Résumé
Systemic sclerosis (SSc) has a variable evolution but may be life-threatening owing to pulmonary, cardiac or renal involvement. Nailfold video capillaroscopy (NVC) is abnormal early in the disease and is crucial for diagnosis. An association between subtypes of scleroderma pattern and disease progression has been suggested. Therefore, we conducted a prospective study to assess whether capillaroscopy can identify SSc patients at risk of progression.
SCLEROCAP was a prospective multicentre observational study that included patients with a diagnosis of SSc followed up for three years. Each patient had yearly standard evaluation and NVC. Images were read by two observers blinded from each other and were classified into subtypes (2 for Maricq's and 3 for Cutolo's classification). Severe progression was defined as cardiac, pulmonary or renal involvement or progression and was assessed by a validation committee.
Three hundred and eighty-seven patients were included of whom 369 were followed-up and 53 (14 %) had severe progression. A simple model using Cutolo's capillaroscopic late stage, short duration of disease and age was as powerful in predicting severe progression as a model using all the parameters known to be predictive (AUC[95 %CI] 0.74[0.67–0.82] vs 0.73[0.64–0.77] respectively.
NVC is a predictor of severe progression and might be helpful for early therapeutic decisions in patients with SSc.