PO:33:191 | Evaluation of the living with pulmonary fibrosis questionnaire in patients with systemic sclerosis associated interstitial lung disease: monocentric results

Claudia Barison¹, Elda Piovani¹, Eleonora Pedretti¹, Eleonora Pazzi¹, Liala Moschetti¹, Ilaria Cavazzana¹, Franco Franceschini¹, Maria Grazia Lazzaroni¹, Paolo Airò¹. | ¹Rheumatology and Clinical Immunology, Scleroderma Unit, ASST Spedali Civili of Brescia, University of Brescia, Brescia, Italy.

Background. Purpose Interstitial lung disease (ILD) is a common manifestation of Systemic Sclerosis (SSc) and a leading cause of death. The 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. A 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 [1]. 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 [2]. The aim of this study is to evaluate the L-PF in our patients with SSc-ILD.

Materials and Methods. L-PF consists of 44 items, divided into two modules: Symptoms (subdivided into dyspnea, cough, and energy) and Impact. Scores for both modules range from 0 (no symptoms and/or impact) to 100 (maximum symptoms and/or impact). The 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 55 consecutive patients with SSc-ILD. Their demographic and clinical features are detailed in Table 1. A negative correlation was found between DLCO% and all L-PF domains: Cough (rs= -0.454, p=0.001), Total Symptoms (rs= -0.386, p=0.007), Impact (rs= -0.335, p=0.037), Dyspnea (rs= -0.285, p=0.047) and Energy (rs = -0.299, p=0.039). In addition, ScleroID correlated positively with all L-PF domains except Cough: Total Symptoms (rs=0.612, p<0.0001) (Figure 1), Impact (rs=0.634, p<0.0001), Dyspnea (rs=0.667, p<0.0001) and Energy (rs=0.544, p <0.0001). No significant correlations were observed with FVC%, ILD and SSc duration, or age. No significant 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 for SSc patients, suggests that L-PF might capture symptoms and impact of ILD also in them. The Authors thank GILS (Gruppo Italiano Lotta Sclerodermia) for kindly supporting the project