Background and Aim: This study aimed to determine the presence of concomitant extrahepatic autoimmune disease (EAD) in patients with autoimmune liver disease (ALD) and the efficacy of the treatment response of ALD with the presence of any EAD. Materials and Methods:Between January 2001 and November 2017, 241 patients with ALD were included in the study. Results:Of the 241 patients, 88, 134, and 19 had autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and overlap syndrome (OS), respectively. Thirty-one patients had cirrhosis: 77% and 23% had compensated and decompensated disease, respectively. The presence of at least one EAD was defined in 38.6% of the patients with ALD (n=93), and 12% of them had ≥1 EAD. EAD was most commonly seen in patients with OS and PBC compared with those with AIH (p=0.036). Autoimmune thyroid disease was the most common association (20%), followed by Sjogren syndrome (12.0%). At the end of the follow-up period, 165 patients (72%) had biochemical response. The presence of EAD did not affect the biochemical response. Conclusion:EAD is most frequently seen in patients with ALD. The presence of EAD is not associated with the treatment response.

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