A young woman presented with high
fever and
edema in January, 1984, and was diagnosed as having
systemic lupus erythematosus.
Prednisolone administration failed to improve her symptoms. In May she was admitted to hospital because of elevated erythrocyte sedimentation rate (ESR),
hypoproteinemia,
hypogammaglobulinemia, hypocomplementemia, positive
antinuclear antibody, elevated
immune complex level, and
diarrhea.
Edema disappeared following administration of
diuretics and
albumin, although the pathogenesis was still undetermined. In September, she was referred to our institution because of severe watery
diarrhea and
hypoproteinemia. Endoscopic examination showed a diffuse inflammatory lesion in the duodenum and the colon. Radioisotopic 51Cr-albumin study results were compatible with
protein-losing enteropathy.
Hypoproteinemia and inflammatory changes of the intestine were improved by
antibiotics, suggesting that the inflammatory lesion was caused by
bacterial infection. Despite the improvements in clinical symptoms and laboratory findings, the serum
IgA level was still low and the
thrombocytopenia remained. The morphological characteristics of the megakaryocytes were consistent with
idiopathic thrombocytopenic purpura. In May, 1986, the
thrombocytopenia deteriorated, causing
purpura.
Prednisolone was administered again, and this resulted in normalization of the platelet count, although the
IgA level remained low. Finally the
prednisolone was stopped, and the
IgA level gradually recovered, with the improvement of the
enterocolitis. The exact pathogenesis of the whole picture in this case is unclear, but an 8-year-long
clinical course suggests that the
protein-losing was caused by an infectious
enterocolitis superimposed on
IgA deficiency.