FRI0508 MALIGNANCY AND IGG4-RELATED DISEASE: THE INCIDENCE, RELATED FACTORS AND PROGNOSIS FROM A PROSPECTIVE COHORT STUDY IN CHINA.
Authors: H. Yang, H. Tang, P. Zhang, Y. Fei, H. Chen, X. Zhang, Y. Zhao, F. Zhang, W. Zhang
Journal: Annals of the Rheumatic Diseases (2020)

Background: The association between IgG4-related disease (IgG4-RD) and malignancies is unclear. No epidemiological data for malignancies in Chinese IgG4-RD patients is available. It is also important to know the risk factors and prognosis for IgG4-RD patients harboring malignancies. Objectives: To investigate the incidence, related factors and prognosis of IgG4-related disease (IgG4-RD) with malignancies in the Chinese cohort. Table 1. Baseline characteristic of IgG4-RD patients with malignancy Patient Sex Age Age at diagnosis of IgG4-RD Age at diagnosis of malignancy Serum IgG4 (g/L) Organs involvements of IgG4-RD (*Organ with biopsy) Sites of malignancy P1 F 59 58 54 1499 Parotid gland*, salivary gland Breast cancer P2 M 74 66 68 10402 Pancreas, bile duct, retroperitoneal fibrosis, kidney, prostate, lymph nodes Rectal cancer P3 M 46 42 40 2630 Lacrimal gland, parotid gland Lipoblastoma P4 M 70 68 64 5780 Pancreas, bile duct, lung, prostate, lymph nodes Thyroid carcinoma P5 F 62 61 61 11600 Pancreas, bile duct, salivary gland*, periaortitis, lymph nodes, pituitary Thyroid carcinoma P6 M 72 68 68 3490 Pancreas, bile duct, lymph nodes Rectal cancer P7 M 60 58 58 2410 Pancreas, bile duct, lymph nodes Renal cancer P8 M 68 63 68 3520 Pancreas, bile duct, retroperitoneal fibrosis, lung, kidney, artery, lymph nodes Rectal cancer P9 M 36 30 35 12400 Pancreas, bile duct Skin cancer P10 M 52 49 52 10000 Pancreas, parotid gland*, lacrimal gland, lung, prostate, lymph nodes Thyroid carcinoma P11 F 70 68 69 17300 Parotid gland, lacrimal gland, salivary gland, sinus Lung cancer P12 M 82 79 79 58000 Pancreas, lacrimal gland* Colon cancer P13 F 50 49 45 14300 Uterus*, ovary Ovarian carcinoma P14 F 52 46 50 10000 Parotid gland*, lacrimal gland Breast cancer P15 F 60 55 57 12500 Pancreas, parotid gland, lacrimal gland, lymph nodes, sinus Lymphoma P16 M 42 37 40 7490 Lung*, lymph nodes Renal cancer P17 M 71 68 69 415 Pancreas*, bile duct Prostate cancer Table 2. Related factors for malignancies in patients with IgG4-RD Variables Univariate analysis Multivariate analysis Univariate OR (95%Cl) P-value Multivariate OR (95%Cl) P-value Head and Neck involvement 0.304 (0.097- 0.952) 0.041 0.604 (0.152-2.401) 0.474 Autoimmune pancreatitis 5.359 (1.651-17.393) 0.005 6.230 (1.559-24.907) 0.010 Eosinophilia 0.117 (0.014-0.966) 0.046 0.094 (0.010-0.883) 0.039 Figure 1. Standardized incidence ratios of malignancies in patients with IgG4-RD from different studies. Methods: We prospectively analyzed the IgG4-RD patients recruited in Peking Union Medical College Hospital from January 2011 to August 2018 and identified patients diagnosed with IgG4-RD complicating malignancies. Data regarding demographics, clinical features, treatment and prognosis of IgG4-RD patients complicating malignancies were collected and compared to those of age- and sex-matched controls. Results: Among the 587 Chinese patients with IgG4-RD, 17 malignancies were identified. Ten of them developed malignancy after the diagnosis of IgG4-RD, given a standard incidence ratio (SIR) of 2.78 (95%CI 1.33-5.12). Multivariate logistic analysis indicated that autoimmune pancreatitis (OR= 6.230, 95%CI 1.559-24.907, p=0.010) was positively associated with malignancy, whereas eosinophilia (OR= 0.094, 95%CI 0.010-0.883, p=0.039) was negatively related with malignancies. During a median follow-up period of 61.4±26.4 months, all patients with IgG4-RD and malignancies survived. Conclusion: An increased incidence of malignancy was found in Chinese IgG4-RD cohort. Autoimmune pancreatitis is a potential risk factor, whereas eosinophilia is a possible protective factor for complicating malignancies. References: [1] Wallace, Z. S.,et al. Association of IgG4-Related Disease With History of Malignancy. Arthritis Rheumatol 68, 2283–2289 (2016). [2] Hirano, K. et al. Incidence of malignancies in patients with IgG4-related disease. Intern. Med. 53, 171–176 (2014). Disclosure of Interests: None declared