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Objective: To emphasize the possibility of obtaining good quality and lasting remission of a severe idiopathic protein-loosing enteropathy with corticosteroid therapy., Observation: A 30 year-old woman was hospitalized for edema of the lower limbs related to hypoalbuminemia due to a protein-loosing enteropathy, demonstrated by measurement of alpha-1 antitrypsin clearance. Diagnosis of lupus was evoked but not confirmed. Nutritional treatment failed. Corticosteroids administered in a bolus, and subsequently per os, rapidly led to complete, lasting (22 months later) remission., Discussion: Corticosteroid therapy is the classical treatment of a protein-loosing enteropathy concomitant to disseminated lupus erythematosus, a rare combination. In the absence of lupus, a few cases of remission from a protein-loosing enteropathy have been reported. However, the delay before remission was longer than in our case report, and the remission-free period generally shorter., Conclusion: Corticosteroid therapy is an effective treatment of an idiopathic protein-loosing enteropathy.

The authors report the case of a 61-year old patient with a Churg-Strauss syndrome revealed by abdominal pain. Investigations showed a diffuse inflammatory digestive tract involvement, documented by endoscopy associated with protein-losing enteropathy. Deep rectal biopsy revealed vasculitis in an otherwise macroscopically normal rectum. Faced with acute and life-threatening course of disease, emergency medical treatment with steroids and cyclophosphamide led to rapid regression of clinical, biological and radiological abnormalities.

A case of Langerhans cell histiocytosis is reported in a neonate. Intestinal involvement was especially diffuse and severe, presenting as a protein-losing enteropathy secondary to massive mucosal infiltration by histiocytic cells. The infant died at the age of 3 1/2 months despite therapy with corticosteroids and vinblastine then etoposide and interferon. Such an outcome confirmed the severity of forms with neonatal onset and digestive involvement.

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.