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

The description of a phospholipid syndrome in patients with systemic lupus erythematodes (SLE) filled up a gap in defining an "atypical" subgroup--quite often negative to DNA antibodies but suffering from arterial thromboses, thrombophlebitis, aseptic bone necroses, and pulmonary hypertension. 54 patients with SLE and 32 of their closet blood relatives were examined for lupus anticoagulation factor, anticardiolipin antibodies and Wassermann test. The results were interpreted in relation to the forms of the disease and its clinical manifestations. Clinically healthy relatives positive for circulating lupus anticoagulation factor or antiphospholipid antibodies are under close control.
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PMID:[The antiphospholipid syndrome in patients with systemic lupus erythematosus and in their closest blood relatives (a preliminary report)]. 212 71

A 52-year-old woman presenting with systemic lupus erythematosus (SLE) and a right recurrent laryngeal nerve palsy is described. The symptoms and signs resolved within days of initiating steroid therapy. This is the first reported case of recurrent laryngeal nerve palsy associated with SLE in the absence of pulmonary hypertension or laryngeal lupus.
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PMID:Systemic lupus erythematosus and right recurrent laryngeal nerve palsy. 237 50

The antiphospholipid syndrome--the association of venous and/or arterial thromboses, often accompanied by thrombocytopenia in the presence of the antiphospholipid antibodies ("lupus anticoagulant" antibodies to cardiolipin)--is seen mainly in patients with systemic lupus erythematosus (SLE) and the closely related "lupus-like" disease, i.e., lupus patients not conforming to the 1982 revised American Rheumatism Association classification for SLE. It is also seen in a group of patients who do not manifest any of the major clinical or serologic features of SLE, the majority of whom do not appear to progress to classical lupus. A multicenter study of 70 of these patients is documented and their major clinical and serologic characteristics examined: They have been characterized as suffering from a "primary" antiphospholipid syndrome and present typically with a history of deep vein thromboses, often accompanied by pulmonary thromboembolism, which in a few is complicated by thromboembolic pulmonary hypertension, arterial occlusions (most commonly strokes), or fetal loss. The events are often recurrent and may be accompanied by hemocytopenias (thrombocytopenia and less frequently Coombs positivity and/or hemolytic anemia). They are often antinuclear antibody-negative and are always negative for antibodies to dsDNA and to ENA, typical serologic features of SLE. There may be a family history of SLE or a familial clotting tendency in a minority. The group of patients presented appears to be closely related, but distinctly separate from SLE.
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PMID:The "primary" antiphospholipid syndrome: major clinical and serological features. 250 56

Antibodies to Extractable Nuclear Antigens (ENAs) namely Sm, nRNP, SS-A and SS-B were studied in 397 patients with various connective tissue diseases (CTD), 146 patients with inflammatory polyarthropathies, 16 cases of systemic vasculitides, and 39 normal subjects using counterimmunoelectrophoresis and double immunodiffusion methods. Anti-ENA antibodies were positive in 40.8 percent cases of Systematic lupus erythematosus (SLE) (n = 191), 36.4 percent of overlap CTD (OCTD, n = 44), 27.8 percent of Sjogren's syndrome (n = 18), 10.6 percent of progressive systemic sclerosis (PSS, n = 66) and 2.7 percent of rheumatoid arthritis (n = 111) patients. The correlation of these antibodies with disease features was done. The significant finding was negative association of anti-nRNP antibodies (when present alone) with renal involvement. Anti-Sm antibodies did not correlate with any disease feature. The other associations included correlation of anti-nRNP with pulmonary parenchymal lesions, anti-SS-A with serositis and pulmonary hypertension, and anti-SS-B with myocarditis and recurrent diarrhoea. We conclude that Anti-ENAs may correlate with certain subsets of these diseases but the subject is controversial.
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PMID:Antibodies to extractable nuclear antigens in connective tissue disorders in India: prevalence and clinical correlations. 262 64

We report a 34-year-old woman with mixed connective tissue disease (MCTD) who developed severe pulmonary and neuromuscular complications. At presentation, pulmonary function tests and pulmonary mechanics were suggestive of pulmonary vascular disease, and she subsequently developed clinical signs of pulmonary hypertension. These noninvasive tests may be useful in the timing of more invasive hemodynamic studies. She initially had myasthenia gravis and then developed polymyositis, profound peripheral neuropathy, and ventilatory muscle failure. She died despite aggressive immunosuppressive therapy and plasmapheresis. Autopsy showed spinal cord changes secondary to a peripheral neuropathy and signs of neurogenic atrophy confined to the ventilatory muscles. Peripheral neuropathy may be an important cause of ventilatory muscle weakness that can be found in MCTD and systemic lupus.
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PMID:Pulmonary and neuromuscular complications of mixed connective tissue disease: a report and review of the literature. 231 66

