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)

We present the case of a young woman with a protein-losing enteropathy occurring in the context of systemic lupus erythematosus. This rare complication has limited gastro-intestinal manifestations and must be systematically looked for when hypoalbuminemia occurs in the absence of a lupus nephritis. High dose corticosteroids therapy (> or = 1 mg/kg/day of prednisone) usually leads to recovery, and should be the first treatment attempted. If this treatment is ineffective, bolus injections of methylprednisolone (1 g/day for three days) may be recommended. Immunosuppressive therapy should be given only if the above treatments are ineffective, or in case of cortico-dependency.
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PMID:[Exudative enteropathy in disseminated lupus erythematosus]. 128 33

We report a case of systemic lupus erythematosus (SLE) in a 15-year-old girl with severe neurological disease, platelet function disorder and pulmonary haemorrhage, which remitted after plasmapheresis. The patient developed protein-losing enteropathy shrinking lung, and acute pancreatitis with pseudocyst formation. These infrequent complication of SLE are discussed.
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PMID:Systemic lupus erythematosus: a case report with unusual manifestations and favourable outcome after plasmapheresis. 150 76

A patient with connective tissue disease presenting with both protein-losing enteropathy and pancreatic involvement is reported. A 52-year-old female was admitted because of mild epigastralgia, anasarca and ascites. Serum albumin, transferrin and zinc, showed low levels. An Upper G.I. series and endoscopy showed thickened folds of the duodenum and the jejunum. Biopsy specimens revealed lymphangiectasia in edematous villi. 99mTc-labeled human serum albumin scintigram showed abnormal radioactivity in the small intestine 90 minutes after intravenous injection, indicating protein-losing enteropathy. Hypoalbuminemia was ameliorated by glucocorticoid therapy, but recurred twice when glucocorticoid treatment was tapered. Hypoalbuminemia has not occurred since intestinal lymphangiectasia was improved with glucocorticoid treatment. Levels of elastase 1 and lipase were high in serum and ascites on admission. Endoscopic retrograde pancreatogram showed no abnormalities. Serum pancreatic enzymes were also ameliorated by glucocorticoid therapy, but slightly high levels continued for about one year and a half. This case might have been diagnosed as systemic lupus erythematosus although mixed connective tissue disease was also suspected. There are few reports of protein-losing enteropathy and pancreatic involvement associated with connective tissue diseases. Protein-losing enteropathy and pancreatic involvement were ameliorated with glucocorticoid treatment, suggesting participation of immunological mechanisms.
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PMID:Protein-losing enteropathy and pancreatic involvement in a case of connective tissue disease. 157 30

We report the case of a female patient with seronegative lupus and predominant bladder and intestinal involvement in the form of interstitial cystitis and protein-losing enteropathy. This association is exceptional in the literature but may be underestimated because of frequent latency of interstitial cystitis. It may define a peculiar subgroup of lupus patients usually responsive to steroid therapy. In this case, only cyclophosphamide markedly improved the protein-losing enteropathy but did not influence the bladder disease.
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PMID:Association of chronic interstitial cystitis, protein-losing enteropathy and paralytic ileus with seronegative systemic lupus erythematosus: case report and review of the literature. 851 8

A 46-year-old woman previously diagnosed as having systemic lupus erythematosus presented with severe hypoalbuminemia and anasarca. She was demonstrated to have protein-losing enteropathy without any other active symptoms of SLE. Her bowel habit was normal and endoscopic examination revealed non-specific colitis and a small ulcer in the duodenum. Serum biochemistry showed an abnormal profile of the serum protein, including severe hyperlipoproteinemia and hyperfibrinogenemia. The process of protein-losing was not selective in terms of the molecular size. All of these symptoms and the abnormalities in laboratory data were improved by corticosteroid therapy.
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PMID:A patient with protein-losing enteropathy associated with systemic lupus erythematosus. 163 62

