FRI0500 BEHÇET SYNDROME AND CENTRAL NERVOUS SYSTEM INVOLVEMENT: A DESCRIPTIVE STUDY IN A COHORT OF PATIENTS IN OUR RHEUMATOLOGY DEPARTMENT

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. 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