Abstract
Systemic sclerosis-related interstitial lung disease (SSc-ILD) is the leading cause of death in SSc. In this study, we aimed to describe the baseline severity and evolution of forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DLCO) in patients with SSc-ILD and to assess the baseline clinical, biological and high-resolution CT scan (HRCT) predictors of this evolution. Baseline and serial FVC and DLCO were collected in 75 SSc-ILD patients followed during 6.4±4.2 years (n = 557 individual data). FVC and DLCO evolution was modelled using a linear mixed model with random effect. During follow-up, FVC was stable while DLCO significantly decreased (-1.5±0.3%/year (p<0.0001). Baseline NYHA functional class III/IV, extensive SSc-ILD on HRCT and DLCO<80% were associated with a lower baseline FVC. Absence of digital ulcers extensive SSc-ILD, and FVC<80% and were associated with a lower baseline DLCO. Presence or history of digital ulcers and presence of pulmonary hypertension at baseline or during follow-up were associated with a faster decline of DLCO overtime. Neither age, gender, subtype of SSc nor specificity of autoantibodies were associated with baseline severity or outcome of lung function tests. In this SSc-ILD population, FVC was therefore stable while DLCO significantly declined over time. ILD extension was associated with baseline FVC and DLCO but not with their evolution. Presence or history of digital ulcers and pulmonary hypertension were predictors of a faster decline of DLCO over time.
Highlights
In systemic sclerosis (SSc), pulmonary complications, namely interstitial lung disease (ILD) and pulmonary hypertension (PH), are frequent and the leading cause of mortality in SSc patients [1,2,3,4,5]
We found in our study that a proportion of our patients (29%) experienced a drop of more than 10% of forced vital capacity (FVC) during the first 72 months of follow up (Fig 2) but the overall FVC curve was flat when modelled by linear mixed model (LMM)
When assessing the factors associated with a higher risk if a significant deline of FVC of more than 10% from baseline, we found that the initial extension of ILD on highresolution computed tomography scan (HRCT) scan as well as baseline DLCO was significantly associated with this risk
Summary
In systemic sclerosis (SSc), pulmonary complications, namely interstitial lung disease (ILD) and pulmonary hypertension (PH), are frequent and the leading cause of mortality in SSc patients [1,2,3,4,5]. Treating ILD is challenging because recent trials have shown no or an apparently modest benefit of immunosuppressants [6, 7] in patients with mostly stable ILD. Predicting the outcome of ILD in SSc is important because treating patients whose lung function tests are worsening or expected to worsen is an important current strategy aimed at improving the benefit/risk ratio of immunosuppressants [8,9,10]. In many centres managing SSc patients, ILD is often mild and there is a paucity of data on the PFTs outcome in these milder SSc-ILD. In most studies, worsening was defined as a decrease of more than 10% of forced vital capacity (FVC) and/or more than 15% of DLCO during serial PFTs [10]. Serial changes of FVC used as a continuous variable or lung transplantation/oxygen requirement were chosen as an endpoint [11]
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