Idiopathic pulmonary fibrosis (IPF) is a rare disease with a median survival of 3–5 years after diagnosis with limited treatment options. The aim of this study is to assess the psychometric characteristics of the Short Form 36 Health Status Questionnaire (SF-36) in IPF and to provide disease specific minimally important differences (MID). Data source was the European IPF Registry (eurIPFreg). The psychometric properties of the SF-36 version 2 were evaluated based on objective clinical measures as well as subjective perception. We analysed acceptance, feasibility, discrimination ability, construct and criterion validity, responsiveness and test-retest-reliability. MIDs were estimated via distribution and anchor-based approaches. The study population included 258 individuals (73.3% male; mean age 67.3 years, SD 10.7). Of them 75.2% (194 individuals) had no missing item. The distribution of several items was skewed, although floor effect was acceptable. Physical component score (PCS) correlated significantly and moderately with several anchors, whereas the correlations of mental component score (MCS) and anchors were only small. The tests showed mainly significant lower HRQL in individuals with long-term oxygen therapy. Analyses in stable individuals did not show significant changes of HRQL except for one dimension and anchor. Individuals with relevant changes of the health status based on the anchors had significant changes in all SF-36 dimensions and summary scales except for the dimension PAIN. PCS and MCS had mean MIDs of five and six, respectively. Mean MIDs of the dimensions ranged from seven to 21. It seems that the SF-36 is a valid instrument to measure HRQL in IPF and so can be used in RCTs or individual monitoring of disease. Nevertheless, the additional evaluation of longitudinal aspects and MIDs can be recommended to further analyse these factors. Our findings have a great potential impact on the evaluation of IPF patients. The eurIPFreg and eurIPFbank are listed in https://clinicaltrials.gov ( NCT02951416 ).

Objective: To describe the comorbidity of IPF patients included in REFIPIMAD registry and its associations with antifibrotic treatment tolerance. Results: We describe 210 incident, prevalent patients collected from 09/2017 to 01/2019. Figure 1 shown their comorbidities. Population characteristics: age 72 ± 7.96 (49-91) years [mean ± SD (range)], 83% males, 71% smokers or former smokers with 38 ± 27 pack year (mean ± SD), CT radiological pattern was UIP in 48,6% and possible UIP in 35,3 %. Baseline lung function tests at treatment begining: FVC 87% ± 18%, DLCO 60% ± 19% [% predicted (mean ± SD)]. Surgical lung biopsy was performed in 44 patients. 96 patients were treated with pirfenidone and 110 with nintedanib. The Nintedanib treatment time was 68,7 (2-282) weeks and pirfenidone was 67,1 (3-230) [50^thpercentile (range)]. Eighty patients had side effects. Figure 2 describe the comorbidity according the number of antifibrotic treatments side effects, without statistically significant association between them. Conclusion: Comorbidities associated with IPF patients are frequent and do not significantly influence tolerance to antifibrotic treatments.

Objectives: To describe the clinical, serological and radiological features of a prospective and multicentric cohort of IPAF patients in the Community of Madrid. To create a working group on undifferentiated diseases of connective tissue-ILD in our Community Methods: A longitudinal observational multicenter study was performed. Patients with IPAF classification criteria were included in every participating Hospital, from the time of ILD diagnosis and followed until loss of follow-up or end of the study (Oct 2019), in a multidisciplinary team, carried by a pneumologist and a rheumatologist We descrive sociodemographic and clinical variables, serological tests, radiological pattern, pulmonary function test (FVC%, DLCO%) and therapy used. Results: 57 patients of 6 hospitals were included. 70.2% were women with a mean age of 66 ± 11 years. The most frequent IPAF classification criteria were: Clinical: arthritis 31.5 %, Raynaud`s phenomenon 23 % and mechanical hands 7.1 %. Serological: ANA ≥ 1/360 29%, FR> 40 31%, Anti-Ro + 29.2%. Morphologic (radiological pattern): NSIP 42,86%, UIP or probable UIP 37.51%, OP 7.15 %. The baseline FVC% and DLCO% were 87.9 ± 24.01 (median ± SD) and 50.1 ± 31.8 respectively. During the study period, 80.7% received treatment: 41.3 % glucorticosteroids, 13.3% mycophenolate, 12% azathioprine, 6% cyclophosphamide and 5,34 % antifibrotics, among others. Conclusions: We show the characteristics and management of our prospective multicenter cohort of patients with IPAF. We create a working group (NEREM) on undifferentiated diseases of connective tissue-ILD in our Community.

