Analysis of clinical characteristics in 85 adult patients with autoimmune disease-associated hemophagocytic syndrome
10.3760/cma.j.cn141217-20250306-00060
- VernacularTitle:成人自身免疫病合并噬血细胞综合征85例临床特点分析
- Author:
Jianyao LIAO
1
;
Zhijian HUANG
;
Meili MO
;
Yan ZHAO
;
Lidan ZHAO
Author Information
1. 中国医学科学院 北京协和医学院 北京协和医院风湿免疫科 国家皮肤与免疫疾病临床研究中心 疑难重症及罕见病国家重点实验室 风湿免疫学教育部重点实验室，北京 100730
- Publication Type:Journal Article
- Keywords:
Hemophagocyte syndrome;
Autoimmune disease;
Macrophage activation syndrome;
Adult-onset Still′s disease;
Lupus erythematosus , systemic
- From:
Chinese Journal of Rheumatology
2025;29(10):830-838
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To investigate the clinical characteristics of adult patients with autoimmune disease-associated hemophagocytic syndrome (AID-HPS) and enhance clinical recognition of this condition.Methods:A retrospective analysis was performed on 85 adult AID-HPS patients admitted to the department of rheumatology and immunology at Peking Union Medical College Hospital from January 2012 to December 2020. Clinical data included demographics, predisposing factors, manifestations, laboratory/imaging findings, treatments, and outcomes. Patients were stratified into three groups based on underlying AIDs: adult-onset Still′s disease with HPS (AOSD-HPS) group, systemic lupus erythematosus with HPS (SLE-HPS) group, and other AID with HPS (other AID-HPS) group. Comparative analyses were conducted to identify intergroup differences. Continuous variables were analyzed using one-way ANOVA, Welch′s test, or Kruskal-Wallis′s test based on data distribution and homogeneity of variance. Categorical variables (rates and proportions) were compared with the chi-square test or Fisher-Freeman-Halton exact test according to expected cell frequencies.Results:Among 85 patients, 67 were female. Underlying AIDs included AOSD (32 cases, 37.6%), SLE (32 cases, 37.6%), and other AIDs (21 cases, 24.7%). Infections (≥1 type) were identified in 54 patients (63.5%), predominantly viral (48 cases, 56.5%), including cytomegalovirus (CMV) (40 cases), Epstein-Barr virus (EBV) (11 cases), and 6 cases were coinfected with both CMV and EBV. All patients presented with fever; hepatomegaly, splenomegaly, and lymphadenopathy were observed in 39 (45.9%), 59 (69.4%), and 70 (82.4%) cases, respectively. Additional manifestations included arthralgia (63 cases, 74.1%) and rash (65 cases, 76.5%). Pancytopenia in 31 cases (36.5%) and bicytopenia in 29 cases (34.1%). Liver dysfunction was noted in 79 cases (92.9%). Elevated lactate dehydrogenase (LDH) (83 cases, 97.6%), elevated triglyceride (TG) (76 cases, 89.4%), decreased fibrinogen (Fbg) (55 cases, 64.7%), and elevated serum ferritin (SF) (84 cases, 98.8%) were common. Elevated soluble CD25(sCD25) (53cases) and reduced NK cell activity (49 cases) were observed. Bone marrow examination revealed hemophagocytosis in 49 cases. There were statistically significant differences in age( F=3.763, P=0.031), lymphadenopathy( χ2=7.098, P=0.029), rash( χ2=12.816, P=0.002), reductions in WBC( H=22.284, P<0.001)、NEU( H=18.882, P<0.001)、PLT( H=15.127, P=0.001), and elevations in LDH( H=7.842; P=0.020)、TG( H=6.177, P=0.046)、CRP( H=6.915, P=0.032)、SF( H=9.661, P=0.008)、sCD25( χ2=5.154, P=0.035) among the three groups: (1) The SLE-HPS group was significantly younger [(28.1 ± 10.4) years) than the other AID-HPS group [(39.5 ± 17.3) years, P=0.028]. (2) The AOSD-HPS group had higher incidence rates of lymphadenopathy (93.8%) and rash (93.8%) than the SLE-HPS group (68.8% and 56.3%, respectively), (lymphadenopathy: χ2=7.098, P=0.029; rash: χ2=12.816, P=0.002). (3) ① WBC in the SLE-HPS group [1.62 (1.18, 2.92) ×10 9/L] were significantly lower than those in the AOSD-HPS group [5.66 (2.75, 11.57)×10 9/L] and the other AID-HPS group [6.05 (2.49, 14.55)×10 9/L] ( Z=-4.032, P<0.001; Z=3.993, P<0.001). ② NEU in the SLE-HPS group [1.10 (0.60, 1.93)×10 9/L] were markedly reduced compared to the AOSD-HPS group [3.73 (1.54, 9.04)×10 9/L] and the other AID-HPS group [2.23 (1.43, 11.57)×10 9/L] ( Z=-3.859, P<0.001; Z=3.506, P=0.001). ③ PLT in the SLE-HPS group [59.50 (28.50, 81.00)×10 9/L] were significantly lower than those in the AOSD-HPS group [109.00 (65.75, 232.00)×10 9/L] and the other AID-HPS group [150.00 (55.00, 221.00)×10 9/L] ( Z=-3.421, P=0.002; Z=3.179, P=0.004). (4) LDH levels in the AOSD-HPS group [1 178 (645, 1 875) U/L] were significantly higher than those in the other AID-HPS group [598 (410, 771) U/L] ( Z=2.795, P=0.016). (5) TG levels in the SLE-HPS group [3.61 (2.46, 6.09) mmol/L] were significantly higher than those in the other AID-HPS group [2.68 (1.71, 3.30)mmol/L] ( Z=2.402, P=0.049). (6) CRP and SF levels in the AOSD-HPS group [79.20 (28.02, 179.53)mg/L and 30 225 (13 494, 53 598)μg/L, respectively] were significantly higher than those in the SLE-HPS group [26.05 (9.41, 83.31)mg/L and 9 862 (4 467, 22 315) μg/L, respectively] ( Z=2.547, P=0.033; Z=3.069, P=0.006 ). (7) The incidence rates with elevated sCD25 in the AOSD-HPS group (100.0%) was significantly higher than that in the other AID-HPS group (76.9%), ( χ2=5.154, P=0.035). After treatment, 83 patients improved, while 2 deaths occurred in the other AID-HPS group. Conclusion:Adult AID-HPS predominantly affects young to middle-aged females, with SLE and AOSD being the most common underlying AIDs. The condition manifests with severe clinical features, frequently triggered by viral infections (particularly CMV and EBV). Distinct differences in clinical and laboratory profiles exist among AID-HPS subtypes. Early recognition and aggressive treatment are critical for improving prognosis.
