TY - JOUR T1 - The Ambitious Goal of Validating Prognostic Biomarkers for Systemic Sclerosis-related Interstitial Lung Disease JF - The Journal of Rheumatology JO - J Rheumatol SP - 1034 LP - 1036 DO - 10.3899/jrheum.130549 VL - 40 IS - 7 AU - FRANCESCO BONELLA AU - PAOLA CARAMASCHI Y1 - 2013/07/01 UR - http://www.jrheum.org/content/40/7/1034.abstract N2 - Interstitial lung disease (ILD) often complicates the course of systemic sclerosis (SSc), mainly in patients with the diffuse subset of disease and with anti-Scl70 antibody positivity1,2. Postmortem examination gives evidence that nearly all patients with SSc develop pulmonary fibrosis, although to a very different extent individually; high-resolution computed tomography (HRCT) shows interstitial lung involvement in about two-thirds of the cases2.Together with pulmonary arterial hypertension, severe pulmonary fibrosis is the leading cause of SSc-related death, as demonstrated by Steen and Medsger among the very large Pittsburgh cohort3. These data were confirmed by a study of the SSc population included in the European League Against Rheumatism Scleroderma Trials and Research group database4. With regard to the course of ILD, some patients develop severe and rapidly progressive interstitial lung involvement during the early phase of the disease, while others may show an indolent course without a relevant influence on pulmonary function or survival1,5. Assessment of lung involvement and close monitoring of lung function are therefore mandatory.During the last decade efforts have been undertaken to identify predictors of rapid deterioration in SSc lung interstitial involvement. The Scleroderma Lung Study (SLS) and the Genetics versus Environment in Scleroderma Outcome Study (GENISOS) assembled the largest cohorts for this purpose6,7. These efforts were intended mainly to drive treatment choice, but also to avoid serial HRCT scans for patient followup to reduce radiation exposure. One of the most important findings was that, similar to idiopathic pulmonary fibrosis8, the decline in forced vital capacity (FVC) and DLCO turned out to mirror the degree of changes observed on HRCT9. An accelerated rate of decline in FVC is associated with poor survival7. Despite these attempts, … Address correspondence to Dr. F. Bonella, Department of Pneumology and Allergy, Ruhrlandklinik, University Hospital, University of Duisburg-Essen, Tüschener Weg 40, 45239 Essen, Germany. E-mail: francesco.bonella{at}ruhrlandklinik.uk-essen.de ER -