A case control study of 14 adult-onset Still's disease patients complicated with macrophage activation syndrome
10.3760/cma.j.issn.1007-7480.2019.07.006
- VernacularTitle:成人斯蒂尔病合并巨噬细胞活化综合征14例临床分析
- Author:
Xiaopei YANG
1
;
Tiange WU
;
Wenlu HU
;
Jinlei SUN
;
Lei ZHANG
;
Wenjuan GUAN
;
Shengyun LIU
Author Information
1. 郑州大学第一附属医院风湿免疫科 450052
- Keywords:
Still's disease;
adult-onset;
Macrophage activation syndrome;
Glucocorticosteroids
- From:
Chinese Journal of Rheumatology
2019;23(7):459-464
- CountryChina
- Language:Chinese
-
Abstract:
Objective To summarize the clinical data of macrophage activation syndrome (MAS) in adult-onset Still's disease (AOSD) patients and provide evidence for clinical diagnosis and treatment. Methods We retrospectively reviewed the clinical data of AOSD with MAS patients in the First Affiliated Hospital of Zhengzhou University from January 2012 to August 2018, and compared with patients with AOSD alone. Data were analyzed by t-test, Mann-Whitney U test, x2 test or Fisher exact test. Results A total of 14 AOSD with MAS patients were enrolled, accounting for 7.6％(14/185) of AOSD patients at the same period, including 2 males and 12 females. The median duration of AOSD in MAS was 1.3 (0.25, 4) months. Compared with the AOSD group, the age of onset was younger in the MAS group (t=-2.038, P=0.037), and the proportion of splenomegaly (t=9.020, P=0.003), pericardial effusion (t=8.663, P=0.003), pleural effusion (t=4.754, P=0.029) was higher. The white blood cell count (t=-4.171, P<0.01), hemoglobin level (t=-2.661, P=0.008), platelet count (t=-5.672, P<0.01), neutrophil count (t=-5.082, P<0.01), albumin (t=-3.426, P<0.01), fibrinogen (t=-5.986, P<0.01), ESR (t=-2.941, P=0.003), CRP (t=-2.014, P=0.044) was significantly decreased, ALT (t=-3.227, P<0.01), AST (t=-3.105, P=0.002), triglyceride (t=-5.612, P<0.01), ferritin>2000 μg/L (t=7.833, P=0.005) was significantly increased. Fourteen patients with AOSD complicated with MAS were treated with glucocorticosteroids, 5 with methylprednisolone, 8 with cyclosporine A, 8 with intravenous immunoglobulin (IVIG), 2 with etoposide, and 1 with tocilizumab. After treatment, 11 cases recovered and 3 cases died. Conclusion Younger AOSD patients tend to complicated with MAS, especially at the early course of the disease, and splenomegaly occur more frequently clinically compared to patients without MAS. When blood cell count, fibrinogen and ESR decreases, triglyceride and ferritin levels increases in AOSD patients, the occurrence of MAS is indicated. Timely treatment can improve the prognosis of patients.