Although nephritis remains a very important clinical disease manifestation of systemic lupus erythematosus (SLE), the focus has shifted from issues of mortality to morbidity. An improved method for measuring renal function is presented, and the value of renal biopsy, specifically the chronicity index, is confirmed as a predictor of end-stage renal disease. Several studies detailing the generally favorable course in SLE patients with renal failure who have eventually required chronic dialysis or renal transplantation are discussed. A high frequency of cardiovascular manifestations, often subclinical, is documented, including abnormalities of ventricular and valvular function. The clinical course and response to treatment of SLE patients with diffuse interstitial lung disease and pulmonary hypertension are outlined. Cases of the unusual gastrointestinal presentation of protein-losing enteropathy in SLE have been described by several authors. The clinical correlates and significance of persistent rheumatoid-like arthritis and radiologic erosive and cystic bony lesions in SLE are discussed. Serologic markers of specific SLE disease manifestations and general predictors of disease activity are evaluated. Complications related to infection, malignancy, and pregnancy are described.
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PMID:Clinical manifestations of disease activity, its measurement, and associated morbidity in systemic lupus erythematosus. 175 10

We describe a 23-year old Brazilian woman with systemic lupus erythematosus and protein-losing enteropathy. Small intestinal biopsy revealed lymphangiectasia. Protein-losing enteropathy responded to corticosteroid therapy and should be suspected in cases of systemic lupus erythematosus with hypoalbuminemia without proteinuria and liver failure. A review of the English literature is presented with comments on pathogenesis.
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PMID:Protein-losing enteropathy in systemic lupus erythematosus. 184 3

Fourteen cases of primary lupus-associated protein-losing enteropathy have now been reported in the English-language literature. These cases were reviewed to find any consistent pattern of presentation. Lupus-associated protein-losing enteropathy typically occurs in young women, and is characterized by the onset of profound edema and hypoalbuminemia. In many cases it is the first obvious manifestations of systemic lupus erythematosus. Diarrhea is present about 50% of the time, but steatorrhea is absent. Diagnosis of protein-losing enteropathy can be successfully made by radioisotopic studies or 24-hour stool alpha 1-antitrypsin clearance. A normal lymphocyte count, elevated serum cholesterol, and absence of lymphangiectasia on intestinal biopsy help distinguish lupus-associated protein-losing enteropathy from protein-losing enteropathies due to direct or indirect lymphatic obstruction. Normal endoscopy and mucosal biopsy can rule out protein loss due to mucosal disruption. Prognosis appears to be excellent with corticosteroids, although other immunosuppressive therapies have been successfully used. A typical and illustrative case is presented as a focal point for review and discussion.
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PMID:Lupus-associated protein-losing enteropathy. 206

This case report concerns a young woman with systemic lupus erythematosus who presented with a protein-losing enteropathy. Intestinal biopsy revealed lymphangiectasia. Mesenteric lymph nodes showed paracortical lymphoid depletion and multiple small sinusoids. Elevated cholesterol levels and normal lymphocyte counts characterized the peripheral blood samples. Lymph nodes, though enlarged, showed no evidence of obstructive pathology. No secondary cause of lymphangiectasia could be identified. The patient exhibited antinuclear antibodies and antibodies to dsDNA at the onset, and then, 5 yr later, the classic features of systemic lupus erythematosus (SLE). The lymphocytopenia and hypolipidemia that characterizes lymphangiectasia is not a feature of the SLE cases reported to date. Furthermore, the mechanism for the dilated lymphatics and villous edema is more likely immunological than mechanical disruption of lymphatics.
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PMID:Protein-losing enteropathy in systemic lupus erythematosus associated with intestinal lymphangiectasia. 222 Jul 36

A 33-year-old woman presented with chronic diarrhea, vomiting and anasarca due to systemic lupus erythematosus with protein-losing enteropathy, interstitial cystitis and glomerulonephritis. Methylprednisolone could not prevent aggravation of diarrhea, obstructive uropathy, and nephrotic syndrome, and prolonged intestinal ileus developed. Because of this steroid-resistance, bolus injections of cyclophosphamide (1 g i.v. monthly) were decided. Protein-losing enteropathy and ileus both disappeared rapidly following the first injection. Protein-losing enteropathy, intestinal ileus and interstitial cystitis are exceptional manifestations of systemic lupus erythematosus; steroid-resistance of the digestive manifestations has only been reported in one case and our observation is the first reporting the efficacy of cyclophosphamide.
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PMID:[Exudative enteropathy and interstitial cystitis due to systemic lupus erythematosus]. 226 25


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