Background/Purpose: There is minimal guidance on specific cancer screening practices for rheumatic diseases (RDs) other than for myositis. The aim of this scoping review was to evaluate the available evidence on the risk of cancer for various RDs and associated risk factors.

Methods: We evaluated cancer associations and risk factors associated with incident cancer development for key systemic RDs: RA, SSc, and SS. We conducted a comprehensive literature search via PubMed to identify relevant publications with the following search terms: “systemic sclerosis” or “Sjogren’s disease” or “rheumatoid arthritis” and “cancer” or “malignancy” or “risk” or “risk factors.” Data for cancer associations was limited to meta-analyses. Associated risk factors for incident cancer were obtained from meta-analyses and retrospective/prospective observational studies.

Results: We reviewed 19 manuscripts: 6 for cancer associations and 13 for risk factors. There were 3 meta-analyses for RA, 3 for SSc, and 1 for SS that reflected standardized incidence ratios (SIRs) for cancer. SS had 6 publications describing risk factors associated with cancer, followed by SSc (4) and RA (3). SSc was most commonly associated with liver cancer or lung cancer, RA with lymphoma and SS with hematologic malignancies, in particular non-Hodgkin’s lymphoma (Table 1). Incident cancer risk for SSc was most commonly associated with anti-transcriptional intermediary factor 1 (TIF-1γ) positivity (Table 1). RF and anti-CCP positivity were associated with lung cancer for patients with RA (Table 1). SS literature identified the highest cancer association with low complement levels and lymphocytopenia (Table 1). For RA and SSc, interstitial lung disease (ILD) was identified for both as a risk factor associated with malignancy.

Conclusion: RA, SSc, and SS are uniquely associated with different cancers. High-powered prospective studies to inform evidence-based, expert driven screening guidelines are needed to identify effectiveness of various cancer screening recommendations and guide appropriate clinical care.

Table 1. Cancer associations and risk factors for incident cancer in SSc, RA, and SS.

Figure 1. Cancer screening in rheumatic diseases.

ACR Meeting Abstracts - https://acrabstracts.org/abstract/cancer-screening-for-patients-with-rheumatic-diseases-a-scoping-review/