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SYSTEMIC SCLEROSIS

Choosing patients wisely when treating interstitial lung disease Richard M. Silver Systemic sclerosis-associated interstitial lung disease (SSc-ILD) requires accurate diagnosis and staging to identify patients with the highest risk of disease progression, who might benefit from treatment with immunosuppressants. New insights into predictors of mortality in patients with SSc-ILD should improve patient care and inform the design of future clinical trials. Refers to Goh, N. S. et al. Short term pulmonary function trends are predictive of mortality in interstitial lung disease associated with systemic sclerosis. Arthritis Rheumatol. http://dx.doi.org/10.1002/art.40130 (2017)

Although trends in survival might have improved1, the management of disease in patients with systemic sclerosis (SSc; sometimes known as scleroderma) remains a challenge. Unfortunately, in the 35 years since the introduction of captopril (an angiotensin-­ converting enzyme (ACE) inhibitor) as the first effective treatment for scleroderma renal crisis, we have not witnessed another therapeutic breakthrough of such magnitude. Despite efforts, major advances in the treatment of SSc-associated interstitial lung disease (SSc-ILD) and SSc-associated pulmonary arterial hypertension (SSc-PAH),

the two leading causes of SSc-related deaths2, have not been achieved. Even with the expanding pharmacologic armamentarium for PAH, patients with SSc-PAH generally do not respond as well to treatment as do patients with idiopathic PAH3. Moreover, reports of successful treatment of SSc-ILD with immunosuppressive agents4–6 must be regarded as being of only marginal benefit. Do these results truly reflect the performance of the drugs under investigation, or was the magnitude of improvement shown in these trials diminished by the patients chosen for each study? New research by Goh et al.7

highlights the importance of patient selection when it comes to planning clinical trials in SSc-ILD. Published a decade ago, the Scleroderma Lung Study (SLS‑I) was the first randomized controlled trial (RCT) to demonstrate a statistically significant effect (P 

Systemic sclerosis: Choosing patients wisely when treating interstitial lung disease.

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