Objective: To investigate the clinical characteristics of systemic lupus erythematosus accompanied by autoimmune liver cirrhosis (SLE‐ALC) patients and differences from the non‐cirrhosis group. Methods: Forty‐three patients with SLE‐ALC were enrolled in this study from 2653 patients with SLE in Peking University People's Hospital. A descriptive case–control study was performed between SLE‐ALC patients and the entry time‐matched non‐cirrhosis group. Results: Among the 43 SLE‐ALC patients, 41 (95.3%) were female. Eight patients (18.6%) were first found to have cirrhosis and then diagnosed with SLE. Eighteen patients (41.9%) had jaundice and 27 (62.8%) had esophageal and gastric varices. The age of SLE‐ALC patients was 51.1 ± 17.2 years, which was significantly older than the non‐cirrhosis group (P < 0.001). Lung involvement was more common as initial manifestations in SLE‐ALC patients during the SLE course (P=0.027). Compared with the non‐cirrhosis group, SLE‐ALC patients had worse liver function. A significantly higher rate of hematological system involvement (anemia, leucopenia, and thrombocytopenia) and a higher level of immunoglobulins were observed in SLE‐ALC patients (P<0.05). Moreover, SLE‐ALC patients displayed a lower positive rate of anti‐double‐stranded DNA and anti‐ribosomal P protein (P<0.05). The most common radiologic manifestations are ascitic fluid (72.1%) and splenomegaly (71.4%) in SLE‐ALC patients. Six SLE‐ALC patients underwent liver biopsy, and interface hepatitis was present in all patients. Conclusions: Cirrhosis is rare in SLE patients but is manifested as a unique pattern of clinical features characterized by late‐onset age, lung involvement, high immunoglobulins, and impaired liver function. [ABSTRACT FROM AUTHOR]