A 57–year–old man with a known history of ischemic cardiomyopathy, high cholesterol, hypertension and diabetes presented with 2–month history of seven daily episodes of watery stools. He lost eleven kg over this period. He had no recent travels, no sick contacts and no new medications. Exam revealed stable vitals, no tachycardia, soft non–tender abdomen with normal bowel sounds. He was taking loperamide, dicyclomine, ranitidine, pantoprazole and his lisinopril, metoprolol. Laboratory workup revealed normal stool analysis and TSH. He also tested negative for C.Diff, celiac disease antibodies, HIV, ANA, Hepatitis A, B and C. Initial EGD and colonoscopy were essentially normal. Sigmoidoscopy done to screen for inflammatory process revealed thick collagenous sub–epithelial layer on duodenal biopsy. He failed gluten–free diet and lost another fifteen kg, progressed to needing total parenteral nutrition while he was on steroids. He was started on mercaptopurine after steroid–failure and he has been stable on it since over a year and a half.
Collagenous sprue is a very rare malabsorption disease first recognized in 1970. This illness is defined by severe malabsorption, sub–epithelial collagenous deposits, and failure to respond to a gluten–free diet. Diagnosis of this rare condition is challenging and often cases get labeled as celiac disease initially. Failure to gluten free diet often prompts a biopsy, which reveals collagenous deposits, hence pointing towards to right diagnosis as in our patient. Management is difficult as there are no data available comparing therapies. Steroid therapy still remains the most effective therapy though long–term complications from it are always a concern. Other options are immunosuppressive therapy and total parenteral nutrition. Prognosis still remains guarded with most patients due to progressive malnutrition often requiring steroid/immunosuppressive/total parenteral nutrition for survival.
Collagenous sprue is a rare malabsorption disease with very poor prognosis.