Systemic sclerosis. Case presenting with tetracycline-responsive malabsorption syndrome

Described herein is a patient with systemic sclerosis who presented with a malabsorption syndrome and who showed marked improvement following each of two courses of tetracycline therapy. Death was caused by acute infarction of the small bowel resulting from volvulus, presumably related to the severe intestinal dilatation and impaired peristalsis. Pathologic findings included a widespread arteriolitis, particularly involving the small intestine. The involved vessels showed no evidence of gamma globulin or complement deposit, suggesting that the process was not primarily an immune one. In addition to typical esophageal fibrosis there was diffuse interstitial pulmonary fibrosis. There was, however, only minimal fibrosis and muscle atrophy of the small bowel wall.