A CASE OF PROTEIN-LOSING ENTEROPATHY IN IDIOPATHIC THROMBOCYTOPENIC PURPURA WITH DECREASED IGA

A young woman presented with high fever and edema in January, 1984, an d was diagnosed as having systemic lupus erythematosus. Prednisolone a dministration failed to improve her symptoms. In May she was admitted to hospital because of elevated erythrocyte sedimentation rate (ESR), hypoproteinemia, hypogammaglobulinemia, hypocomplementemia, positive a ntinuclear antibody, elevated immune complex level, and diarrhea. Edem a disappeared following administration of diuretics and albumin, altho ugh the pathogenesis was still undetermined. In September, she was ref erred to our institution because of severe watery diarrhea and hypopro teinemia. Endoscopic examination showed a diffuse inflammatory lesion in the duodenum and the colon. RadioiSotopiC Cr-51-albumin study resul ts were compatible with protein-losing enteropathy. Hypoproteinemia an d inflammatory changes of the intestine were improved by antibiotics, suggesting that the inflammatory lesion was caused by bacterial infect ion. Despite the improvements in clinical symptoms and laboratory find ings, the serum IgA level was still low and the thrombocytopenia remai ned. The morphological characteristics of the megakaryocytes were cons istent with idiopathic thrombocytopenic purpura. In May, 1986, the thr ombocytopenia deteriorated, causing purpura. Prednisolone was administ ered again, and this resulted in normalization of the platelet count, although the IgA level remained low. Finally the prednisolone was stop ped, and the IgA level gradually recovered, with the improvement of th e enterocolitis. The exact pathogenesis of the whole picture in this c ase is unclear, but an 8-year-long clinical course suggests that the p rotein-losing was caused by an infectious enterocolitis superimposed o n IgA deficiency.