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Rosai-Dorfman disease mimicking IgG4-related diseases: a single-center experience in China
Li Wang1, Wei Li1,2, Shangzhu Zhang1
1Departments of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, The Ministry of Education Key Laboratory, National Clinical Research Center for Dermatologic and Immunologic Diseases, Beijing, 100730, China.
Background:
Rosai-Dorfman disease (RDD) and IgG4-related (IgG4-RD) disease are both rare diseases, but in some cases, RDD mimics IgG4-RD clinically and pathologically. RDD mimicking IgG4-RDs (RDD mimic IgG4-RD), referring to disease initially diagnosed as IgG4-RD but finally pathologically confirmed to be RDD, is a clinically rare and confusing disease. To summarize the characteristics of this disease, we prospectively analyzed the clinical features, laboratory parameters, pathological characteristics, treatment and prognosis of patients diagnosed with RDD mimic IgG4-RD. Moreover, by analyzing characteristics of RDD mimic IgG4-RD, RDD and IgG4-RD, we further compared the similarities and differences between RDD and IgG4-RD.
Results:
7 patients with RDD mimic IgG4-RD were included in this study and all of them had extranodal organ involvement, especially the central nervous system, which occurred in 5 patients (71.4%). Although serum IgG4 level was elevated in 6 cases (1360-54,100 mg/L), overall, it was still lower than that in IgG4-RD patients. Furthermore, we found a new cut-off value of serum IgG4 concentration for differentiating RDD and IgG4-RD with higher specificity. Pathological findings of RDD also showed features resembling IgG4-RD: IgG4-positive plasma cell enrichments were observed in all RDD mimic IgG4-RD patients, and the proportion of IgG4/IgG in tissues was 10-40% in 4 patients and more than 40% in 2 patients. However, none of the RDD mimic IgG4-RD patients or RDD patients displayed obliterative phlebitis or storiform fibrosis. Most of the RDD mimic IgG4-RD patients were treated with glucocorticoids combined with immunosuppressants, and a good prognosis was obtained following treatment.
Conclusions:
RDD has clinical manifestations that mimic IgG4-RD. However, detailed differences in laboratory parameters and pathological characteristics are present between these two diseases. Our study underlines the necessity to rule out RDD while diagnosing IgG4-RD using pathological findings as the identification criteria and provides advice for both differentiating these two diseases and clinical treatment of RDD mimic IgG4-RD.
Insights
Rosai-Dorfman disease (RDD) can mimic IgG4-related disease (IgG4-RD) clinically and pathologically. This study identifies key differences in laboratory and pathological findings to help differentiate these rare conditions and guide treatment for RDD mimicking IgG4-RD.
Area of Science:
- Pathology
- Immunology
- Rare Diseases
Background:
- Rosai-Dorfman disease (RDD) and IgG4-related disease (IgG4-RD) are rare conditions.
- RDD can present with clinical and pathological features that mimic IgG4-RD, leading to diagnostic challenges.
- RDD mimicking IgG4-RD is a rare entity requiring careful differentiation.
Purpose of the Study:
- To characterize RDD mimicking IgG4-RD.
- To compare the clinical, laboratory, and pathological features of RDD mimicking IgG4-RD with RDD and IgG4-RD.
- To provide guidance for differentiating these diseases and managing RDD mimicking IgG4-RD.
Main Methods:
- Prospective analysis of 7 patients diagnosed with RDD mimicking IgG4-RD.
- Evaluation of clinical features, laboratory parameters, and pathological characteristics.
- Comparison with established data for RDD and IgG4-RD.
Main Results:
- RDD mimicking IgG4-RD frequently involves extranodal organs, including the central nervous system.
- Elevated serum IgG4 levels were observed, but generally lower than in IgG4-RD.
- Pathological findings showed IgG4-positive plasma cell enrichment, but lacked obliterative phlebitis and storiform fibrosis, distinguishing features from IgG4-RD.
- A new cut-off value for serum IgG4 was identified for better differentiation.
- Patients treated with glucocorticoids and immunosuppressants showed good prognosis.
Conclusions:
- RDD can present with features overlapping IgG4-RD, necessitating careful pathological examination for accurate diagnosis.
- Distinct laboratory and pathological characteristics differentiate RDD from IgG4-RD.
- Pathological findings are crucial for ruling out RDD in suspected IgG4-RD cases.
- This study aids in differentiating RDD mimicking IgG4-RD and informs treatment strategies.
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