Systemic lupus erythematosus and chronic intestinal pseudoobstruction

We describe the first case of a patient with systemic lupus erythematosus in which clinical and radiological features, small bowel manometry and histological studies were consistent with the diagnosis of chronic intestinal pseudoobstruction. Our patient developed a fatal form of lupus with cerebral angiitis and tubulointerstitial lupus nephritis, with no sign of progressive systemic sclerosis or dermatopolymyositis. Postmortem examination confirmed the presence of an extensive fibrotic process in the muscularis layers, whereas the muscularis mucosae and the innervation of the digestive wall were spared.