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Published on: December 15, 2011
Clinical characteristics of a concurrent condition of IgG4-RD and Castleman's disease
Xia Zhang1, Panpan Zhang1, Linyi Peng1
1Department of Rheumatology, Peking Union Medical College Hospital, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, China.
Insights
This study identifies IgG4-related disease (IgG4-RD) and Castleman
Area of Science:
- Rheumatology and Immunology
- Pathology
- Clinical Medicine
Background:
- Immunoglobulin G4-related disease (IgG4-RD) and Castleman's disease (CD) present with overlapping clinical and histopathological features.
- Distinguishing between IgG4-RD and CD can be challenging when both conditions are present, necessitating the provisional definition of IgG4-CD.
- Understanding the distinct clinical characteristics of IgG4-CD is crucial for accurate diagnosis and management.
Purpose of the Study:
- To review and characterize the clinical features of IgG4-CD, a condition exhibiting features of both IgG4-RD and CD.
- To compare the clinical manifestations and laboratory findings of IgG4-CD with those of definite IgG4-RD and multicentric Castleman's disease (MCD).
- To evaluate the clinical outcomes of patients diagnosed with IgG4-CD.
Main Methods:
- Retrospective analysis of a prospectively acquired database from China's largest IgG4-RD and Mimicry cohort.
- Definition of IgG4-CD based on histopathological criteria fulfilling diagnoses for both IgG4-RD and CD.
- Comparison of clinical features (organ involvement) and laboratory markers (serum IgG4, IgG, IgE, ESR, CRP, IL-6) between IgG4-CD, IgG4-RD, and MCD cohorts.
Main Results:
- Fifteen patients (2.8%) met the criteria for IgG4-CD.
- IgG4-CD patients demonstrated increased lymph node involvement compared to IgG4-RD patients, who had more salivary gland involvement.
- IgG4-CD patients exhibited significantly higher levels of inflammatory markers (ESR, CRP) and immunoglobulins (IgG, IgE, IgG4) than IgG4-RD patients, and distinct profiles compared to MCD patients, with generally favorable outcomes.
Conclusions:
- IgG4-CD represents a distinct clinical entity with specific organ involvement patterns and laboratory findings.
- Patients with IgG4-CD show a unique immunological profile compared to IgG4-RD and MCD.
- IgG4-CD patients appear to have a relatively favorable prognosis, though the long-term relationship between CD and IgG4-RD requires further elucidation.
Abstract:
IgG4-related disease (IgG4-RD) and Castleman's disease (CD) share similar clinical manifestations. When the histopathology coincides with the diagnosis of both IgG4-RD and CD, it is hard to depart the two disease entities utterly; here we call it IgG4-CD provisionally. In this study, we aim to review the clinical features of IgG4-CD. This study is based on a retrospective analysis of a prospectively acquired database. IgG4-CD was defined histopathologically in patients who fulfilled the diagnosis of both IgG4-RD and CD. Forty-five definite IgG4-RD and 16 multicentric CD (MCD) patients were recruited as controls. Clinical features including organ involvement, serum IgG4, IgG, IgE, ESR, CRP, and IL-6 levels were collected and analyzed. Fifteen patients (2.8%) out of 534 patients with IgG4-RD in China's largest prospective IgG4-RD and Mimicry cohort fulfilled the definition of IgG4-CD. There were 14 males and 1 female, whose mean age was 47 ± 18 years old, and the median disease duration before diagnosis was 12 (1-132) months. Eight patients have allergic disease history. IgG4-CD patients had more lymph node involvement (100 vs 57.8%, P < 0.01), while IgG4-RD patients had more submandibular (33.3 vs 77.1%, P < 0.01) and parotid gland (13.3 vs 40.9%, P < 0.05) affected. IgG4-CD patients had significantly higher levels of ESR, CRP, IgG, IgG1, IgG3, IgG4, and IgE than IgG4-RD patients. Compared with MCD patients, IgG4-CD patients showed higher incidence of salivary gland and paranasal sinus involvement, higher hemoglobin, eosinophil count, serum IgG4 level and IgG4/IgG ratio, and lower CRP, IL-6 levels, and IgG1/IgG, IgG2/IgG ratio. All patients with IgG4-CD exhibited relatively favorable outcomes. Both IgG4-RD and CD can involve multiple organs. There are a small group of patients who had clinical and pathological characteristics of both CD and IgG4-RD showed better clinical outcome. In the long-term prognosis of these patients, the relationship of CD and IgG4-RD is waiting to be further elucidated.
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