Systemic sclerosis and malignancy

Systemic sclerosis (SSc) has increased risk of morbidity and mortality due to pulmonary fibrosis, pulmonary arterial hypertension, renal crisis, infections and malignancies. Chemical exposure, smoking and cytotoxic drugs increase the malignancy risk in rheumatic diseases including SSc. We aim to evaluate characteristics, identify risk factors and mortality in SSc patients with malignancies.

One hundred and fifty-three patients with SSc (24 male, 129 female, with mean age of 56.4 ± 12.9 years) according to 2013 American College of Rheumatology/European League Against Rheumatism classification criteria for SSc were enrolled in this study. Clinical, demographic, laboratory and radiological characteristics of the patients were evaluated by using SPSS 17.0. Chi-square test was used to compare qualitative data and descriptive statistics are shown as n (%).

Seven cases of cancer (three male, four female, with mean age of 51.2 ± 7.0 years) were identified. The tumor types included lung cancer, gastrointestinal tract cancer (gastric adenocarcinoma and gastric neuroendocrine tumor), myelodysplastic syndrome and malignant melanoma (eye and skin). Five (3.4%) died due to infection, cardiopulmonary involvement and renal crisis. Six of them had a diffuse cutaneous SSc clinical subtype, and the ratio for anti-Scl-70 was 71.4%. There was no significant relationship between age, sex, anti-Scl-70, pulmonary and cardiac involvement between cancer and non-cancer patients.

The malignancies may occur before, together or after the diagnosis of SSc. Both of these disorders have increased morbidity and mortality compared with the general population. Rational treatment, monitoring and reducing the risk factors may prevent the development of malignancy. Also, the clinical features, risk factors and prevalence ratios are similar to that reported by large cohorts.