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)

An 83-year-old woman with histologically confirmed Hunner's cystitis presented with persistent fever, progressive dyspnea, and pulmonary patchy infiltrates. A transbronchial biopsy specimen revealed bronchiolitis obliterans organizing pneumonia. She progressively had development of renal insufficiency, due to systemic lupus erythematosus, proved by renal biopsy specimen. She recovered under corticosteroid treatment, but irreversible renal failure made long-term hemodialysis necessary.
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PMID:The association of bronchiolitis obliterans organizing pneumonia, systemic lupus erythematosus, and Hunner's cystitis. 833 71

The primary antiphospholipid/anticardiolipin syndrome is a recently described entity wherein multiorgan thrombotic events occur in the absence of objective evidence of systemic lupus erythematosus. The spectrum of renal involvement remains poorly described. Two patients with coagulation abnormalities consistent with the primary antiphospholipid/anticardiolipin syndrome who developed profound renal insufficiency are reported. Striking microangiopathic lesions were documented on renal biopsy. Renal function improved concomitant to the institution of steroid therapy. Reversible renal failure should be added to the spectrum of clinical manifestations of this entity. The diagnosis of the primary antiphospholipid/anticardiolipin syndrome should be contemplated in individuals with unexplained acute renal failure.
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PMID:Reversible renal failure in the primary antiphospholipid syndrome--a report of two cases. 840 66

An inception cohort of 87 patients with lupus nephritis was evaluated using a classification tree regression technique. Four relevant outcomes were studied: (1) renal insufficiency (serum creatinine > 5.0 mg/dl); (2) renal failure; (3) death due to renal involvement; and (4) any death due to systemic lupus erythematosus. All 4 outcomes could be predicted by one or more renal severity measures (serum creatinine, 24-h urinary protein excretion, nephrotic syndrome, or duration of prior renal disease), and among those with nonsevere renal disease, with a single disease activity measure (the National Institutes of Health or le Riche index). In general 3 prognostic groups (high, intermediate and low risk) could be identified for each outcome. Our results demonstrate the value of regression tree techniques in studies of prognosis and are compatible with a hypothesis of the interaction of disease activity with organ damage in lupus nephritis.
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PMID:The longterm prognosis of lupus nephritis: the impact of disease activity. 844 Nov 67

The mechanism, or mechanisms, responsible for enhancement of renal disease after episodes of infection are poorly understood. We used the BXSB mouse as a lupus model of autoimmune disease and we used bacterial lipopolysaccharide (LPS) as a surrogate infectious agent to gain some insight into the mechanism by which infections promote enhancement of autoimmune disease to chronicity. BXSB mice were exposed to LPS for 5 weeks, LPS was withdrawn and various tests and measurements were performed 6 weeks thereafter. Matched BXSB mice exposed to vehicle injections for 5 weeks served as controls. We verified that previous exposure to LPS enhances polyclonal B cell activation, impairs carrier function of blood cells for immune complexes, increases deposition of immune complexes in the microcirculation and promotes glomerular inflammation and sclerosis. These changes occurred at 6 weeks after withdrawal of LPS in the presence of unimpaired function of mononuclear phagocytes. Some of the effects of LPS are reversible, others are partially so and others are irreversible. Altered immune functions elicited by prior exposure to LPS can result in enhanced involvement of various renal compartments and can result in renal insufficiency.
Lupus 1995 Oct
PMID:Enhancement of renal disease in BXSB lupus prone mice after prior exposure to bacterial lipopolysaccharide. 856 25

A case of rapidly progressive nephritis is reported in a female patient having systemic lupus erythematosus (SLE) with antiphospholipid syndrome. Clinical presentation of progressive lupus nephritis with intensifying renal insufficiency, arterial hypertension, hematuria, nephrotic syndrome was associated with unusual morphological manifestations of mesangiocapillary glomerulonephritis with advanced vasculitis. The authors attribute a malignant nephritis course atypical for patients with antiphospholipid syndrome to development of renal vasculitis. The discussion covers lupus genesis of vascular involvement, a probable triggering role of antibodies to phospholipids in impairment of endothelial cells.
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PMID:[The characteristics of kidney involvement in a female patient with systemic lupus erythematosus and the antiphospholipid syndrome]. 857 27

