Study of 43SLEpatients with autoimmune liver cirrhosis: emphasis on clinical features and differences from lupus without cirrhosis

Abstract 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.