Prevalence of Celiac Disease in adult patients with Non-Cirrhotic Portal Fibrosis

Background and Aim: Celiac Disease (CeD) is a multisystemic disease and in addition to the small intestine, also affects liver. The spectrum of liver involvement in CeD includes cryptogenic hypertransaminasaemia/cirrhosis, autoimmune liver diseases and vascular liver diseases like Non-cirrhotic portal fibrosis (NCPF). There is lack of systematic study on the prevalence of CeD in patients with NCPF. Methods: All new patients with biopsy-proven NCPF reporting to clinic were recruited. Additionally, database of all other biopsy-proven patients with NCPF was reviewed and they were invited to participate in study. All were screened for CeD using IgA anti-tissue transglutaminase antibody (anti-tTG Ab). Anti-tTG Ab-positive patients were subjected to anti-endomysial antibody (AEA) testing and duodenal biopsy examination. Liver biopsies and corresponding duodenal biopsies of patients with NCPF and CeD were subjected to IgA/anti-tTG colocalization studies using immunohistochemistry. Results: Seventy-two patients with biopsy-proven NCPF were recruited. Of them, 9 had anti-tTG Ab positive suggesting seroprevalence of CeD of 12.5%. Most of these patients had strongly positive anti-tTG Ab (mean fold rise was 22 times above the upper limit of normal). Duodenal biopsies showed villous abnormalities of modified Marsh grade 3 in 7 patients suggesting a prevalence of CeD in them to be 9.7%. AEA was positive in 6 of 7 patients having villous abnormalities. IgA anti-tTG Ab colocalization in liver and duodenum was demonstrable in all 7 and 6 patients respectively. All patients of NCPF with CeD were started on gluten-free diet (GFD) and being followed. Conclusion: The seroprevalence and prevalence of biopsy-proven CeD in patients with biopsy-proven NCPF are 12.5% and 9.7% respectively, which is substantially more than prevalence of CeD in general population. While effects of GFD on liver in patients with NCPF are unclear, based on data of this preliminary study, we suggest that patients with NCPF should be screened for CeD.