Malignancy is associated with systemic sclerosis in between 3.6 and 10.7% of patients diagnosed with systemic sclerosis. The diagnosis of systemic sclerosis may occur before, concurrent with, or after the diagnosis of malignancy. All published reports of malignancy with systemic sclerosis are reviewed. Lung cancer is the most frequent type of cancer seen in patients with systemic sclerosis, followed by breast cancer. Risk factors for the development of malignancy in patients with systemic sclerosis are female gender, increased age, and diffuse systemic sclerosis. Autoantibodies such as anticentromere and antitopoisomerase I are inconsistent in their risk for developing malignancy. Vigilance is recommended in this group of patients based on their increased risk of developing cancer.
Key Points
* Malignancy is found in up to 10% of patients with systemic sclerosis. Risk factors for malignancy include female gender, increasing age, and the presence of diffuse systemic sclerosis.
* Lung cancer and breast cancer remain the most frequently reported types of malignancy in series dealing with systemic sclerosis and malignancy.
* Other common malignancies observed in systemic sclerosis patients include gastrointestinal, urogenital, and lymphatic malignancies.
* There is no consistent relationship reported between the presence of malignancy and typical autoantibodies followed in systemic sclerosis patients.