Progressive interstitial lung disease in patients with systemic sclerosis-associated interstitial lung disease in the EUSTAR database

Abstract

ObjectivesTo identify overall disease course, progression patterns and risk factors predictive for progressive interstitial lung disease (ILD) in patients with systemic sclerosis-associated ILD (SSc-ILD), using data from the European Scleroderma Trials And Research (EUSTAR) database over long-term follow-up.MethodsEligible patients with SSc-ILD were registered in the EUSTAR database and had measurements of forced vital capacity (FVC) at baseline and after 12±3 months. Long-term progressive ILD and progression patterns were assessed in patients with multiple FVC measurements. Potential predictors of ILD progression were analysed using multivariable mixed-effect models.Results826 patients with SSc-ILD were included. Over 12±3 months, 219 (27%) showed progressive ILD: either moderate (FVC decline 5% to 10%) or significant (FVC decline >10%). A total of 535 (65%) patients had multiple FVC measurements available over mean 5-year follow-up. In each 12-month period, 23% to 27% of SSc-ILD patients showed progressive ILD, but only a minority of patients showed progression in consecutive periods. Most patients with progressive ILD (58%) had a pattern of slow lung function decline, with more periods of stability/improvement than decline, whereas only 8% showed rapid, continuously declining FVC; 178 (33%) experienced no episode of FVC decline. The strongest predictive factors for FVC decline over 5 years were male sex, higher modified Rodnan skin score and reflux/dysphagia symptoms.ConclusionSSc-ILD shows a heterogeneous and variable disease course, and thus monitoring all patients closely is important. Novel treatment concepts, with treatment initiation before FVC decline occurs, should aim for prevention of progression to avoid irreversible organ damage.

Rapidly Progressive Systemic Sclerosis–associated Interstitial Lung Disease After Intravesical Bacillus Calmette-Guérin Therapy for Early-stage Bladder Cancer

“…However, only a minority showed a pattern of continuously declining FVC and/or increasing extent of fibrosis with no periods of stability/improvement. 4 In our patient with longstanding stable disease, the development of rapidly progressive disease after a second course of intravesical BCG is unlikely to be coincidental. It is remarkable that despite a severe baseline skin disease, the flare following BCG therapy induced ILD progression without recurrent skin disease.…”

The Relationship between Pulmonary Damage and Peripheral Vascular Manifestations in Systemic Sclerosis Patients

“…It is also reported that the patients with dcSSc have a higher incidence of interstitial disease [ 1 , 2 , 3 ]. Predictors of severe restrictive lung disease (defined by a forced vital capacity (FVC) of 50% predicted) include African American ethnicity, male gender, the degree of physiological abnormalities at diagnosis (FVC and diffusing lung capacity for carbon monoxide (DLCO)) and a younger age [ 1 , 2 , 46 , 47 , 48 ].…”

Progression in the Management of Non-Idiopathic Pulmonary Fibrosis Interstitial Lung Diseases, Where Are We Now and Where We Would Like to Be

“…SSc-ILD has a heterogeneous and variable disease course and close monitoring is important in considering when to start treatment. In a recent published cohort study of 826 SSc-ILD patients, 27% showed progressive ILD (FVC decline >5%) during the initial 12-month period, and 67% experienced progression any time over the mean five-year follow-up [ 39 ]. In current clinical practice, treatment is often initiated after progression has occurred, and novel treatment concepts are needed in patients at risk of developing a progressive fibrosing phenotype that aim for the prevention of progression.…”