Interstitial lung disease in systemic sclerosis: data from the spanish scleroderma study group.

Area of Science:

• Rheumatology
• Pulmonology
• Internal Medicine

Background:

• Systemic sclerosis (SSc) is a complex autoimmune disease with significant multi-organ involvement.
• Interstitial lung disease (ILD) is a major cause of morbidity and mortality in SSc patients.
• Understanding the clinical characteristics and outcomes of ILD in SSc is crucial for patient management.

Purpose of the Study:

• To evaluate the clinical characteristics of ILD in a large cohort of SSc patients.
• To analyze differences in ILD based on SSc subtypes (limited cutaneous SSc, diffuse cutaneous SSc, SSc sine scleroderma).
• To identify factors associated with moderate-to-severe lung function impairment, mortality, and causes of death in SSc-ILD.

Main Methods:

• A descriptive study utilizing data from the Spanish Scleroderma Study Group registry.
• Inclusion of 1374 SSc patients from 21 referral centers.
• Analysis of ILD prevalence, clinical features, lung function (FVC, DLco), HRCT patterns, and mortality across SSc subtypes.

Main Results:

• 43% of SSc patients (595/1374) had ILD, with varying prevalence across subtypes: 53% in lcSSc, 40% in dcSSc, 7% in ssSSc.
• ILD in lcSSc and ssSSc presented later with less severe lung function impairment and fewer ground-glass patterns on HRCT compared to dcSSc.
• Factors associated with FVC < 70% included dcSSc, anti-topoisomerase I antibodies, ground-glass pattern, active capillaroscopy, lower DLco, older age at onset, and longer time to ILD diagnosis.

Conclusions:

• Interstitial lung disease is a significant complication in all SSc subtypes, associated with considerable morbidity and mortality.
• The diffuse cutaneous SSc subtype is linked to more severe ILD and higher mortality.
• Pulmonary arterial hypertension is a notable cause of death, particularly in lcSSc-associated ILD.