Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences · 2026
[Clinical characterization and prognosis of connective tissue disease-associated hemophago-cytic syndrome in adults].
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Yuan Xujing, Luo Zhijun, Song You, Wang Weiwei, Lin Wenxun, Wu Jiajia, DU Rong
Аннотация
OBJECTIVE: To investigate the clinical characteristics and outcomes of macrophage activation syndrome (MAS) associated with connective tissue diseases in adults.
METHODS: We 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.
RESULTS: A 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+ T lymphocytes, and C-reactive protein-to-albumin ratio (CAR), but higher triglyceride levels and percentages of B lymphocytes. Thirteen patients (11.9%) died during hospitalization. Multivariable Logistic regression analysis identified total protein, albumin, globulin, and D-dimer as independent factors associated with in-hospital mortality. ROC curve analysis showed that the model combining total protein and D-dimer yielded an area under the curve (AUC) of 0.872 for predicting in-hospital mortality, compared with 0.820 for total protein alone and 0.659 for D-dimer alone.
CONCLUSION: Patients 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.
METHODS: We 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.
RESULTS: A 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+ T lymphocytes, and C-reactive protein-to-albumin ratio (CAR), but higher triglyceride levels and percentages of B lymphocytes. Thirteen patients (11.9%) died during hospitalization. Multivariable Logistic regression analysis identified total protein, albumin, globulin, and D-dimer as independent factors associated with in-hospital mortality. ROC curve analysis showed that the model combining total protein and D-dimer yielded an area under the curve (AUC) of 0.872 for predicting in-hospital mortality, compared with 0.820 for total protein alone and 0.659 for D-dimer alone.
CONCLUSION: Patients 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.