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)

Venous gangrene of the upper extremity is a rare entity and is the result of massive occlusion of all venous outflow of the extremity. The syndrome is strongly associated with hypercoagulable states including malignancy, low cardiac output states, and hereditary or acquired hematological abnormalities. Diagnosis can be straightforward but must be made early in the course of the process for treatment to be effective. Treatment has historically produced only modest results, and patients continue to suffer a high morbidity and mortality. We present a series of 6 patients with venous gangrene or impending venous gangrene of the upper extremities--a relatively large series. Two patients suffered from malignancy, 3 patients suffered from low-flow cardiac states, and 1 patient suffered from an overdose of calcium channel blockers. Hematological abnormalities included heparin-induced thrombocytopenia and thrombosis in 3 patients, activated protein C resistance in 1 patient, and lupus anticoagulant in 1 patient. Three patients experienced other major venous thrombotic complications, two of whom died (renal and cerebral venous infarction). Venous gangrene of the upper extremity remains a rare occurrence but one in which early identification and intervention may lead to improved outcomes.
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PMID:Venous gangrene of the upper extremity. 955 91

A case of intestinal perforation associated with SLE is presented. A 54-year-old woman was diagnosed as having SLE twenty-five years ago when she had facial erythema, photosensitivity, oral aphtha, polyarthraliga, leukopenia, positive LE cell and positive antinuclear antibody. She had been treated with prednisolone and admitted to Kushiro City General Hospital because of one month history of fever and anorexia in February 1996. Laboratory findings did not reveal activity of SLE, and a diagnosis of urinary tract infection was made based on the findings of urinalysis. After severe diarrhea, disseminated intravascular coagulation (DIC) developed. A rectal perforation was revealed by endoscopic and radiological examination. An emergency laparotomy revealed necrosis of the rectum and sigmoidostomy was performed. The biopsied specimen of the rectum were diagnosed as gangrene of ischemic colitis histologically. Because of a penetration to the urinary bladder, an ureterocutaneostomy was performed. She died of sepsis and DIC on the 127th day of admission. Only 11 cases of intestinal perforation associated with SLE have been reported in Japan, and the association of vasculitis has been considered. In the present case, the prolonged use of prednisolone might cause the necrotizing ischemic colitis.
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PMID:[A case of systemic lupus erythematosus developed with intestinal perforation]. 972 61

Antiphospholipid (aPL) syndrome, or APS,--a cluster of conditions that includes arterial or venous thromboses and thrombocytopenia, as well as recurrent fetal loss associated with elevation of aPL antibody--has been reported to occur 2-5 times more frequently in women than men. Strong familial associations lead to the suspicion that aPL positivity, estimated to be present in 2% of the population, is a heritable trait in some cases. Currently, 2 major categories of the illness are recognized--primary and secondary. Secondary APS may be associated with autoimmune disease, malignancy, infectious disease, or drug-induced states. Two assays, one for lupus anticoagulant antibodies and the other for anticardiolipin (aCL) antibodies, are recognized to be the gold standards for serologic diagnosis of the disease. Despite extensive attempts at international standardization of aCL test results, no consensus exists for a value beyond which the test is considered positive. Interestingly, a "dose-effect" relationship for aCL antibody titers has been noted--higher titers of the antibody correlate with increased numbers of thrombotic events. An experimental assay for antibody against beta 2-glycoprotein 1 (beta-2-GP1), a phospholipid-binding protein, may become the most important assay for aPL. Skin findings in APS include livedo reticularis, ulceration, gangrene, or purpura, and, when present, may be the key to diagnosis of this sometimes insidious syndrome. Anticoagulation, usually with warfarin, is the mainstay of therapy, although steroids, immunosuppressive agents, hydroxychloroquine sulfate, and plasmapheresis may all be beneficial adjunctive therapy.
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PMID:Impact of the Antiphospholipid Syndrome: A Critical Coagulation Disorder in Women. 974 73

