Summary

As yet, there is no conclusive evidence linking systemic scleroderma to an increased risk for malignant diseases. However, based on case-reports and the results of several controlled studies involving hundreds of patients, a small but significant increase in the risk of cancer was found in association with PSS.

The 5-year survival rate in scleroderma patients with cancer was worse for men than women, and poorer for patients with a later onset of the disease. Scleroderma and cancer may develop sequentially or together in elderly patients. The occurrence of both diseases may represent depression of the normal immune response.

No consistent pattern of HLA class was found in scleroderma patients with cancer. There is no consistent association between immunosuppressive treatment (given for scleroderma) and the development of malignant disease.

The histological changes brought about by the scleroderma in the affected organs may predispose to the development of the malignancy. The fibrous tissue in the lungs, or the structural changes in the esophagus of patients with systemic sclerosis and involvement of these organs, may provide the necessary environment for the neoplastic changes leading to the development of the malignant disease.

Since the immune response of patients with autoimmune diseases (PSS among them) is altered, this may place them at a greater risk of malignancies. Vigilance and further studies are required to elucidate the question of the association between scleroderma and cancer.

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