TY - JOUR T1 - Scleroderma Lung Involvement, Autoantibodies, and Outcome Prediction: The Confounding Effect of Time JF - The Journal of Rheumatology JO - J Rheumatol SP - 404 LP - 406 DO - 10.3899/jrheum.170055 VL - 44 IS - 4 AU - SVETLANA I. NIHTYANOVA AU - CHRISTOPHER P. DENTON Y1 - 2017/04/01 UR - http://www.jrheum.org/content/44/4/404.abstract N2 - Systemic sclerosis (SSc) remains a poorly understood disease and so far none of the routinely used immunosuppressive treatments has been definitively shown to benefit longterm disease outcome. Scleroderma-related cardiopulmonary involvement has been the leading cause of death in more recent decades, and pulmonary fibrosis (PF) and pulmonary hypertension (PH) account for a substantial proportion of the SSc-related deaths, as demonstrated by several large metaanalyses1,2,3.Early detection and monitoring of PF and PH may benefit outcome by permitting earlier intervention in severe or progressive cases4. Multiple attempts have been made to develop prediction models, both for development and for outcome, in already present SSc-related lung disease. Consistently, autoantibody specificities are among the strongest but not the only predictors of organ disease in scleroderma patients, and autoantibody characterization is a mandatory part of investigations in new SSc cases.It is well established that while positivity for anticentromere antibodies (ACA) is associated with the limited cutaneous subset of the disease and a low risk of pulmonary or renal involvement, presence of antitopoisomerase I antibodies (ATA) conveys a substantially increased risk of PF development; moreover anti-RNA polymerase antibodies (ARA) are strongly associated with the diffuse cutaneous subset (dcSSc) and development of scleroderma renal crisis (SRC)5. Most cohort studies describe no particular association between ARA positivity and PH or PF development. Although this was true in an unadjusted analysis, when correcting for other variables, ARA positivity was shown to associate with an increase in the hazard of PH development in a large single-center cohort analysis6. In addition, compared to ACA-positive patients, those carrying ARA have been … Address correspondence to Dr. S.I. Nihtyanova, Centre for Rheumatology and Connective Tissue Diseases, UCL Medical School, Royal Free Campus, Rowland Hill Street, London NW3 2PF, UK. E-mail: s.nihtyanova{at}ucl.ac.uk ER -