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1/31. Protein-losing enteropathy associated with hypocomplementemia and anti-nuclear antibodies.

    We report a case of protein-losing enteropathy associated with an autoimmune disorder, presumably systemic lupus erythematosus. Although typical manifestations of systemic lupus erythematosus were lacking, a high serum cholesterol level, a low serum complement level, positivity for anti-nuclear antibody, and positivity for anti-single-strand dna antibody suggested an autoimmune mechanism as the cause of the condition. Although immunohistological examination of duodenal and ileal biopsy specimens failed to reveal deposits of immune complex or complement in the vessels, capillary hyperpermeability was suspected as the mechanism of the condition.
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ranking = 1
keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus
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2/31. Protein-losing enteropathy exacerbated with the appearance of symptoms of systemic lupus erythematosus.

    A 38-year-old woman visited our hospital with edema on her face and conjunctivae. The underlying disease was not clarified, and she did not visit the hospital afterwards. She suffered from diarrhea, polyarthralgia, Raynaud's phenomenon, malar rash and hair loss in the subsequent two years, and was hospitalized because of hypoproteinemia. Her urine, liver and heart test results did not account for her hypoproteinemia. She was diagnosed as having protein-losing enteropathy (PLE) associated with SLE based on the 99mtechnetium-labeled human serum albumin scintigraphy findings, clinical findings and laboratory results of antinuclear and anti-Sm antibodies. This case report demonstrates a strong association between PLE and SLE because PLE was aggravated along with the appearance of SLE symptoms and PLE subsided with prednisolone treatment along with improvement of SLE.
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ranking = 2
keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus
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3/31. Primary protein-losing enteropathy in anti-double-stranded dna disease: the initial and sole clinical manifestation of occult systemic lupus erythematosus?

    hypoalbuminemia and generalized edema is a common clinical problem and the etiology is usually clear: cirrhosis, nephrotic syndrome, primary gastrointestinal disorders, malnutrition, etc. We present a 23-year-old previously healthy woman of Korean background who presented with generalized edema and a serum albumin of 9 g/L (normal, 35-45 g/L). Intensive investigations failed to reveal liver, renal, or inflammatory gastrointestinal mucosal disease. The antinuclear antibody was positive at a titer of 1:80, and extractable nuclear antigens were positive for SSA/anti-Ro. Anti-double-stranded dna was markedly elevated at 4.6 kU/L (normal, 0-2.0 kU/L). A technetium 99M-labeled albumin study revealed a protein-losing enteropathy, despite normal histologic full-thickness jejunal biopsies. A diagnosis of occult systemic lupus erythematosus resulting in increased intestinal vascular permeability was made. The hypoalbuminemia remained in long-term remission after the initiation of induction and maintenance immunosuppression.
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ranking = 2.5
keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus
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4/31. Protein-losing enteropathy in a young African-American woman with abdominal pain, diarrhea and hydronephrosis.

    The case of a 21-year-old African-American woman who presented with abdominal pain, diarrhea and hydronephrosis and who proved to have protein-losing enteropathy secondary to systemic lupus erythematosus is discussed. This is an unusual complication of lupus.
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ranking = 0.51601310531961
keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus
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5/31. Protein-losing gastropathy associated with autoimmune disease: successful treatment with prednisolone.

    We report a patient with protein-losing gastropathy probably associated with autoimmune disease, in whom prednisolone treatment was highly effective. A 45-year-old woman was admitted to our hospital with edema of the face and lower extremities. blood examination revealed hypoproteinemia, hypoalbuminemia, and hypercholesteremia. Renal biopsy revealed no definite findings of lupus nephritis, including vasculitis. A diagnosis of protein-losing gastropathy was made on the basis of increased alpha1-antitrypsin clearance and 99mTc-labeled human serum albumin scintigram showing abnormal radioactivity in the stomach. Endoscopic gastric biopsies revealed nonspecific inflammation, but marked intramural edema. Based on a slight elevation of antinuclear antibody level, autoimmune disease was suspected to be involved in this patient. Administration of prednisolone, as a diagnostic therapy, alleviated the hypoproteinemia, hypoalbuminemia, and hypercholesteremia. These findings suggest that an autoimmune mechanism could have been involved in this case of protein-losing gastropathy.
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ranking = 0.01601310531961
keywords = lupus
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6/31. Severe hypoalbuminaemia in a systemic lupus erythematosus-like patient.