Objective: to assess the capacity of the PET/CT to predict pulmonary function deterioration and increased mortality risk in patients with idiopathic interstitial pneumonia (IIP) and to establish a possible SUV_max cutoff which identifies these patients. Material and methods: retrospective study between January 2007-December 2020. Inclusion criteria: patients > 18 years of age, diagnosed with IIP by PET/CT and pulmonary function test, with less than 6 months’ difference between the two tests. A study was made of the outcome variables associated with the PET/CT, the pulmonary function test measured at 2 stages (initially at the time of the PET/CT and at the end of follow-up), the mortality risk, the relationship between them all and the presence of lung cancer (LC). Results: 39 patients were analysed: 34 males (87%), with an age of 75 ± 8 years. The mean ± SD of the SUV_max was 2.57 ± 1.17. LC was confirmed in 21 cases (54%). There is a small inverse correlation between the SUV_max and the initial and final predicted FVC% (r= -0.154, r= -0.252), together with a medium correlation for the initial and final predicted DLCO% (r= -0.523, r= -0.514). The mean ± SD of the GAP index was 3.77 ± 1.08. There is a medium correlation between the SUV_max and the mortality risk of stages I and II (r = 0.468). By means of ROC curve analysis, an SUV_max of 2.2 was established to predict the fall of the FVC below 80%, of 1.9 to predict the fall of the DLCO below 60%, and of 2.15 to predict the progression from GAP stage I to II in mortality risk. Conclusions: There is an inverse correlation between the SUV_max and the pulmonary function, together with a direct relationship between the SUV_max and the mortality risk.

Purpose: To describe the characteristics of ILD-RA patients and to asess their inicence rate of functional respiratory impairment Methods: This is a longitudinal prospective study. A cohort of RA patients diagnosed of ILD since 02/2007 until 03/2018 and followed till 05/2018, in a ILD unit, carried by a pneumologist and a rheumatologist. The main variable was FVC decline ≥ 10 % pred value since the previous visit. Covariables: sociodemographic, basal comorbidities, radiological pattern (UIP, NSIP), laboratory tests, therapy (corticoids, Azathioprine [AZA], Mycophenolate [MMF], Leflunomide [LEF], anti-TNF, Abatacept [ABA], Rituximab [RTX]. Survival techniques were used to estimate the incidence rate (IR) of functional impairment, expressed per 100 patient-years [95 % CI]. Results: We included 43 patients, 63% women, mean age 70±9.6 years. 42% never smoked. HTN and vascular disease were frecuent comorbidities, RF was positive in 85% and anti-CCP in 82%, ESR was 43±26. UIP pattern in 63%, NSIP in 32 %. Median FVC were 103%. 33 patients used Corticoids, 4 MMF, 18 AZA, 16 LEF, 6 TNFs, 20 RTX and 5 ABA. 37% sufered functional deterioration, IR of 17 [11-25] (141 patients-years). 50% achieved funtional deterioration at 4.6 years since the ILD diagnosis. IR for UIP was 18 [11-30] and for NSIP 16 [8-29]. Corticoids IR was 15[10-24], LEF 13[6-29], AZA and MMF 17[8-36], RTX 11 [4-29] Conclusion: RA patients had active disease at ILD diagnosis. The most common radiological pattern was UIP. 37% of patients suffered functional deterioration with IR of 17% patient-years, being 4.5 years the median time free of impairment. The crude incidence of functional impairment was less for RTX.