Collagenous Sprue — An Unrecognized Type of Malabsorption

Collagenous sprue is a distinctive lesion of the intestinal mucosa associated with progressive malabsorption. The intestinal pathology, as observed in a 51-year-old woman, was initially identical with the characteristic "flat" lesion of untreated celiac sprue. Thereafter, bands of eosinophilic hyaline material within the lamina propria became increasingly apparent. On electron microscopy this eosinophilic material was identified as collagen. As the disease progressed, the mucosa became progressively thinner. Nevertheless a few short segments of normal mucosa remained even at the time of death. Massive steatorrhea, water and electrolyte loss were the main problems. Corticosteroids reduced malabsorption somewhat but had deleterious side effects. All other therapy, including a gluten-free diet, failed. Extensive studies during life and careful post-mortem examination did not establish a known cause for collagenous sprue. Some cases currently designated "refractory" or "unclassified" sprue may prove to be collagenous sprue.