated by severe (2) or mild (1) superimposed pre-eclampsia and the onset of glomerulonephritis (1), resulting in three premature deliveries and a spontaneous abortion. The remaining four pregnancies were uncomplicated but resulted in one term delivery, one elective abortion, and two spontaneous abortions. None of the patients developed either renal failure or a rapidly progressive course following pregnancy.
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PMID:Pregnancy and systemic lupus erythematosus. 742 97

We describe the case of a patient with primary familiar antiphospholipid syndrome and acute myocardial infarction. A previously healthy 15-year-old adolescent was admitted with severe chest pain lasting from 1 hour associated with inferoposterolateral ST-segment elevation. The patient received intravenous thrombolysis. A 2-dimensional echocardiogram revealed an area localized in the basal posterolateral left ventricular myocardium, that was akinetic and abnormally thin throughout the cardiac cycle. Peak creatinine kinase level was 1461 U/I. Subsequent electrocardiogram revealed inferoposterior infarction. Plasma anticardiolipin (aCL) IgG antibodies resulted positive (24 U.GPL) in repeated determinations. A dypiridamole echocardiographic test resulted negative. The patient's parents refused cardiac catheterization. He continues to do well at home 28 months after discharge. The patient's sister is affected by primary antiphospholipid syndrome characterized by recurrent abortion, very low platelet count and lupus anticoagulant positivity. Plasma aCL antibodies resulted positive also in the mother who did not have clinical manifestations.
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PMID:[Primary antiphospholipid syndrome with a familial element and myocardial infarct in an adolescent]. 749 21

Antiphospholipid syndrome is a well-defined clinical and serological entity characterized by venous and arterial thrombosis, recurrent abortion and thrombocytopenia. The immunological feature shows the presence of anticardiolipin antibodies and lupus anticoagulant. Cardiac involvement includes pericardial effusion, myocardial dysfunction, valvular disease, coronary artery occlusion. The literature reports only a few cases of intracardiac thrombosis associated with primary antiphospholipid syndrome. Here we describe a case of a 54 year-old woman with thrombocytopenia and history of recurrent abortions. When she was 51 she presented a left deep iliac venous thrombosis, followed by recurrent pulmonary embolism; a caval filter was placed in the inferior vena cava. Transthoracic and transoesophageal echocardiogram showed 3 masses in the right atrium: the first one was connected with the atrial wall, hyperechogenic and extending to the right ventricle in diastole: th second was connected with the interatrial septum and less echogenic; the third was in connection with septum-tricuspid valve junction and it was floating in the atrium. The immunological feature showed the presence of lupus anticoagulant and antiphospholipid antibodies; the histological examination of the masses, surgically removed, proved they were thrombi coated by endothelial cells. The case reported is very uncommon and offers the opportunity to emphasize the difficulty of diagnostic differentiation of intracardiac masses using echocardiographic imaging: thrombotic masses may present similar characteristics of seat, morphology and echogenicity of other cardiac masses. For these reasons it can be useful to look for antiphospholipid antibodies and lupus anticoagulant in such clinical conditions.
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PMID:[Primary antiphospholipid syndrome and cardiac involvement. Description of a clinical case of right atrial thrombosis]. 770 May 44

The antiphospholipid syndrome (APS) is usually defined by the association of a clinical manifestation (venous or arterial thrombosis or miscarriage) with the presence of antiphospholipid antibodies (lupus anticoagulant and/or anticardiolipin antibodies). It frequently occurs in the course of systemic lupus erythematosus but is also encountered as a "primary" disease. APS is responsible for diverse respiratory manifestations. Pulmonary embolism is common. The site of the causal venous thrombosis is frequently unusual. Pulmonary hypertension may be a consequence of repeated embolism or may belong to the primary idiopathic variety. Pulmonary manifestations may also result from left-sided heart failure due to mitral or aortic valve abnormalities, myocardial infarction or a specific myocardiopathy. APS is probably involved in the occurrence of some cases of adult respiratory distress syndrome. Long term secondary prevention of recurrent thrombosis is a central point in the management of APS.
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PMID:[Antiphospholipid syndrome and the pneumologist]. 772 79

