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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Since 1981, 21 children diagnosed with mixed connective tissue disease (MCTD) have been reported in detail in the literature in Japan. Overlapping clinical features and laboratory findings of these children were analyzed according to the established criteria of the Ministry of Health and Welfare, Japan. Mixed connective tissue disease in childhood typically begins with Raynaud's phenomenon preceded by several months or years with the appearance of other symptoms and signs including fever, arthralgia, myalgia, and/or progressive systemic sclerosis like skin manifestations. Serologically all the children with MCTD were positive for anti-ribonuclear protein (RNP) antibody with speckled-type antinuclear antibody. Hypergammaglobulinemia, positive rheumatoid factor, and normocomplementemia were characteristic. In general, prognosis is considered to be fairly good as opposed to systemic lupus erythematosus in childhood, but severe pericarditis/myocarditis or nephrotic syndrome can occur. Long-term follow-up study and improved laboratory detection of anti-ribonucleoprotein antibodies will be necessary for further characterization of MCTD in childhood, and for the improvement of therapy.
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PMID:Mixed connective tissue disease in childhood. 825 33

Systemic lupus erythematosus (SLE) affects approximately 0.6 children per 100,000. The disease is extremely rare in children under 5 years of age and is diagnosed predominantly in adolescent females. Children tend to present with more severe multisystem involvement than adults. Pericarditis occurs in approximately 25% of patients with SLE in all age groups. Progression to tamponade is extremely uncommon in the pediatric population. In the current report, an adolescent girl is diagnosed with SLE after presenting with signs and symptoms consistent with cardiac tamponade. A review of other pediatric patients with a similar presentation is also included.
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PMID:Cardiac tamponade in an adolescent female: an unusual manifestation of systemic lupus erythematosus. 825 15

To study the cardiac involvement in systemic lupus erythematosus, the clinical and necroscopy records of 29 patients were analyzed. Of these, 28 were female and 1 was male with a mean age of 28.6 +/- 10.4 years. The mean duration of the disease was 23.8 +/- 20.1 months. Twenty patients had the diagnosis of renal failure, 11 of arterial hypertension and 8 of congestive heart failure. No valvular or coronary arterial disease was diagnosed in any patient. The most frequent cause of death was sepsis, followed by renal failure. Only one patient died of causes directly related to heart disease. At necroscopy, 12 patients had pericarditis and 10 had cardiomegaly. Nonspecific valvular abnormalities were observed in 2 patients, Libman-Sacks' endocarditis in 1 and bacterial endocarditis in 2. Cardiovascular abnormalities were absent in only 8 (7.5%) patients. It was concluded that although frequent and contributing to mortality in some patients, cardiovascular involvement in systemic lupus erythematosus is multifactorial and, usually, nonspecific.
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PMID:[Cardiac involvement in systemic lupus erythematosus: anatomo-pathological study]. 828 Dec

A 40-year-old woman suffered from toxemia of pregnancy in 1977 and was admitted to hospital. Thereafter, she developed nephrotic syndrome, underwent a renal biopsy, and a diagnosis of membranoproliferative glomerulonephritis (MPGN) was made. She received steroid therapy, immunosuppressive drug and anticoagulant therapy, and recovered sufficiently to be discharged from hospital in April, 1979. During subsequent ambulatory treatment at our outpatient department, her renal function deteriorated gradually, and maintenance hemodialysis was started from June, 1990. In July, 1991, she was admitted to our hospital with pleurisy and pericarditis. There was no improvement despite antibiotic treatments. Laboratory data revealed leukopenia and lymphopenia. Under suspicion of systemic lupus erythematosus (SLE), relevant tests were carried out. Immunological abnormalities such as positive LE cells and the presence of various autoantibodies, together with clinical signs of hypersensitivity to sunlight, stomatitis and serositis, satisfied the diagnostic criteria of the ARA and a diagnosis of SLE was made. This case did not exhibit any clinical or serological abnormalities except for the renal disorder for a 10-year period after the histological diagnosis of MPGN, but was eventually diagnosed as SLE as a result of the manifestation of SLE symptoms for the first time after one year of maintenance hemodialysis. Immunological abnormalities and SLE during maintenance hemodialysis are discussed in relation to other reports.
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PMID:A case complicated with SLE during maintenance hemodialysis. 834 Oct 22

