FRI0512 IGG4-Related Disease Is Associated with A History of Malignancy. (15th July 2016)

• Record Type: Journal Article
• Authors: Wallace, Z.; Wallace, C.; Lu, L.; Choi, H.; Stone, J.
• Is Part Of: Annals of the rheumatic diseases. Volume 75(2016)Supplement 2
• Page Start: 624
• Page End: 624
• Publication Date: 2016-07-15
• Subjects: Rheumatism -- Periodicals; 616.723005
• DOI: 10.1136/annrheumdis-2016-eular.5862
• Languages: English
• ISSNs: 0003-4967

Abstract

Abstract : Background: IgG4-related disease (IgG4-RD) is a fibroinflammatory disease of unclear etiology 1 . Some studies suggest that IgG4-RD predisposes patients to malignancy or is a forme fruste of cancer 2, but we have frequently observed IgG4-RD patients with a history of malignancy preceding the clinical onset of IgG4-RD. Objectives: We sought to determine whether a history of malignancy was more common among patients at the onset of IgG4-RD compared to controls. Methods: We identified IgG4-RD patients with a history of invasive malignancy from a well-defined cohort of 125 patients and compared their malignancy history to those of two reference groups. First, we calculated a standardized prevalence ratio against general US population estimates from the Surveillance, Epidemiology, and End Results (SEER) database. Second, we identified up to five age- and gender-matched controls for each case and calculated the odds of malignancy among those with IgG4-RD compared to controls using conditional logistic regression. Results: The mean age at IgG4-RD onset was 50.3±14.9 years and 61% of the patients were male. Twenty (16%) had been diagnosed with 21 malignancies before the diagnosis of IgG4-RD. The observed prevalence of malignancy in this cohort was 2.5 times higher (95% CI:1.1–3.6) than expected compared to the SEER database. Compared with matched controls, a history of malignancy was more than three-fold higher in IgG4-RD (OR 3.1;95% CI:1.6–6.2). Conclusions: Our findings suggest that malignancy is associated with the subsequent development of IgG4-RD in a subset of patients with IgG4-RD. Potential explanations include shared risk factors for both IgG4-RD and cancer, the triggering by cancer of autoantigen expression leading to IgG4-RD, and an increased risk of IgG4-RD resulting from cancer treatment. References: Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012;366(6):539–551. Hart PA, Law RJ, Dierkhising RA, Smyrk TC, Takahashi N, Chari ST. Risk of cancer in autoimmune pancreatitis: A case-control study and review of the literature. Pancreas. 2014;43(3):417–421. Disclosure of Interest: None declared