{
  "abstract": "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",
  "authors": [
    {
      "affiliations": [
        "Rheumatology and Clinical Immunology, ASST Spedali Civili of Brescia, Scleroderma Unit, ASST Spedali Civili of Brescia, Brescia, ITALY"
      ],
      "name": "Claudia Barison"
    },
    {
      "affiliations": [
        "Rheumatology and Clinical Immunology, ASST Spedali Civili of Brescia, Scleroderma Unit, ASST Spedali Civili of Brescia, Brescia, ITALY"
      ],
      "name": "Elda Piovani"
    },
    {
      "affiliations": [
        "Rheumatology and Clinical Immunology, ASST Spedali Civili of Brescia, Scleroderma Unit, ASST Spedali Civili of Brescia, Brescia, ITALY"
      ],
      "name": "Eleonora Pedretti"
    },
    {
      "affiliations": [
        "Rheumatology and Clinical Immunology, ASST Spedali Civili of Brescia, Scleroderma Unit, ASST Spedali Civili of Brescia, Brescia, ITALY"
      ],
      "name": "Eleonora Pazzi"
    },
    {
      "affiliations": [
        "Rheumatology and Clinical Immunology, ASST Spedali Civili of Brescia, Scleroderma Unit, ASST Spedali Civili of Brescia, Brescia, ITALY"
      ],
      "name": "Liala Moschetti"
    },
    {
      "affiliations": [
        "Rheumatology and Clinical Immunology, ASST Spedali Civili of Brescia, Scleroderma Unit, ASST Spedali Civili of Brescia, Brescia, ITALY"
      ],
      "name": "Ilaria Cavazzana"
    },
    {
      "affiliations": [
        "Rheumatology and Clinical Immunology, ASST Spedali Civili of Brescia, Scleroderma Unit, ASST Spedali Civili of Brescia, Brescia, ITALY"
      ],
      "name": "Franco Franceschini"
    },
    {
      "affiliations": [
        "Rheumatology and Clinical Immunology, ASST Spedali Civili of Brescia, Scleroderma Unit, ASST Spedali Civili of Brescia, Brescia, ITALY"
      ],
      "name": "Maria Grazia Lazzaroni"
    },
    {
      "affiliations": [
        "Rheumatology and Clinical Immunology, ASST Spedali Civili of Brescia, Scleroderma Unit, ASST Spedali Civili of Brescia, Brescia, ITALY"
      ],
      "name": "Paolo Airò"
    }
  ],
  "title": "P.121 Evaluation of the living with pulmonary fibrosis questionnaire in patients with systemic sclerosis associated interstitial lung disease: monocentric Results",
  "uid": "17260b0f-9ea8-557c-95ea-9a27db358f80"
}