ArticleBeijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences2026
[Clinical characterization and prognosis of connective tissue disease-associated hemophago-cytic syndrome in adults].
Article in Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
objectiveTo investigate the clinical characteristics and outcomes of macrophage activation syndrome (MAS) associated with connective tissue diseases in adults.
methodsWe retrospectively reviewed patients aged ≥16 years with MAS who were hospitalized in the Department of Rheumatology and Immunology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, between 2012 and 2022. The distribution of underlying rheumatic diseases was summarized, and demographic, clinical, and laboratory characteristics were compared across disease groups. The patients were further classified according to in-hospital outcome as those who died during hospitalization or were discharged alive. Univariate and multivariable Logistic regression analyses were performed to identify factors associated with in-hospital mortality. Receiver operating characteristic (ROC) curve analysis was used to evaluate the predictive performance of individual variables and the combined model for in-hospital mortality.
resultsA total of 109 patients with MAS were included. The mean age at MAS onset was (36.39± 13.65) years, and 82 patients (75.2%) were female. Fever and hyperferritinemia were among the most common clinical and laboratory findings. Systemic lupus erythematosus (SLE)-associated MAS and adult-onset Still's disease (AOSD)-associated MAS were the two most common subtypes, accounting for 48.6% (53/109) and 25.7% (28/109) of cases, respectively. Patients with AOSD-associated MAS had a longer duration of fever than those with SLE-associated MAS. Compared with patients with AOSD-associated MAS, those with SLE-associated MAS had significantly lower white blood cell and platelet counts, ferritin levels, percentages of CD4
conclusionPatients with SLE-associated MAS and AOSD-associated MAS differed in several clinical and laboratory features, suggesting clinical heterogeneity of MAS across different underlying rheumatic diseases. Assessment and management of MAS should therefore take the underlying disease into account. In-hospital mortality remained substantial in this cohort, and the combination of total protein and D-dimer showed potential value for risk stratification of in-hospital mortality.
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