Immunoglobulin G4-related diseases mimics Rosai-Dorfman diseases: a series of 6 cases in China / 中华风湿病学杂志

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.