Cronkhite-Canada Syndrome: A Rare Cause of Chronic Diarrhea.

Cronkhite-Canada syndrome (CCS) is a rare non-hereditary disease characterized by chronic diarrhea, diffuse intestinal polyposis and onychodystrophy. We present here a case of a middle-aged female who presented with chronic intermittent bloody diarrhea associated alopecia and loss of finger and toe nails. Labs were remarkable for microcytic anemia and severe hypoalbuminemia. Endoscopy showed numerous polyps scattered throughout the colon. She was treated with nutritional support and corticosteroid with complete resolution of her symptoms and endoscopic findings. CCS is associated with high mortality and gastrointestinal malignancies. Clinicians should consider CCS in a patient with unexplained chronic diarrhea and ectodermal abnormalities.

Refractory Celiac Disease Successfully Treated With Azathioprine.

Refractory celiac disease (CD) is a clinical diagnosis defined by the persistence of signs/symptoms, laboratory abnormalities or villous atrophy typical of CD despite strict adherence to a gluten-free diet for at least 6 - 12 months. It should be suspected when patients with CD fail to respond primarily or secondarily to a gluten-free diet, especially if there is significant weight loss. Differentiation between types is important both for management and predicting prognosis. Type I can be managed with mild immunosuppression with nutritional support. Type II requires strong immunosuppression like azathioprine. Recently, autologous stem cell transplantation has also been used to treat type II.

Association of Autoimmune Hepatitis and Celiac Disease: Role of Gluten-Free Diet in Reversing Liver Dysfunction.

Autoimmune hepatitis (AIH) is a chronic inflammation of liver with unclear etiology. It is frequently associated other autoimmune diseases, and its association with celiac disease (CD) is well established. In this article, we describe the case of a 50-year-old male with long-standing AIH taking azathioprine for 10 years, evaluated for flares in transaminases. Despite adding high-dose corticosteroids, his transaminases and bilirubin remained high. Serology for CD was ordered, which revealed elevated tissue transglutaminase antibody IgG and endomysial IgA, which was further confirmed by endoscopic biopsy. Strict gluten-free diet was advised and now for over 2 years he is in remission with azathioprine and budesonide. This emphasizes the role of gluten-free diet in reversing liver dysfunction in patients with AIH, and clinicians should consider screening for CD in patients with AIH with persistent elevation of liver enzymes despite immunosuppressant treatment.