Immunoglobulin G4-related diseases mimics Rosai-Dorfman diseases: a series of 6 cases in China
10.3760/cma.j.issn.1007-7480.2018.02.004
- VernacularTitle:模拟免疫球蛋白G4相关疾病的罗道病临床分析
- Author:
Li WANG
1
;
Panpan ZHANG
;
Shuoning SONG
;
Wei ZHANG
;
Xinxin CAO
;
Ruie FENG
;
Wen ZHANG
Author Information
1. 100730,中国医学科学院北京协和医院风湿免疫科风湿免疫病学教育部重点实验室
- Keywords:
Histiocytosis,Sinus;
Pathologic processes;
Prognosis;
Immunoglobulin G4-related diseases
- From:
Chinese Journal of Rheumatology
2018;22(2):87-90
- CountryChina
- Language:Chinese
-
Abstract:
Objective To summarize the clinical and pathologic characteristics of patients with RosaiDorfman disease (RDD) mimicking immunoglobulin (Ig) G4-related disease (IgG4-RD).Methods Retrospectively analyze the clinical manifestations,laboratory tests,pathologic features,treatment and prognosis of RDD patients whose clinical presentations mimicked IgG4-RD in Peking Union Medical College Hospital during January 2015 to June 2017.Results Six RDD patients mimicking IgG4-RD were described,which accounted for 1.5％ of the 450 registered IgG4-RD patients.All patients were male,with the median age of 53 year and the median disease duration of 12 months.All patients had extra-nodal involvements,of which the locations included spinal cord (3/6),intracranial (2/6),skin (2/6) and liver (1/6).Increased serum IgG4 (＞1 350 mg/L) was found in 4 cases (1 360-6 410 mg/L).Seven specimens were obtained from these patients.The number of IgG4-positive plasma cells was found to be more than 50 per HPF in 3 cases,30 per HPF in 1 case.The IgG4/IgG ratio was 40％ in 2 specimens,between 10％ and 30％ in 2 specimens and 10％ in 2 specimens.All patients were treated by glucocorticoids and 4 of them were treated with combined cyclophosphamide.Five patients got partial remission by these treatments while 1 patient withdrew from further follow up.Conclusion RDD is one of the mimics of IgG4-RD.There are several differences in lab tests and pathologic features between RDD and IgG4-RD.Before the IgG4-RD is diagnosed,RDD should be excluded by specific pathologic manifestations at the first place.