    Diffuse oedema due to hypoalbuminaemia is a common manifestation in patients affected with systemic lupus erythematosus. Hypoalbuminaemia is usually secondary to an ongoing glomerulonephritis leading to proteinuria or, more rarely, to decreased protein synthesis or deficient protein intake. Here, we report the case of a 6-year-old girl with a previous history of Evans' syndrome and mesangial glomerulonephritis who subsequently developed severe anasarca without apparent proteinuria. Faecal oal-antitrypsin showed a persistent severe intestinal protein loss (ranging from 1500 to 6.500 yl/g humid faeces, normal value < 200), consistent with the diagnosis of protein-losing enteropathy. An echographic examination of the abdomen revealed a diffuse thickening of the intestinal wall, particularly at the level of the small bowel, with an almost exclusive involvement of the submucosal layer. colonoscopy revealed the presence of diffuse purpuric lesions at the level of the submucosa. Aggressive immunosuppressive treatment completely resolved the clinical picture. CONCLUSION: protein-losing enteropathy is an uncommon cause of hypoalbuminaemia during the course of systemic lupus erythrematosus. It may be considered as a clinical syndrome related to many pathological conditions leading to an excessive intestinal protein loss. Some conditions are related with an altered mucosal permeability, others with primary or secondary intestinal lymphangiectasia. A review of the possible causes of systemic lupus erythrematosus-associated protein-losing enteropathy reported in the paediatric literature is given.
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ranking = 2.5757527895407
keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus
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7/31. Protein-losing enteropathy in systemic lupus erythematosus: report of a severe, persistent case and review of pathophysiology.

    Protein-losing enteropathy (PLE) is a rare manifestation of systemic lupus erythematosus (SLE). We report a severe and resistant case of PLE, discuss pathophysiology and possible role of cytokines in the disease process. We also present a review of the current literature.
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ranking = 2.5
keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus
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8/31. edema due to protein-losing enteropathy--a disorder rarely considered by nephrologists.

    In the differential diagnosis, protein-losing enteropathy (PLE) is a rarely considered explanation of edema. Three such cases are reported in this article. Clinical presentations varied from severe generalized anasarca and respiratory distress to mild pitting edema on the pretibial surface. hypoalbuminemia (< 35 g/l) was another common finding in addition to edema. The patients were carefully examined to exclude other causes of hypoalbuminemia. Two patients experienced diarrhea. Endoscopic studies (plus biopsies) for any mucosal lesion in the stomach and colon were also performed. PLE was confirmed by the positive radionuclide scanning results after infusing intravenous 99mTc human serum albumin (USA). Investigation for the etiologies showed intestinal lymphangiectasia in 1 patient, Menetrier's disease in another, and no recognizable cause in the third. The severe anasarca of the patient with intestinal lymphangiectasia didn't respond to corticosteroids and albumin supplement plus large doses of furosemide. She died of overwhelming pulmonary infection despite the use of powerful antibiotics (ceftriaxone and amikacin). We planned to treat the Menetrier's disease patient with somatostatin to decrease the exocrine activities of the intestinal tract. The patient with presumable idiopathic PLE had the least severe edema and was not treated with any medication. In addition to the above patients, another 3 patients with hypoalbuminemia and edema were also noted to have positive HSA scan results. However, 2 of these patients had systemic lupus erythromatosus and the third pulmonary tuberculosis and biopsy-proven membranous nephropathy. Treatment of their underlying diseases showed satisfactory remission of edema.
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ranking = 0.037876394770335
keywords = systemic lupus, lupus
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9/31. Concurrent occurrence of chylothorax, chylous ascites, and protein-losing enteropathy in systemic lupus erythematosus.

    We describe 2 patients with systemic lupus erythematosus (SLE) who presented with chylothorax, chylous ascites, and protein-losing enteropathy. Analysis of pleural or peritoneal fluid revealed a high level of triglyceride and elevated 24 h stool alpha1-antitrypsin clearance in keeping with protein-losing enteropathy. One patient failed to respond to high dose corticosteroid therapy but recovered after 3 cycles of monthly cyclophosphamide treatment. The other patient initially responded to high dose corticosteroid therapy, but succumbed to infectious complications. This is the first report of occurrence of chylothorax and chylous ascites associated with SLE.
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ranking = 2.5
keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus
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10/31. Systemic lupus erythematosus: a case report with unusual manifestations and favourable outcome after plasmapheresis.

    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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ranking = 2.0758874722548
keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus, lupus
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