Bone marrow necrosis (BMN), defined morphologically by destruction of hematopoietic tissue, including the stroma, with preservation of the bone, is a rare syndrome. The conditions in which it is seen include sickle cell disease, acute leukemia, metastatic neoplasia, and bacterial infection, particularly when hypovolemia and septic shock are present. BMN is also associated with disseminated intravascular coagulation (DIC) following irradiation and antineoplastic therapy. The antiphospolipid syndrome (APS) is characterized by antibodies directed against the antiphospolipid substrate. Because this substrate is prominently involved in the coagulation cascade and widely distributed on cell walls, patients present with venous or arterial thromboses, recurrent abortion, thrombocytopenia, and Coombs' positive hemolytic anemia, typically with raised anticardiolipin antibodies or a diagnostic lupus anticoagulant test. BMN does not appear to have been previously recognized in this context. We report what we believe to be the first such case and suggest that the high titers of antibodies present may have played a central role in its pathogenesis.
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PMID:Extensive bone marrow necrosis associated with antiphospholipid antibodies. 777 73

The antiphospholipid syndrome may occur either in association with systemic lupus erythematosus (SLE) or as a primary condition. Diagnostic criteria include the detection of antiphospholipid antibodies using cardiolipin antigen (anticardiolipin antibodies, ACA) or lupus anticoagulant, and at least two clinical manifestations, primarily venous or arterial thrombosis, thrombocytopenia or repeated miscarriage. Among 200 patients with various rheumatic diseases, a pathological ACA test in excess of 44 U/ml was detected with ELISA in 81 cases (41%), and of these 30% had values exceeding 100 U/ml. Both overall positive reactions and the particularly high titers were clearly more common among patients with rheumatoid arthritis (RA) and collagen disease, namely 51% and 33%, respectively, than in patients with sero-negative spondylarthritis and non-inflammatory diseases of the locomotory system (22% pathological, 13% highly pathological, titers. Correlations were also found with miscarriage in women, positive rheumatoid and antinuclear factors, and the number of diagnostic criteria of RA or SLE presenting. A questionnaire survey - evaluating 84 subjects, half with positive and half with negative, ACA reactions - confirmed the correlation between miscarriage and the detection of ACA.
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PMID:[Clinical relevance of the antiphospholipid syndrome. Antiphospholipid antibodies in a mixed rheumatologic patient sample]. 780 18

The present study was conducted to elucidate the clinical significance of autoantibodies in infertility. Among 203 cases of infertility, 27 cases (A group) were positive for antinuclear antibodies (ANA), and 18 cases (B group) were positive for antiphospholipid antibodies (APA) regardless of the presence of ANA. The progress of pregnancy over time in the study period was clarified in 13 cases in A group and 12 cases in B group. Although only luteal support was given to the A group, appropriate for gestational age babies were obtained in all cases except 3 cases in which there occurred early abortion. In B group, babies were obtained successfully in 8 cases by steroid-aspirin therapy, but intrauterine fetal death occurred in the second trimester in 2 cases, and in the other 2 cases early abortion occurred. In cases positive for the antibody (beta 2(-)ACA) to cardiolipin, fetal distress did not occur in any of the 3 cases. On the other hand, in cases positive for the antibody (beta 2(+)ACA) to the cardiolipin-beta 2-glycoprotein I complex and/or lupus anticoagulant (LA), marked fetal distress occurred in all except one of the 7 cases. In conclusion, there was little correlation between ANA, beta 2(-)ACA and infertility, suggesting that the cause of infertility is the induction of placental circulating disorder by beta 2(+)ACA and LA.
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PMID:[Effect of autoantibodies on women with infertility]. 784 38

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

In order to evaluate the presence of antiphospholipid antibodies (aPL) in patients with systemic lupus erythematosus (SLE), we measured IgG and IgM anticardiolipin antibodies (aCL) in a group of out- and in-patients of the Rheumatology Division at the Catholic University of Rome. Lupus anticoagulant and VDRL were also measured in 30 patients. One hundred thirteen women and 7 men (mean age 38.5 years, mean disease duration 4.27 years) were studied. The control group consisted of 60 age and sex matched healthy subjects. aCL IgG and IgM were positive in 48 (40%) and 49 (40.8%) patients respectively. aCL IgG correlated positively with the activated partial thromboplastin time. aCL IgM correlated positively with immunoglobulins of the G and M classes and with circulating immune complexes, and negatively with the C4 fraction. aCL antibodies did not correlate with spontaneous abortion or neurologic and psychiatric disturbances, although in women with a history of abortion, aCL IgG concentration was correlated with the number of spontaneous abortions. The presence of aPL does not seem to indicate a subgroup of SLE patients. The occurrence of abortion and/or neurologic and psychiatric disturbances in SLE seems to point to a complex pathogenesis with aPL as one of the causative agents.
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PMID:[Antiphospholipid antibodies in systemic lupus erythematosus: study of a caseload of 120 patients]. 789 73


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