Systemic Sclerosis Association with Malignancy

The association of systemic sclerosis (SSc) and cancer is well known from several decades suggesting common genetic and environmental risk factors involved in the development of both diseases. Immunosuppressive drugs widely used in SSc may increase the risk of cancer occurrence and different SSc cli...

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Bibliographic Details
Published in:Clinical reviews in allergy & immunology 2022-12, Vol.63 (3), p.398-416
Main Authors: Lepri, Gemma, Catalano, Martina, Bellando-Randone, Silvia, Pillozzi, Serena, Giommoni, Elisa, Giorgione, Roberta, Botteri, Cristina, Matucci-Cerinic, Marco, Antonuzzo, Lorenzo, Guiducci, Serena
Format: Article
Language:English
Subjects:
Allergology
Autoantibodies
Autoimmune diseases
Autoimmunity
Breast cancer
Cancer
Cancer patients
Cyclophosphamide
Development and progression
DNA topoisomerase
DNA-directed RNA polymerase
Drug development
Fibrosis
Immune checkpoint inhibitors
Immunology
Immunosuppressive agents
Internal Medicine
Lung cancer
Malignancy
Medicine
Medicine & Public Health
Patients
Risk factors
RNA polymerase
Scleroderma
Scleroderma (Disease)
Serology
Systemic scleroderma
Systemic sclerosis
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Summary:The association of systemic sclerosis (SSc) and cancer is well known from several decades suggesting common genetic and environmental risk factors involved in the development of both diseases. Immunosuppressive drugs widely used in SSc may increase the risk of cancer occurrence and different SSc clinical and serological features identify patients at major risk to develop malignancy. In this context, among serological features, presence of anti-RNA polymerase III and anti-topoisomerase I autoantibodies seems to increase cancer frequency in SSc patients (particularly lung and breast cancers). Lung fibrosis and a long standing SSc pulmonary involvement have been largely proposed as lung cancer risk factors, and the exposure to cyclophosphamide and an upper gastrointestinal involvement have been traditionally linked to bladder and oesophagus cancers, respectively. Furthermore, immune checkpoint inhibitors used for cancer therapy can induce immune-related adverse events, which are more frequent and severe in patients with pre-existing autoimmune diseases such as SSc. The strong association between SSc and cancer occurrence steers clinicians to carefully survey SSc patients performing periodical malignancy screening. In the present review, the most relevant bilateral relationships between SSc and cancer will be addressed.
ISSN:1559-0267
1080-0549
1559-0267
DOI:10.1007/s12016-022-08930-4