We assessed the mucocutaneous signs in 57 children with classical systemic lupus erythematosus seen during a 6 year period. The female:male ratio was 4.2:1. Ages ranged from 4 to 15 years (mean 11.9 years) at the time of diagnosis. Cutaneous manifestations (77%) were the second most common finding, next to renal involvement (84%). The skin changes noted were malar rash (74%), oral ulcer (46%), vasculitis (42%), photosensitivity (40%), alopecia (32%), and discoid lupus erythematosus (LE) (19%). All 11 discoid LE patients were girls. Periungual erythema, Raynaud's phenomenon, periungual gangrene, nail involvement, and subacute LE were rare. Antinuclear antibody reaction was positive in 93% and anti-dsDNA was positive in 46%. Eight patients died, six from severe infection and two from renal failure.
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PMID:Cutaneous manifestations of childhood systemic lupus erythematosus. 979 81

Lupus-like anticoagulant is commonly encountered in human immunodeficiency virus infection although thromboembolic manifestations are rare in HIV patients. We report the case of an HIV patient who developed gangrene of both forefeet associated with anticardiolipin antibodies. A 42-year-old woman had a 12-year history of HIV infection (stage B2). She presented with painful gangrene involving the forefeet of 4-day duration. Doppler ultrasonography, electromyography and nailfold capillaroscopy were normal. Skin biopsy revealed intracapillary thrombi and severe necrosis within the hypodermis; there was no evidence of vasculitis. Laboratory findings showed a marked inflammatory syndrome and the presence of anticardiolipin antibodies (IgG: 22 GPL U/ml). Several cutaneous manifestations are known to be associated with antiphospholipid syndrome, such as livedo reticularis, ulcers and gangrene of the extremities. Skin biopsy often shows noninflammatory thrombosis of small vessels within the dermis. Microcirculation damages have also been described in HIV infection, mainly vasculitis. In the present case report, the absence of both vasculitis and other causes suggest that anticardiolipin could be the culprit. But, it is possible that painful gangrene of the forefeet was secondary to HIV infection.
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PMID:[Are antiphospholipid antibodies thrombogenic in the course of human immunodeficiency virus infection?]. 1019 38

Malignant neoplasms are associated with a wide variety of paraneoplastic rheumatological syndromes. Among these, hypertrophic osteoarthropathy, carcinoma polyarthritis, dermatomyositis/polymyositis, and paraneoplastic vasculitis are the most frequently recognized. Other less known associations are based upon a smaller number of reported patients, and include fasciitis, panniculitis, erythema nodosum, Raynaud's syndrome, digital gangrene, erythromelalgia and lupus-like syndromes. Musculoskeletal manifestations of malignancy may coincide, follow or antedate the diagnosis of cancer, or herald its recurrence. The clinical course generally parallels that of the primary tumour, and treatment of the underlying malignancy often results in regression of the rheumatic disorder. Awareness that cancer can cause certain non-metastatic symptoms is important for early diagnosis and treatment of an occult neoplasm. Rheumatic manifestations suggesting a hidden cancer include: rapid onset of an unusual inflammatory arthritis clubbing or diffuse bone pains in a patient 50 years of age or older, chronic unexplained vasculitis, refractory fasciitis, Raynaud's syndrome unresponsive to vasodilator therapy, rapidly progressive digital gangrene or Lambert-Eaton myasthenic syndrome. Management consists of control of the underlying cancer and symptomatic treatment of the rheumatic syndrome with non-steroidal anti-inflammatory drugs or corticosteroids.
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PMID:Paraneoplastic rheumatic syndromes. 1098 84

