Celiac Disease Is Common in Adults With Cryptogenic Cirrhosis and Responds Favorably to Gluten-Free Diet

INTRODUCTION: Liver involvement is common in celiac disease (CeD), and up to 4.6% of patients with cryptogenic cirrhosis have CeD. We investigated the prevalence of CeD in patients with cryptogenic cirrhosis and assessed liver-related outcomes in them on GFD when compared with a propensity score-matched cohort of patients with cryptogenic cirrhosis without CeD. METHODS: Consecutive patients with cryptogenic cirrhosis were screened for CeD using IgA anti-tissue transglutaminase antibody (anti-tTG) followed by antiendomysial antibody and duodenal and liver biopsies, on which IgA/anti-tTG colocalization studies were performed. These patients and a cohort of patients with cryptogenic cirrhosis without CeD (1:4 CeD: no CeD matched using propensity score matched for age, sex, Child-Turcotte-Pugh [CTP] and model for end-stage liver disease [MELD]) were initiated on GFD plus standard of care and standard of care, respectively, and followed up for liver-related outcomes for 1 year. RESULTS: Of 232 patients with cryptogenic cirrhosis, 14 had high anti-tTG Ab (16.9 ± 10.5 fold rise), with 9 antiendomysial antibody-positive and 11 (4.7%) biopsy-proven CeD. IgA/anti-tTG Ab colocalization was demonstrated in 7/8 liver and 10/11 duodenal biopsies. Patients with cryptogenic cirrhosis with definite CeD (n = 11) and matched cohort without CeD (n = 44) were similar at baseline (age: 31.3 ± 7.7 vs 31.8 ± 9.3 years; 5 [45.5%] vs 15 [34.1%] females; MELDNa 9 [interquartile-range: 8-15.5] vs 12 [9-15]; CTP 7 [6-7.5] vs 6 [5.75-7]). Patients with CeD on GFD improved significantly on follow-up compared with those without CeD (follow-up MELDNa: 9 [7.5-10.5] vs 18.5 [12-20]; P = 0.001 and follow-up CTP: 5 [5-5] vs 8 [7-9]; P < 0.001) with less frequent further decompensations and similar mortality (9.1% vs 18.2%; P = 0.67). DISCUSSION: Approximately 4.7% of patients with cryptogenic cirrhosis have biopsy-proven CeD, and their liver-related outcomes improve with GFD.