Systemic lupus erythematosus and intestinal venulitis

Weiser, Milton M.; Andres, Giuseppe A.; Brentjens, Jan R.; Evans, James T.; Reichlin, Morris

doi:10.1016/0016-5085(81)90612-0

Cited by 90 publications

(34 citation statements)

References 20 publications

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“…So far, almost 25 cases have been described [8] and only four in the paediatric age [1,2,3,5]. The presence of only three diagnostic criteria of the American Rheumatology Association did not allow a definitive diagnosis of SLE in our patient.…”

Section: Discussioncontrasting

confidence: 59%

“…This variability may be related to the wide range of possible causes of PLE. In the present case, the indolence of the clinical picture together with the purpuric-like appearance of the colonic lesions in the absence of frank ischaemic lesions, raised the possibility of an intestinal venulitis involving the submucosa, as already reported in adult SLE patients [8]. After discussion with the parents, explorative laparoscopy for possible transmural biopsy was not performed because of the possible risks related to the procedure.…”

Section: Discussionmentioning

confidence: 81%

“…In adult SLE, PLE is classically reported to promptly respond to low-doses of oral steroids [7,8]. However, a small subset of patients is reported to require aggressive immunosuppressive treatment in order to achieve complete control of the gastrointestinal symptoms, such as in the present and in another paediatric case [2].…”

Section: Discussionmentioning

confidence: 97%

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Severe hypoalbuminaemia in a systemic lupus erythematosus-like patient

Gattorno¹,

Buoncompagni²,

Barabino³

et al. 2001

Eur J Pediatr

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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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Section: Discussioncontrasting

confidence: 59%

Section: Discussionmentioning

confidence: 81%

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Severe hypoalbuminaemia in a systemic lupus erythematosus-like patient

Gattorno¹,

Buoncompagni²,

Barabino³

et al. 2001

Eur J Pediatr

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“…Treatment consists of two measures a. Nutritional replacement using high-protein diet and medium-chain triglycerides [5,39]. b.…”

Section: Therapy and Prognosismentioning

confidence: 99%

Lupus protein-losing enteropathy (LUPLE): A systematic review

Al-Mogairen

2011

Rheumatol Int

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Lupus protein-losing enteropathy (LUPLE) is a well reported but a rare manifestation of systemic lupus erythematosus (SLE). The main objectives of this study are to raise awareness of LUPLE that can be easily missed by internist, rheumatologist, gastroenterologist and nephrologist, and then to be considered in any patient with unexplained edema, ascites, and hypoalbuminemia. A systematic review was performed with 112 patients who met the eligibility criteria and were critically appraised. The LUPLE was ultimately diagnosed by either Tc-(99m) albumin scintography ((99m)Tc-HAS) or fecal alpha-1-antitrypsin clearance test. Clinical features of patients, at the time of LUPLE diagnosis, were as follows: age was 34 ± 14.2 years; the female to male ratio was 5.8:1; the mean time to development of LUPLE after diagnosis of SLE was 4.19 ± 4.7 years. There was a predominance of Asian (64.7%) while 29.5% were white or Hispanic patients. Eighty percent had peripheral edema, 48% had ascites, 38% had pleural effusion, and 21% had pericardial effusion. Forty-six percent had diarrhea, 27% had abdominal pain, 22% had nausea, and 19% had vomiting. Hypoalbuminemia was the most common characteristic laboratory finding (96%). A 24-h urine protein was less than 0.5 gm in (71%). Almost all patients (96%) had positive ANA with predominant speckled patterns (55%) and hypocomplementemia (79%). Colonoscopy showed mucosal thickening in 44% of patients, and the majority of patients (52%) revealed no abnormalities; on the other hand, intestinal histology either revealed mucosal edema, inflammatory cell infiltrate, lymphangiectasia, mucosal atrophy or vasculitis in 80% of patients. All patients were started on steroids. Thirty-four percent responded to steroids alone. Sixty-six percent were started with other immunosuppressive therapies, which include cyclophosphamide (46%), azathioprine (33%), and a combination of cyclophosphamide and azathioprine (7%). A few reported cases responded to either cyclosporine or etanercept. Prognosis was very good with steroids combined with immunosuppressive therapy. This is the first systematic review of LUPLE and should be considered as an etiology of unidentified edema, ascites, and hypoalbuminemia.

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“…In the absence of lymphangiectasia or mucosal pathologic findings, investigators have focused on vascular causes of protein loss, ranging from frank immunocomplex vasculitis to widespread capillary leakiness mediated by a vascular permeability-increasing lymphokine. 3,6,11,16 Most of the cholesterol in the sera exists as very large components, such as very low-density lipoprotein, low-density lipopro-tein, and high-density lipoproteincholesterol. The smallest compound, high-density lipoprotein-cholesterol, has a molecular weight of around 500 000, which is too large to be lost in capillary transudate.…”

Section: Discussionmentioning

confidence: 99%

Protein-losing gastropathy associated with autoimmune disease: successful treatment with prednisolone

et al. 2002

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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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Systemic lupus erythematosus and intestinal venulitis

Cited by 90 publications

References 20 publications

Severe hypoalbuminaemia in a systemic lupus erythematosus-like patient

Severe hypoalbuminaemia in a systemic lupus erythematosus-like patient

Lupus protein-losing enteropathy (LUPLE): A systematic review

Protein-losing gastropathy associated with autoimmune disease: successful treatment with prednisolone

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