Cancer Risk in Sjögren’s Disease
SJÖGREN’S disease was linked to higher cancer risk in patients and close relatives in this cohort study.
Researchers assessed cancer risk in 323 patients with primary Sjögren’s disease and 1,750 close relatives, including parents, siblings, and children. All patients met the 2016 ACR-EULAR classification criteria and were actively followed in a rheumatology outpatient clinic. Cancer history was gathered through face-to-face interviews and telephone surveys, and cancer incidence was compared with the general population of Türkiye.
Higher Malignancy Rates Observed
Among patients with primary Sjögren’s disease 9% of individuals had a history of malignancy. Nineteen cases were solid organ cancers and 10 were hematological malignancies. Breast cancer was the most common solid tumor. Over a median follow up of 3.6 years, the standardized incidence ratio for all cancers was 3.3, showing that patients developed malignancy at more than three times the expected rate in the general population.
The increase was especially striking for leukemia and lymphoma, which had a standardized incidence ratio of 22.5. Among female patients, breast cancer risk was also elevated, with a standardized incidence ratio of 3.8. These findings suggest that malignancy surveillance may deserve particular attention in this population, especially for hematological cancers.
Familial Cancer Risk Also Increased
The analysis also showed a higher burden of cancer among close relatives of patients with primary Sjögren’s disease. A total of 128 patients, or 43.3%, had at least one close relative with cancer, accounting for 176 cancer cases overall. The standardized incidence ratio for cancer among relatives was 3.5 compared with the general population.
Notably, malignancy risk in patients did not differ according to age, sex, smoking history, Schirmer test results, anti-SSA or anti-SSB status, ANA, complement levels, ESSDAI status, or focus score. However, cancer risk was higher when a close relative also had a history of cancer.
Clinical Implications
These findings suggest that cancer susceptibility in primary Sjögren’s disease may extend beyond the affected individual and could reflect shared familial or genetic mechanisms related to immune dysregulation. For clinicians, the results reinforce the need to remain alert to malignancy risk in patients with primary Sjögren’s disease and may also prompt closer attention to family history during routine assessment.
Reference
Tuzcu TU et al. Risk of Cancer in Patients With Primary Sjögren’s Disease and Their Relatives. Int J Rheum Dis. 2026;29(3):e70613.
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