Celiac Disease Presenting as Cardiomyopathy - A Rare Extra Intestinal Manifestation

Abstract

Cardiac manifestations of celiac disease has been poorly described in literature though some studies have emphasized on correlation of ischemic heart disease, atrial fibrillation and dilated cardiomyopathy with celiac disease. We describe a patient with celiac disease associated cardiomyopathy whose cardiac function improved substantially after treatment with a gluten-free diet. A young lady of 35 years age was admitted with complaints of chronic diarrhea, vomiting, significant weight loss, dizziness and chronic iron deficiency anemia since last 3 months. Physical examination revealed tachycardia, tachypnoea and hypotension requiring multiple high dose inotropic support. She had pallor, skin changes and pedal edema suggestive of malabsorption. Investigations revealed iron deficiency anemia, high bilirubin and transaminases, prothrombin time, low albumin and elevated tissue trasglutaminase antibody. Echocardiography showed global hypokinesia with a left ventricular ejection fraction of 20%. Diagnosis of celiac disease made on clinic features, serology and biopsy finding of subtotal villous atrophy, crypt hyperplasia, and increased intraepithelial lymphocytes. She was kept on gluten free diet and on intravenous steroids for acute celiac crises and celiac related cardiomyopathy as patient had severe diarrhea, hypocalcaemia, weight loss, hypoproteinemia and hypotension. Patient improved within 48 hours of steroid with discontinuation of inotropes. She was discharged with gluten free diet and follow up after 6 months showed near normalization of all biochemical abnormalities and ejection fraction of 55%. Cardiomyopathy associated with celiac disease and celiac crisis is a serious and potentially lethal condition. However, with early diagnosis and treatment with a gluten free diet, and steroids cardiomyopathy in patients with celiac disease may be completely reversible.