Restrictive Lung Diseases

Progressive Interstitial Lung Disease Trends in Systemic Sclerosis-Associated ILD

Avatar photoVirginia A. Schad, PharmD, RPh
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Systemic Sclerosis and Scleroderma
Scleroderma is an autoimmune disorder where the body’s immune system attacks its own cells. Scleroderma results in the persistent hardening of the body’s connective tissues, for example the skin. It can affect blood vessels by causing them to harden and thicken, forming vascular lesions. In its most extreme state it can affect the vascular supply to the major organs of the body, eventually causing death.
Close monitoring of patients with systemic sclerosis-associated interstitial lung disease and awareness of the variable course of progression is highly important in considering when to initiate treatment.

Close monitoring of patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD) and awareness of the variable course of progression is highly important in considering when to initiate treatment, according to study results published in the Annals of the Rheumatic Diseases.

Some patients with SSc-ILD may develop progressive ILD, which is associated with higher mortality, and therefore current clinical practice emphasizes treatment initiation of patients with SSc-ILD who have progressive ILD. Because the prevalence of progressive ILD and the overall disease course and patterns of SSc-ILD are unknown, researchers sought to identify these as well as the risk factors predictive for progressive ILD in 826 patients with SSc-ILD using data from the European Scleroderma Trials and Research (EUSTAR) database over long-term follow-up.

Approximately 30% of patients with SSc-ILD experienced ILD progression during any 12-month period, and 67% of all patients with SSc-ILD experienced progression at any time over the mean 5-year follow-up. In addition, ILD patterns in patients with SSc-ILD are very heterogeneous, with most patients showing both progressive and stable periods. However, of all patients with progressive SSc-ILD, only a minority demonstrated a pattern of rapid, continuously declining forced vital capacity (FVC) with several consecutive episodes of FVC decline and no periods of FVC stability or improvement.

“These results highlight a pitfall in current clinical practice, where treatment is often initiated after FVC decline has happened, and thus when lung damage has already occurred,” the study authors concluded. “Novel treatment concepts, with treatment initiation before FVC decline occurs, should aim for prevention of progression to avoid irreversible organ damage.”

Disclosure: This clinical trial was supported by Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany. Please see the original reference for a full list of authors’ disclosures.  

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Reference

Hoffmann-Vold A-M, Allanore Y, Alves M, et al; on behalf of EUSTAR collaborators. Progressive interstitial lung disease in patients with systemic sclerosis-associated interstitial lung disease in the EUSTAR database. Ann Rheum Dis. Published online September 28, 2020. doi:10.1136/annrheumdis-2020-217455

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