The clinical and immunological manifestations of 51 children with onset of systemic lupus erythematosus (SLE) before the age of 15 were compared with those of 308 adult patients with disease onset between the age of 15-49 and another 27 elderly lupus patients whose disease onset occurred at or after the age of 50. Overall disease activity determined by mean SLEDAI score was highest in the childhood group followed by the adult and the elderly group respectively. More severe form of cutaneous involvement, adenopathy, hypertension, renal involvement with renal insufficiency and anti-nDNA antibodies occurred predominantly in the childhood lupus. The clinical features distinguishing old-age lupus were chronic disease with a long interval between the time of onset and diagnosis, higher incidence of discoid rash and lower incidence of malar rash and renal involvement. Frequencies of anti-nDNA antibodies and renal involvement gradually decreased from childhood, to adulthood and to elderly lupus respectively. Anti-Sm antibodies were predominant in the adult onset group. Genetic markers, sex hormones and senility of the immune system may play a role in these age-related differences in clinical and immunological manifestations in SLE.
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PMID:Age-related differences on clinical and immunological manifestations of SLE. 870 43

A patient with documented SLE is described who presented with dysuria, gastrointestinal (GI) symptoms and renal insufficiency associated with the unusual occurrence of bilateral hydro-ureteronephrosis due to ureterovesical junction stricture (obstructive uropathy) on radiographic findings. Pathologic investigations disclosed chronic interstitial cystitis (IC) with evidence of focal immune complex deposition in blood vessel walls of the bladder. The GI symptoms and dysuria regressed with initial therapy of SLE by steroid but persistent obstructive uropathy (OU) and renal insufficiency required bilateral nephrostomy. Then, following continuous medical treatment with steroid plus intravenous pulse injection of cyclophosphamide, obstructive uropathy was relieved even with removing nephrostomy tube and renal function remained stable.
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PMID:Obstructive uropathy due to interstitial cystitis in a patient with systemic lupus erythematosus. 870 64

For many years, valvulitis in systemic lupus erythematosus has been known to occur. Our patient was a 17-year-old girl who presented with severe mitral incompetence and renal insufficiency due to lupus valvulitis. The patient was first treated by mitral valve repair, but follow-up disclosed precocious calcification of the valve, necessitating mitral valve replacement with a cryopreserved homograft. At follow-up after 1 year, echocardiography has shown the valve to be functioning normally. A reconstructive mitral valve operation would seem to be preferable. However, a conservative operation does not alter the natural history of the disease and the progression of valve thickening and calcification.
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PMID:Systemic lupus erythematosus valvulitis: mitral valve replacement with a homograft. 878 90

Hemolytic anemia observed in patients with SLE include autoimmune hemolytic anemia (AIHA) and thrombotic microangiopathic hemolytic anemia (TMHA). AIHA is caused by anti-erythrocyte antibody, which can be detected by direct Coombs' test. Recently, anti-phospholipid antibody draw attention as a cause of AIHA in SLE. TMHA include thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). Patients with TTP show CNS symptoms and renal damage, while those with HUS develop renal insufficiency, but show minimal CNS symptoms. Clinical findings of TMHA are fever, hemolytic anemia, thrombocytopenia, CNS symptoms, and renal insufficiency, which are quite same as the findings of SLE itself. TMHA should be considered for differential diagnosis in acute exacerbation of SLE.
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PMID:[Hemolytic anemias observed in SLE]. 889 May 89

To evaluate the clinical effectiveness of intermittent intravenous cyclophosphamide in the treatment of severe systemic lupus erythematosus, 20 patients with systemic lupus erythematosus (SLE) and evidence of severe renal involvement or systemic vasculitis, consecutively admitted to the hospital were studied. Cyclophosphamide was administered intravenously at a dosage of 1.0 g/m2 monthly, during 6 months and maintained every 3 months during 12 additional months. Of 10 patients with active lupus nephritis, a reduction or disappearance of proteinuria and maintenance of normal renal function was recorded in 6. Improvement of renal function was observed in 4 out of 7 patients with renal insufficiency at initial evaluation; resolution of renal insufficiency was more frequently observed in patients with recent onset renal failure. At the end of the follow-up (18.0 +/- 14.5 months) disappearance or reduction of nephrotic range proteinuria was recorded in 6 out of 14 patients; there was progression toward renal failure in 4 patients (20%). Response to intravenous cyclophosphamide therapy was observed in 4 of 5 patients with severe extrarenal SLE. Side effects, recorded in 12 patients, were mild and transient and in no patient was the treatment discontinued. Four patients died during the follow-up, although in 2 of them the deaths were not attributable to therapy. Even though this was an open and uncontrolled study, intermittent, intravenous cyclophosphamide was an effective therapy for severe, steroid refractory SLE.
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PMID:Clinical effects of intermittent, intravenous cyclophosphamide in severe systemic lupus erythematosus. 889 47


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