Article Text

FRI0512 IGG4-Related Disease Is Associated with A History of Malignancy
  1. Z. Wallace1,
  2. C. Wallace2,
  3. L. Lu1,
  4. H. Choi2,
  5. J. Stone2
  1. 1Rheumatology Unit
  2. 2Rheumatology, Massachusetts General Hospital, Boston, United States


Background IgG4-related disease (IgG4-RD) is a fibroinflammatory disease of unclear etiology1. Some studies suggest that IgG4-RD predisposes patients to malignancy or is a forme fruste of cancer2, 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.

  1. Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012;366(6):539–551.

  2. 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

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