Association of Systemic Lupus Erythematosus and Primary Biliary Cholangitis

Background and Aim: Comorbid systemic lupus erythematosus (SLE) and primary biliary cholangitis (PBC) are uncommon. Therefore, this study reviewed published studies involving patients with comorbid SLE and PBC.

Methods: A literature search and review of patients with comorbid SLE and PBC were conducted.

Results and Conclusion: Among the 27 patients with comorbid SLE and PBC, 96.3% (26/27) were women, 62.5% (15/24) were initially diagnosed with PBC, and three patients were diagnosed with both SLE and PBC almost simultaneously. Sjögren’s syndrome was the most common autoimmune complication associated with comorbid SLE and PBC (18.5% and 5/27). Immune thrombocytopenia was diagnosed in two patients (7.4%). Moreover, two patients had familial PBC. Three patients died: two elderly patients with liver failure as a result of worsening PBC and one with an unknown etiology.