Two patients with the lupus anticoagulant exhibited unusual cutaneous manifestations. They both fulfilled four criteria for systemic lupus erythematosus and had experienced deep venous thrombosis. The first patient suffered from a leg ulcer that resembled a pyoderma gangrenosum. The second patient presented erythematous and purplish macules on the fingertips. The histologic studies showed only microthrombosis in the dermal vessels without vasculitis, although such lesions in systemic lupus erythematosus are usually attributed to vasculitis. The association of these cutaneous lesions with lupus anticoagulant has never been reported. It is likely that this association is not fortuitous. After a review of the literature, it seems possible to individualize a new syndrome characterized by the presence of a subgroup of antiphospholipid antibodies. Thrombosis, spontaneous abortions, neurologic manifestations, pulmonary hypertension, positive results of a Coombs' test, and thrombocytopenia can be included in this syndrome, which overlaps with systemic lupus erythematosus. Certain cutaneous symptoms are associated with the presence of lupus anticoagulant or other antiphospholipid antibodies: leg ulcers, distal cutaneous ischemia, widespread cutaneous necrosis, and livedo. They can be considered as the dermatologic manifestations of this syndrome.
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PMID:Cutaneous manifestations associated with the presence of the lupus anticoagulant. A report of two cases and a review of the literature. 309 56

Recent reviews have suggested a higher frequency of the lupus anticoagulant or related antiphospholipid antibodies in patients with systemic lupus erythematosus (21% to 65%) than was found in earlier studies (6% to 18%). In our study of 60 consecutive patients, we found the frequency of the lupus anticoagulant by Russell viper venom time was 6.7% (95% confidence interval, 16.2 to 1.8) and by anticardiolipin antibody assay was 25% (95% Cl, 37.0 to 15.7), compared with 0% (p = not significant) and 2.5% (p = 0.002), respectively, in the normal control population. The Russell viper venom time (p = 0.0001 by t-test) and anticardiolipin antibody levels (p = 0.01) were significantly associated with presumed thrombotic events (stroke, deep venous thrombosis, and digital gangrene). No association with miscarriage or pulmonary hypertension was detected. The Russell viper venom time was more specific than the anticardiolipin antibody level in the prediction of past presumed thrombotic events, miscarriage, or pulmonary hypertension (100% compared with 84%, p = 0.01).
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PMID:The frequency of lupus anticoagulant in systemic lupus erythematosus. A study of sixty consecutive patients by activated partial thromboplastin time, Russell viper venom time, and anticardiolipin antibody level. 310 10

The authors present some literature and their own data on the antiphospholipid syndrome (venous and arterial thromboses; obstetric pathology: spontaneous abortions and intrauterine death of a fetus; CNS involvement: from headaches and disorientation to psychic disorders and cerebral infarctions; thrombocytopenia, early aseptic osteonecrosis and pulmonary hypertension). Altogether 58 patients with lupus erythematosus and 20 patients with a complicated obstetric history were examined. Lupoid anticoagulant and antibodies to cardiolipin were revealed in 27 patients. The determination of antiphospholipid antibodies was shown to be of practical value from the view-point of the knowledge of humoral immunity disorders in systemic lupus erythematosus, more detailed clinico-laboratory characterization of patients, the diagnosis and prediction of disorders of the blood coagulation system, and the development of obstetric pathology.
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PMID:[Clinical value of determining the levels of lupus anticoagulant and anti-cardiolipin antibodies]. 314 84

The patient, a 30 year-old Caucasian female with a 6-year history of systemic lupus erythematosus was suspected of having pulmonary hypertension following chest X-Ray and routine echocardiography. Whilst awaiting further cardiological investigations she developed acute respiratory distress accompanied by gross signs of cor pulmonale and died despite full intropic and ventilatory support, in addition to intravenous "Pulse" methyl prednisolone. Postmortem findings showed typical features of the adult respiratory distress syndrome ('ARDS') but minimal vascular changes attributable to the pulmonary hypertension. The 'ARDS' was presumably associated with an acute 'flare' of the lupus.
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PMID:Systemic lupus erythematosus, pulmonary hypertension and adult respiratory distress syndrome (ARDS). 318 May 53

Antiphospholipid antibodies (APAB) were first detected as a result of the disturbances they cause in routine biological tests. These effects include lupus anticoagulant (LA) in APTT test and false serological reactions for syphilis in VDRL test. With more sensitive techniques using purified phospholipid antibodies (ELISA, RIA) antibodies directed specifically against cardiolipidin or other phospholipids can be assayed. Beside being responsible for positivity of the VDRL test in the context of syphilis, APAB (false serological reactions for syphilis, LA, anticardiolipin antibodies) have also been detected in systemic lupus erythematosus (SLE) and lupus-like syndromes, after intake of certain drugs, and, more rarely, in a number of diseases (table I). The specificity of APAB detected in syphilis (antiphosphatidylcholine) is different from that of APAB detected in SLE (antiphosphatidylserine and anticardiolipin). A given APAB can react with several phospholipids, but such crossreactions do not occur haphazardly with all phospholipids. Crossreactions are observed between APAB and polynucleotides on the one hand, and between antinuclear antibodies and phospholipids on the other. This pattern has led to speculate that APAB are directly involved in the pathogenesis of SLE; however since these crossreactions are not extensive, such speculation is controversial. The presence of APAB in the form of LA, false serological reactions for syphilis or anticardiolipin antibodies is associated with a number of problems including recurrent thrombosis, repeated abortions, thrombocytopenia and positive Coombs' tests or, more rarely, pulmonary hypertension, migraine, epilepsy, chorea, and transverse myelitis.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Antiphospholipid antibodies: new data in the pathogenesis of collagenosis]. 354 43


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