Nationwide analysis of adult hospitalizations with hemophagocytic lymphohistiocytosis and systemic lupus erythematosus

Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a well-recognized complication of systemic lupus erythematosus (SLE). This study aims to characterize HLH with and without SLE in the US adult inpatient population.

Methods: We performed a retrospective study of HLH with and without SLE from the 2016-2019 National Inpatient Sample (NIS) database. We described the demographic characteristics of HLH with and without SLE. Multivariable analysis was performed to calculate odds ratios (OR) for in-hospital death.

Results: A total of 8690 hospitalizations had HLH. Of those 605 (7%) had SLE, and 8085 (93%) did not have SLE. Relative to the non-SLE group, the SLE group was younger, had more females, less whites, more African Americans, more Hispanics, and more Asian/Pacific Islanders. Over 60% of HLH with or without SLE had a concurrent infection. Sixty (9.9%) of HLH hospitalizations with SLE died compared to 1735 (21.5%) of those without SLE. Among HLH hospitalizations, multivariable analysis showed that age (OR 1.02; 95% C.I. 1.016-1.031), Charlson Comorbidity Index (OR 1.15; 95% C.I. 1.091-1.213), infections (OR 3.35; 95% C.I. 2.467-4.557), and leukemia/lymphoma (OR 1.46; 95% C.I. 1.112-1.905) had higher odds of in-hospital death. SLE did not increase the odds of death.

Conclusions: Inpatients with both HLH and SLE were younger, had a higher proportion of racial/ethnic minorities, and were predominately female. One out of every 10 hospitalizations for HLH ended in death but SLE itself was not an independent risk factor for death. Concurrent infection was the variable most associated with HLH death. Key Points • HLH and SLE group were younger and had higher proportions of female and racial/ethnic minorities. • SLE was not an independent risk factor for death in HLH patients.