Background/Purpose: Interstitial lung disease (ILD) is a common complication of systemic sclerosis (SSc) and is one of the leading causes of morbidity and mortality. Recently, several international associations (American Thoracic Society (ATS), European Respiratory Society (ERS), Japanese Respiratory Society (JRS), and the Latin American Thorax Association (ALAT)) have joined forces and developed new international criteria for the diagnosis of progressive pulmonary fibrosis (PPF). We aim to identify the factors associated with the development of PPF, according to the new criteria of the ATS/ERS/JRS/ALAT societies, in patients with SSc-ILD.

Methods: A retrospective study of 51 patients with SSc-ILD diagnosed in the rheumatology department of the University Hospital Complex of León since the year 2000. Demographic, clinical, analytical, radiological, and pulmonary function variables were collected. The fulfillment of the new PPF criteria was evaluated, as well as their prognostic value. We compared clinical manifestations, antibody status, treatment, malignancy and death rates. Continuous variables were compared by Student’s or Mann Whitney’s test, and categorical variables by Chi² test or Fisher’s exact test.

Results: Out of a total of 133 patients in our cohort, 51 patients with SSc-ILD were included, with 58.8% being women. The age at diagnosis of SSc and SSc-ILD was 59.2 ± 14.6 and 62.6 ± 14 years, respectively. 72.5% of the patients had limited cutaneous involvement and 45.1% were Anti-topoisomerase I positive. Tables 1 and 2 show the main clinical-serological and the morphological and functional pulmonary characteristics of our cohort. Twenty-two patients met the ATS/ERS/JRS/ALAT criteria over an 8.8 ± 7.3-year follow-up period from the diagnosis of SSc, representing 43.1% of the sample, with a mean time from the diagnosis of SSc-ILD of 36.7 ± 28.8 months. The proportion of patients with SSc-ILD who experienced progressive ILD during the first year was 17.6%, and 35.3% at five years. Smoking habits (p 0.010), younger age at diagnosis of SSc-ILD (p 0.049) and ESR levels (p 0.005) are independent factors for the presence of PPF (Table 3). No relationship was observed with sex or the different characteristics of the disease. Greater initial radiological involvement (extent by HRCT >20%, p 0.013) and functional impairment (%FVC, p 0.022; %DLCO, p 0.007) were related to the development of PPF. The use of corticosteroids (p 0.005), CYC (p 0.034), RTX (p 0.039) and Ninte (p 0.011) was higher in patients with PPF. Similarly, they received more combined therapeutic strategies (p 0.010). During follow-up, 14 patients died, 7 of them from complications related to ILD, with a higher incidence in progressive patients (27.3% vs. 3.4%, p 0.034).

Conclusion: Almost half of our cohort meets the criteria for PPF according to ATS/ERS/JRS/ALAT criteria. These patients present greater pulmonary involvement at diagnosis and higher pulmonary-related mortality.

« Back to ACR Convergence 2024

ACR Meeting Abstracts - https://acrabstracts.org/abstract/evaluation-of-the-new-criteria-for-progressive-pulmonary-fibrosis-in-patients-with-systemic-sclerosis/