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Clinical characteristics of a concurrent condition of IgG4-RD and Castleman's disease.
Clin Rheumatol 2018; 37(12):3387-3395CR

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.

Authors+Show Affiliations

Department 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.Department 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.Department 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.Department 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.Department of Hematology, Peking Union Medical College Hospital, Beijing, China.Department of Pathology, Peking Union Medical College Hospital, Beijing, China. fengruie1@163.com.Department 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. zhangwen91@sina.com.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

29948354

Citation

Zhang, Xia, et al. "Clinical Characteristics of a Concurrent Condition of IgG4-RD and Castleman's Disease." Clinical Rheumatology, vol. 37, no. 12, 2018, pp. 3387-3395.
Zhang X, Zhang P, Peng L, et al. Clinical characteristics of a concurrent condition of IgG4-RD and Castleman's disease. Clin Rheumatol. 2018;37(12):3387-3395.
Zhang, X., Zhang, P., Peng, L., Fei, Y., Zhang, W., Feng, R., & Zhang, W. (2018). Clinical characteristics of a concurrent condition of IgG4-RD and Castleman's disease. Clinical Rheumatology, 37(12), pp. 3387-3395. doi:10.1007/s10067-018-4165-4.
Zhang X, et al. Clinical Characteristics of a Concurrent Condition of IgG4-RD and Castleman's Disease. Clin Rheumatol. 2018;37(12):3387-3395. PubMed PMID: 29948354.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Clinical characteristics of a concurrent condition of IgG4-RD and Castleman's disease. AU - Zhang,Xia, AU - Zhang,Panpan, AU - Peng,Linyi, AU - Fei,Yunyun, AU - Zhang,Wei, AU - Feng,Ruie, AU - Zhang,Wen, Y1 - 2018/06/14/ PY - 2017/12/24/received PY - 2018/05/31/accepted PY - 2018/05/09/revised PY - 2018/6/28/pubmed PY - 2019/7/2/medline PY - 2018/6/28/entrez KW - Castleman’s disease KW - Clinical feature KW - Cohort KW - IgG4-related disease SP - 3387 EP - 3395 JF - Clinical rheumatology JO - Clin. Rheumatol. VL - 37 IS - 12 N2 - 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. SN - 1434-9949 UR - https://www.unboundmedicine.com/medline/citation/29948354/Clinical_characteristics_of_a_concurrent_condition_of_IgG4_RD_and_Castleman's_disease_ L2 - https://dx.doi.org/10.1007/s10067-018-4165-4 DB - PRIME DP - Unbound Medicine ER -