In a multivariate analysis, the absence of liver disease symptoms at the disease presentation (OR 11.509; 95% CI 1.210–109.421; p = 0.033), the presence of hepatic non-necrotizing epithelioid cell granulomas (OR 17.754; 95% CI 1.805–174.631; p = 0.014), higher serum IgM (OR 1.535; 95% CI 1.067–2.208; p = 0.020) and higher blood leukocyte count (OR 2.356; 95% CI 1.170–4.747; p = 0.016) were independent risk factors associated with ILD in PBC. Here, CD40LG is linked to primary biliary cholangitis.