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Incidence of malignancy in systemic sclerosis: A systematic review and meta-analysis
Journal article   Peer reviewed

Incidence of malignancy in systemic sclerosis: A systematic review and meta-analysis

Wei Shan Teoh, Jaivikash Raghupathy, Abhiram Kanneganti, Hanis Abdul Kadir, Gim Gee Teng, Pearl Shuang Ye Tong, Lauren V Host, Kathleen Morrisroe, Mandana Nikpour, Andrea Hsiu Ling Low, …
Seminars in arthritis and rheumatism, Vol.79, 153006
08/2026
DOI: 10.1016/j.semarthrit.2026.153006
PMID: 42322931

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Abstract

SSc is associated with increased malignancy risk, a growing cause of mortality in SSc. However, standardised incidence ratios (SIR) vary widely across studies and different malignancy subtypes. This meta-analysis aimed to determine the SIR of different malignancy subtypes and the temporal relationship with SSc diagnosis. A systematic review and meta-analysis were conducted by searching Embase, MEDLINE and Cochrane Library databases from inception to 7 December 2024. Cohort studies reporting SIRs of malignancies in SSc patients were included. Studies where SIR was not reported were excluded. Random-effects models were used to calculate pooled estimates, while study quality was assessed using the Newcastle-Ottawa Scale. This review is registered on PROSPERO (CRD42023445876). Thirty-one cohort studies comprising at least 32,134 SSc patients were included. Overall malignancy risk was significantly increased (SIR 1.66; 95% CI 1.32 - 2.08). Highest risks were observed for esophageal, liver, cervical, lung, and haematological malignancies (SIR 3.35 - 13.95), while breast malignancy showed modest increased risk (SIR 1.34, 95% CI 1.00 - 1.80) that was not statistically significant. High-quality studies confirmed these associations except for esophageal malignancy, with only one high quality study for liver malignancy. There was a mean interval of 7.4 years (95% CI 5.3 - 9.5) between SSc and malignancy diagnosis. Our findings confirm an increased malignancy risk, particularly lung, haematological and cervical malignancies, and an approximate 7-year interval between SSc diagnosis and malignancy development. Further studies are needed to clarify the risk factors for different malignancy subtypes to develop cancer screening strategies. NIL
Scleroderma Cervical Hematological Lung Malignancy

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