To examine the possible relationship between anti-centromere antibodies (ACA) and pediatric rheumatologic diseases, we investigated the presence of ACA (using enzyme immunoassay) in the sera of 45 children and adolescents with such diseases and compared the results with a group of 42 age- and gender-matched healthy subjects. ACA were present ( > or =10 U/ml) in three out of five patients (60%) with scleroderma (SCD), in seven out of 16 (43.8%) patients with systemic lupus erythematosus (SLE), in two out of five patients (40%) with mixed connective tissue disease (MCTD), in one out of four patients (25%) with dermatomyositis (DMS), and in two out of 14 patients (14.3%) with juvenile rheumatoid arthritis (JRA). ACA were also detected in a single patient with anti-phospholipid syndrome (APL) who had digital gangrene and hemiparesis, as well as in two healthy subjects. ACA positivity was related to the presence of Raynaud's phenomenon in the studied sample, as 86% of patients suffering from the phenomenon were ACA positive. ACA positivity was associated with older age, high blood pressure and high erythrocyte sedimentation rate (ESR) values, and lower hemoglobin and weight and height percentile values. It was also higher among anti-nuclear antibody-positive subjects. Raynaud's phenomenon and ACA positivity shared almost the same clinical and laboratory associations in the studied patients. Thus, ACA are probably among the markers of Raynaud's phenomenon in pediatric rheumatologic diseases. Their value as predictors of future development of the phenomenon needs further evaluation.
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PMID:Anti-centromere antibodies as a marker of Raynaud's phenomenon in pediatric rheumatologic diseases. 1111 May 81

A case of digital gangrene in a patient with systemic lupus erythematosus without secondary anti-phospholipid syndrome is reported. The acute onset of the necrotizing acrovasculitis occurred without a history of Raynaud's phenomenon. Preceding symptoms of the systemic lupus erythematosus were arthritis, photosensitivity, alopecia and anorexia. Despite the seriousness of the acute episode the patient achieved an excellent outcome.
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PMID:Digital gangrene: a rare skin symptom in systemic lupus erythematosus. 1130 90

A patient with end stage renal disease secondary to systemic lupus erythematosus (SLE) ultimately required amputation of the four extremities and developed mesenteric ischemia. The patient presented with widespread medial calcification involving various small to medium sized arteries, although no noticeable secondary hyperparathyroidism was observed. We speculated that SLE associated with systemic vasculitis and uremic milieu over a number of years may represent the perfect preexisting condition for calcific arteriolopathy to occur following which several factors including chronic administration of corticosteroids, photosensitivity in lupus, and significant weight loss may have contributed to acral gangrene and mesenteric ischemia.
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PMID:Calciphylaxis in a patient with end-stage renal disease secondary to systemic lupus erythematosus associated with acral gangrene and mesenteric ischemia. 1181 38

Patients with systemic lupus erythematosus (SLE) may develop premature atherosclerosis, notably peripheral vascular disease (PVD) presenting with intermittent claudication or gangrene. Therefore, it is important to investigate if high prevalence of poor muscle perfusion of lower extremities in SLE patients with abnormal myocardial perfusion is related to more cardiovascular risk factors. We used a well-established and noninvasive radionuclide method (xenon 133 muscle washout) to evaluate objectively the anterior tibial muscle perfusion of 34 SLE female patients without symptoms/signs of PVD in the lower extremities. The patients were separated into two groups according to myocardial perfusion imaging results. Meanwhile, 30 normal female controls with matched age distribution were also included for comparison. The muscle perfusion differed significantly ( P <0.05) between patients (1.90+/-0.41 ml/100 g per min) and controls (2.91+/-0.50 ml/100 g per min), as well as between 18 SLE patients with abnormal myocardial perfusion (1.33+/-0.43 ml/100 g per min) and 16 with normal myocardial perfusion (2.26+/-0.45 ml/100 g per min). Based on the xenon 133 muscle washout method, we conclude that muscle perfusion in the lower extremities of SLE patients without symptoms/signs of PVD is significantly decreased and related to abnormal myocardial perfusion.
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PMID:High prevalence of asymptomatically poor muscle perfusion of lower extremities measured in systemic lupus erythematosus patients with abnormal myocardial perfusion. 1287 74


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