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.
Highlights
Immunoglobulin G4-related disease (IgG4-RD) is a relatively recently described disease category which affects multipleCastleman’s disease (CD) is an atypical lymphoproliferative disorder, which can be classified into three types: plasma cell, hyaline vascular, and their mixture
It is widely accepted that a diagnosis of immunoglobulin G4 (IgG4)-RD can be made under a premise of ruling out a series of diseases
Histological examination of HE staining was performed in all tissue samples, as well as immunohistochemical staining with anti-CD3, anti-CD38, anti-CD138, anti-CD20, anti-CD21, anti-IgG, and antiIgG4 mAb
Summary
Castleman’s disease (CD) is an atypical lymphoproliferative disorder, which can be classified into three types: plasma cell, hyaline vascular, and their mixture. Plasma cell and mixed types frequently have systemic manifestations, which termed multicentric Castleman’s disease (MCD). It is widely accepted that a diagnosis of IgG4-RD can be made under a premise of ruling out a series of diseases. In some cases with CD, the histopathological findings, such as IgG4-positive plasma cell infiltrations, are evident [6,7,8]. When the pathological performance meets the diagnosis of both IgG4-RD and CD (IgG4-CD) simultaneously, how can we differentiate the two disease entities? Do the clinical course and treatment response differ from those of IgG4-RD or MCD? We attempted to determine the clinical differences between these three groups of patients using clinical data
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