Association of celiac disease and portal hypertension: Cirrhotic or noncirrhotic

Sir, We read with great interest the article “Celiac disease and chronic liver disease: Is there a relationship” published recently [1]. Association of celiac disease (CD) and chronic liver disease (CLD) has been well described [2, 3]. In this series, the mean age of patients was 28.8 years. Duration of symptoms of cirrhosis was relatively longer (83.7 months) as compared to natural history of cirrhosis and 12 % patients presented with splenomegaly [4]. Eighty percent had cryptogenic cirrhosis (CC) and most of the symptoms of CLD improved significantly on gluten-free diet (GFD). In India, noncirrhotic portal fibrosis (NCPF) is an important cause of portal hypertension (PHTN) seen in up to 18 % cases [5]. NCPF presents in the third and fourth decade and in 20 % to 30 % of cases the liver slowly undergoes parenchymal atrophy with subsequent decompensation [6, 7]. In a study of liver explants, which were initially thought to be having CC, NCPF was found to be an important cause of end-stage CLD considered for liver transplantation [8]. In a recent study, NCPF was found to be associated with CD in 12 % of cases [3]. Etiology of NCPF is largely not known; however, infection and immune mechanisms may play important roles, which have also been described in the pathogenesis of CD. We analyzed case records of CD and PHTN from January 2012 to December 2013 and nine patients were found to have both. Etiology of PHTN was EHPVO in two cases and CLD in seven cases (five cryptogenic, one hepatic venous outflow tract obstruction, and one Wilson’s disease). The mean (SD) duration of symptoms in five patients of cryptogenic cirrhosis was 77.2 months (34.1) before diagnosis, and three patients presented with massive splenomegaly. These results are similar to the series published in your journal. It may be possible that some of the cases of CC with CD, mainly those with long duration of symptoms, young age, large spleen, and responding to GFD, may actually be the cases of NCPF which needs to be studied in larger prospective studies with histology.