Clinical characteristics of a concurrent condition of IgG4-RD and Castleman’s disease

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.

Steroid Therapy and Steroid Response in Autoimmune Pancreatitis

“…The pathological mimickers of AIP or IgG4-related diseases, such as Castleman's disease [47], follicular pancreatitis [48], anti-neutrophil cytoplasmic antibody-associated vasculitides [49,50], inflammatory myofibroblastic tumor [51], and lymphoma [52], are listed from the viewpoints of lymphoplasmacytic proliferation.…”

Malignant transformation of biliary adenofibroma combined with benign lymphadenopathy mimicking advanced liver carcinoma: A case report

“…Lymphadenopathy showed no evidence of malignancy. It was first suspected to be IgG4-related or multicentric Castleman’s disease due to its histology and positive IHC staining for IgG4[ 10 , 11 ]. However, the patient’s serum IgG4, IgE, and IL-6 levels were within the normal range; therefore, we did not reach a definite diagnosis for her lymphadenopathy.…”

Immunoglobulin G4-related lymph node disease with an orbital mass mimicking Castleman disease: A case report

“…IgG4-RD share similarities with Castleman disease, but there are also differences. MCD and IgG4-RD can be distinguished based on the following aspects: (1) Clinical manifestations: lymph node enlargement in MCD is more prominent and is often accompanied by fever, anemia, severe hypoproteinemia, and other systemic symptoms, while lymph node lesions of IgG4-RD are usually less than 2 cm in diameter and often involve the lacrimal glands, salivary glands, pancreas, and retroperitoneum; (2) Inflammation indicators: CRP, IL-6, and vascular endothelial growth factor are usually significantly increased in MCD patients; (3) In terms of immunoglobulin and complement, increased IgG in MCD patients may be accompanied by increased IgA and IgM, with normal complement levels, while the course of IgG4-RD may involve the activation process of complement, leading to decreased complement levels; (4) Pathological features: IgG4+ plasma cells may appear in MCD patients, but IgG4+/IgG+ plasma cells usually account for less than 40%; and (5) Therapeutic response of glucocorticoids: IgG4-RD patients respond well to initial treatment with glucocorticoids, while MCD patients generally respond poorly[ 4 , 23 - 25 ].…”

“…Castleman disease is a clinically rare lymphoproliferative disorder. Castleman disease and IgG4-RD may present some common clinical manifestations, such as enlarged lymph nodes and elevated serum IgG4 levels, which make the clinical diagnosis and differential diagnosis more difficult and challenging[ 4 ]. Here, we report a case of IgG4-related lymph node disease with an orbital mass mimicking Castleman disease and review the relevant literature.…”