The authors present the case of a 47-year old female patient. She suffered from a moderate activity SLE for two years. The outcome of SLE was changed by an ulcer because of leg injury, which was repeatedly infected. Severe polyarthritis, leucopenia, thrombocytopenia, clinical signs of vasculitis and in the last two months fever, high ESR, and pericarditis appeared. The angina pectoris, the cardiac decompensation, the electrocardiogram, the echocardiogram was suspect to diffuse myocardial lesion. Cardiac decompensation caused the death of the patient. Besides the activation of her autoimmune disease, infection was suspect in the patient taken immunosuppressive and steroid drugs, though it could not be verified. Autopsy verified besides the recent necrosis of heart without reaction, and multifocal myocardial abscess, which appeared possible in the terminal phase.
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PMID:[Fatal myocardial lesion in systemic lupus erythematosus]. 824 23

We describe a 62-year-old woman in whom systemic lupus erythematosus presented as life-threatening effuso-constrictive pericarditis. Surgical drainage of the pericardium was required and the patient made a satisfactory recovery. At six-months follow-up, while taking hydroxychloroquine and a non-steroidal anti-inflammatory agent, she remains well.
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PMID:Systemic lupus erythematosus presenting as effuso-constrictive pericarditis. 854 94

Two patients (a boy and a girl), with systemic lupus erythematosus (SLE), in which pericarditis with threatening tamponade was an initial symptom of disease, are presented. Pericardial tamponade is very rare initial manifestation, described in only 1-3% of all the cases of SLE. The typical clinical features of tamponade are described (tachycardia, hypotension, venous congestion) and the importance of echocardiography in early diagnosis of pericarditis and pericardial tamponade is pointed out. Therefore, the echocardiography is considered as the complementary method in diagnosis of SLE.
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PMID:[Incipient pericardial tamponade as the first symptom of systemic lupus erythematosus in 2 children]. 869 2

To assess the impact of demographic and clinical factors on prognosis in patients with systemic lupus erythematosus (SLE), we examined the survival rate by lifetable analysis in 566 patients. All patients were Shanghai citizens who were diagnosed as having SLE in Huashan Hospital between 1959 and 1992. According to American Rheumatism Association's preliminary criteria. The survival rate from the time of SLE onset was 93% at 1 year, 73% at 5 years and 60% at 10 years. On univariate analysis, we found that the following factors worsened the probability of survival; male, neuropsychiatric manifestation, pleurisy-pericarditis, anemia, thrombocytopenia, lymphocytopenia, proteinuria, hematuria, urinary cast, azotemia, decreased endogenous creatinine clearance, increased cholesterol in serum, hypocomplementemia, abnormal electrocardiograph and high corticosteroid dose of treatment. On multivariate analysis, we found the four independent risk factors were male, azotemia, hypocomplementemia and high corticosteroid dose of treatment.
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PMID:[Survival rates in patients with systemic lupus erythematosus]. 869 80

We describe a patient with systemic lupus erythematosus (SLE), whose pregnancy was complicated by fulminant lupus pneumonitis and pericarditis. Maternal disease responded to therapy and twin girls were delivered, both with thrombocytopenia, one of whom died of an intraventricular haemorrhage. Pneumonitis is a rare complication of lupus in pregnancy which may be fatal. We suggest patients with previous severe pneumonitis should have lung function tests at the onset of pregnancy, and treatment be modified to suppress flare if there is any indication of severe pneumonitis in early pregnancy.
Lupus 1996 Apr
PMID:Pneumonitis in a lupus twin pregnancy: a case report. 874 28

A 56-year-old Japanese woman had a 8-year history of systemic lupus erythematosus (SLE) with recurrent flares. When she was 48 years old, she was diagnosed as having SLE on the basis of fever, polyarthritis, oral ulcers, leukopenia, and positive anti-DNA antibody. Three years later she developed pericarditis and pleuritis, that were improved with treatment with 30 mg of prednisone a day. With tapering of prednisone dose to 9 mg a day in January 1993, she was admitted due to shortness of breath on exertion. Chest radiograph revealed bilateral elevated diaphragms and sluggish movement with clear lung field. Pulmonary function tests showed restrictive defect with a vital capacity 38% of predicted value. A diagnosis of "shrinking lung syndrome" was made. Simultaneously, blood test revealed leukopenia, elevated red cell sedimentation rate, and elevated anti-DNA antibody titer. Therefore, we suspected this pulmonary involvement was related to lupus flare-up. Treatment with 20 mg of prednisone a day resulted in resolution of the patient's dyspnea and in improvement of her vital capacity. Corticosteroid therapy for acute "shrinking lung syndrome" in active SLE can improve symptoms and pulmonary function.
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PMID:[A case of the "shrinking lung syndrome" in SLE--improvement with corticosteroid therapy]. 881 May 47


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