Celiac disease is common in adults with cryptogenic cirrhosis and responds favourably to gluten-free diet

Abstract

Introduction: Liver involvement is common in celiac disease (CeD), and upto 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 to a propensity-score matched (PSM) 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 anti-endomysial antibody (AEA) 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 PSM for age, sex, Child-Turcotte-Pugh(CTP) and model for end-stage-liver-disease(MELD)] were initiated on GFD plus standard of care (SOC) and SOC, respectively and followed up for liver-related outcomes for one year. Results: Of 232 patients with cryptogenic cirrhosis, 14 had high anti-tTG Ab (16.9±10.5 fold rise), with 9 AEA 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[IQR]: 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 to 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). Conclusion: Approximately 4.7% of patients with cryptogenic cirrhosis have biopsy-proven CeD and their liver-related outcomes improve with GFD.