Predictive markers for development of severe organ involvement in patients with systemic sclerosis

Prognosis of systemic sclerosis (SSc) is associated with the extent of skin involvement and the presence of lung, heart, kidney, and/or digestive tract damage. Most patients do not require disease-modifying therapy. However, to avoid irreversible tissue injury, early detection of visceral involvement is crucial for prompt initiation of therapy. The aim of this study is to identify, among initial investigations performed at time of diagnosis, predictive markers for the development of severe organ involvement (SOI). We retrospectively reviewed the medical records of 41 patients with SSc who were followed for 5 years after diagnosis, including those who died because of SSc within this 5-year period. We considered 68 clinical, biological, and technical parameters obtained at time of diagnosis and studied their association with development of SOI during this 5-year period, using a multivariate logistic regression. Eighteen patients (44%) developed SOI, with a median delay of 1.5 years following diagnosis. Three independent markers were identified: reduced vital capacity, fulfilment of American College of Rheumatology criteria at diagnosis, and presence of rheumatoid factor. To identify patients at risk for developing severe disease, given the short delay between diagnosis of SSc and development of SOI, we recommend monitoring these markers at diagnosis.