Clinical characteristics and prognosis of concomitant systemic lupus erythematosus and primary biliary cholangitis.

OBJECTIVE: This study aimed to identify the clinical characteristics of systemic lupus erythematosus-primary biliary cholangitis (SLE-PBC) patients and to compare the manifestations and prognosis with systemic lupus erythematosus (SLE) patients.

METHODS: Twenty-one inpatients with concomitant SLE and primary biliary cholangitis (PBC) were identified in our hospital. Baseline clinical manifestations, laboratory results, disease activity, and organ damage, as well as changes in disease manifestations and therapies, were retrospectively analyzed. Baseline clinical characteristics, survival rate, and flare-ups were compared with 254 SLE patients also from our hospital.

RESULTS: The prevalence of concomitant PBC in SLE inpatients was 0.27%. Over half of the patients were diagnosed with SLE and PBC simultaneously. Compared with SLE patients, SLE-PBC patients started the symptom of SLE at an older age, with a longer delay before the diagnosis of SLE (P < 0.05). Hematological and muscular involvement, pulmonary arterial hypertension, and interstitial lung disease were more common in SLE-PBC patients (P < 0.05). Kaplan-Meier estimate showed a significantly lower survival rate in SLE-PBC group, with 3-year survival rate at 88.4%.

CONCLUSION: Concomitant PBC might have a negative impact on the survival of SLE, with older age at SLE onset, longer delay before SLE diagnosis, and higher baseline damage. More intensive therapy and prevention of hepatic toxicity need to be considered. Key Points • Hematological and muscular involvement, PAH, and ILD were more common in SLE PBC than in SLE. • The study firstly reported the survival rate of SLE PBC patients. • More intensive therapy and prevention of hepatic toxicity are needed for SLE-PBC.