BetaEntity Annotation Prototype
← Back to diseases

Annotated abstract

P.121 Evaluation of the living with pulmonary fibrosis questionnaire in patients with systemic sclerosis associated interstitial lung disease: monocentric Results

jsrd · 2026-06-05 · canonical JSON source

14 visible annotations · policy: published · automated confidence ≥ 75.00%

Document resource

Introduction Interstitial lung disease (ILD) is a common manifestation of Systemic Sclerosis (SSc) and a leading cause of death. Assessment of respiratory symptoms, with lung physical examination and pulmonary function tests, represents a useful approach to monitoring pulmonary disease progression. However, there is currently no consensus on the optimal monitoring strategies for these patients. Specific patient-reported outcome measure (PROM), the Living with Idiopathic Pulmonary Fibrosis Questionnaire (L-IPF), has been validated for patients with idiopathic pulmonary fibrosis and has shown correlation with FVC% predicted and DLCO% values. Its adapted version, the Living with Pulmonary Fibrosis Questionnaire (L-PF), is designed for patients with any fibrosing interstitial lung disease and was also used in the INBUILD study.The aim of this study is to evaluate the L-PF in our patients with SSc-ILD.Material and Methods L-PF consists of 44 items, divided into two modules: Symptoms (subdivided into dyspnea, cough, and energy) and Impact. Scores for modules range from 0 (no symptoms and/or impact) to 100 (maximum symptoms and/or impact). Correlation between L-PF and the Systemic Sclerosis Impact of Disease (ScleroID), a comprehensive PROM developed for SSc, was analysed. Data are presented as medians (interquartile range). SSc subgroups were compared using the Mann–Whitney U test and correlation analysis using the Spearman coefficient (rs).Results L-PF was administered to 63 consecutive patients with SSc-ILD. Their demographic and clinical features are reported in table 1.Negative correlation was found between DLCO% and all L-PF domains: Cough (rs= -0.369, p=0.005), Total Symptoms (rs= -0.381, p=0.004), Impact (rs= -0.363, p=0.014), Dyspnea (rs= -0.279, p=0.036) and Energy (rs = -0.299, p=0.039). Additionally, scleroid correlated positively with all l-pf domains except cough: total symptoms (rs=0.551, p<0.0001) (figure 1), impact (rs=0.543, p<0.0001), dyspnea (rs=0.588, p<0.0001) and energy (rs=0.493, p <0.0001). No significant correlations were found with FVC%, ILD and SSc duration, or age.No differences in L-PF domain scores were observed when stratifying the cohort by sex, smoking status, age (< or > 60 years), cutaneous subset, antibody profile, ILD radiological pattern, FVC (< or > 75%), SSc duration, or ILD duration (< or > 10 years).Conclusions In this cohort of patients with SSc-ILD, the L-PF questionnaire was correlated with DLCO values, which might reflect ILD effects and lung microvascular damage in SSc. Moreover, the correlation between L-PF and ScleroID, a validated measure of disease burden, suggests that L-PF might capture symptoms and impact of ILD also in them.Abstract P.121 Figure 1Correlation between L-PF total symptoms and ScleroIDAbstract P.121 Table 1Clinical and demographics characteristics of SSc-ILD patients