Clinical characterisis and prognosis analysis of 107 adult onset Still's disease
10.3760/cma.j.issn.1007-7480.2016.03.008
- VernacularTitle:成人斯蒂尔病107例临床特点及预后分析
- Author:
Zhou QIAO
;
Long LI
;
Zhou BIN
- Publication Type:Journal Article
- Keywords:
Still's Disease,adult-onset;
Clinical characteristics;
Prognostic analysis
- From:
Chinese Journal of Rheumatology
2016;20(3):181-186
- CountryChina
- Language:Chinese
-
Abstract:
Objective To analyze the clinical characteristics, laboratory results, treatment and prognosis of adult onset Still's disease (AOSD). Methods The clinical and laboratory data of 107 AOSD patients were retrospectively analyzed. SPSS 22.0 was used for statistical analysis. Categorical data were described as frequency and percentage, and numerical data were described using x±s deviation. Chi-square test was used to compare categorical data between groups and analysis of variance test was used to compare numerical data between groups. K-W test was used to compare skewed distributed data between groups and J-T test was compared in pairwise comparison. Logistic regression was applied to examine prognostic factors. Results The average age of 107 patients (12 male and 95 female) was(39±16) years and the average disease course was (1.4±2.7) years. The patients were classified into 3 types based on their disease course: monocyclic (38 cases, 35.5%), polycyclic (29 cases, 27.1%) and chronic arthritis (40 cases, 37.4%). The clinical characteristics were high fever (107 cases , 100%), rash (97 cases, 90.7%) and arthralgia/arthritis (86 cases, 80.4%). The most common involved joints were knee (24 cases, 23.3%), wrist (19 cases, 18.4%) and proximal interphalangeal joints (14, 13.7%). The laboratory results were elevated including white blood cells count (88/105, 83.8%) and elevated serum ferritin (107, 100%). Seventy-three out of 84 patients (86.9%) had negative rheumatic factor and 9 out of 91 (9.9%) had positive ANA, which were low titre in general. Eleven patients (10.3%) had joint erosions suggested by X-ray at diagnosis. Intergroup analysis revealed that patients with polycyclic type were less likely to have abnormal liver function tests at disease onset (χ2=9, P<0.05). Twenty-five patients (23.8%) in total had complications, which were mainly pulmonary interstitial fibrosis (9 cases ) and hemophagocytic syndrome (7 cases). Male patients (n=6, χ2=5.93, P=0.015) were more likely to have complications. One hundred and two (95.3%) patients were treated with glucocorticoid and among them, 86 (84.3%) were combined with immunosuppressive agents. After 4 weeks of treatment, 89 patients (89.2%) achieved complete remission. The number of patients with glucocorticoid dependence or treatment failure was less in monocyclic group, although the differences were not significant. The analysis of prognostic factors founded that patients with Tmax>39.5 ℃ (P=0.030) were mostly fell into the monocyclic group. Compared with monocyclic type, patients with polycyclic or chronic arthritis type were less likely to have thrombocytosis (P=0.019, P=0.004). However, when compared with chronic arthritis type, patients with monocyclic or poly - cyclic type were less likely to have arthralgia/arthritis (P=0.030, P=0.000), especially the involvement of large joints (P<0.05). Conclusion AOSD mainly presents as high fever, rash, arthralgia and elevated inflammatory markers. Joint erosion may occur during the course of disease. The treatment of glucocorticoid and immunosup - pressive agent combinations is effective and most patients could achieve remission during 4 weeks of treatment. Patients with thrombocytosis at disease onset might be monocyclic type, while patients with arthritis, especially involvement of large joints at disease onset might be chronic arthritis type.
