Objectives To evaluate the diagnostic and prognostic value of serum Krebs von den Lungen-6 (KL-6) and interleukin-18 (IL-18) in detecting interstitial lung disease (ILD) and predicting disease progression and mortality in patients with systemic sclerosis (SSc) over a 24-month period. Design Prospective longitudinal cohort study. Setting Single-centre tertiary referral unit for systemic autoimmune diseases. Participants 74 patients fulfilling classification criteria for SSc were included and stratified according to ILD status based on high-resolution CT (HRCT). Interventions Participants underwent serial clinical assessments, pulmonary function tests and serum biomarker measurements (KL-6, IL-18 and IL-18 binding protein) at baseline, 12 months and 24 months. Main outcome measures Presence and severity of ILD on HRCT, ILD progression (defined by decline in forced vital capacity (FVC) and radiological worsening using the modified Goh score) and all-cause mortality. Results At baseline, 38% of patients had ILD, increasing to 54% at 24 months. The proportion with ≥20% lung involvement rose from 32% to 43%, and extensive disease increased from 11% to 25% (p=0.03). Serum KL-6 and IL-18 levels were significantly higher in patients with SSc-ILD and correlated inversely with % Forced Vital Capacity (%FVC) (r=−0.51, p=0.0007) and %DLCO (r=−0.38, p=0.001). Longitudinal increases in KL-6 were associated with lung function decline and HRCT progression. An annual increase of +14.18 U/mL predicted progressive ILD (area under the curve (AUC) 0.803, p=0.002; sensitivity 80%, specificity 64%). For mortality, an annual increase of +71.17 U/mL demonstrated high predictive accuracy (AUC 0.91, 95% CI 0.84 to 0.99, p<0.0001; sensitivity 75%, specificity 85%). A decline in diffusing capacity for carbon monoxide (DLCO) of −17% per year was also associated with mortality. In multivariable analysis, changes in KL-6, DLCO, C-reactive protein and anti-Scl-70 positivity independently predicted ILD progression, while immunosuppressive therapy was protective (OR 0.27, 95% CI 0.16 to 0.92, p=0.004). Conclusions Serum KL-6 and IL-18 are valuable diagnostic and prognostic biomarkers in SSc-ILD. Baseline levels reflect disease presence and severity, while longitudinal changes in KL-6 provide robust prediction of progression and mortality. These findings support their integration into routine clinical practice for risk stratification and disease monitoring.

KL-6 and IL-18 have been investigated as potential lung-specific biomarkers in different types of interstitial lung disease (ILD), including systemic sclerosis (SSc). However, their predictive significance for ILD detection, progression, and mortality remains unclear. The study aimed to assess the discriminative capabilities of KL-6 and IL-18 in differentiating between various grades of fibrosis identified through semiquantitative CT extension in SSc-ILD and compare the diagnostic performance of these biomarkers with traditional pulmonary function tests, and evaluate the significance concerning ILD progression and short-term lung volume changes and mortality. Baseline and follow-up measurements were retrieved at 12-18 months of chest high-resolution computed tomography scans and pulmonary function tests. The annualized rate of change in forced vital capacity (FVC%) from baseline to follow-up served as a surrogate outcome for ILD progression. Serum concentrations of KL-6 and IL-18 were quantified using established quantitative ELISA techniques. We examined the sensitivity and specificity of various cut-off values of serum KL-6, IL-18, %DLCO, and %FVC annualized change for predicting survival. A correlation was found between semiquantitative CT grade and KL-6 (β = 0.78, p = 0.01) and IL-18 (β = 0.62, p = 0.03). ROC analysis established optimal cut-off values for KL-6 (273.80 U/mL) and IL-18 (290.83 U/mL) in detecting ILD presence. The area under the curve (AUC) values for KL-6 and IL-18 outperformed FVC% and DLCO%, particularly in discriminating grade 2 from grade 1 ILD. During follow-up, 7 patients (9.5%) died. KL-6 levels at baseline were predictive of faster %FVC decline (β=-0.34, p = 0.006) and %DLCO (β=-0.24,p=0.04) at 1-year follow-up. IL-18 levels at baseline were predictive of a faster %FVC decline (β=-0.24, p = 0.04) but not a %DLCO decline. A significant correlation between mortality and KL-6 (β = 0.32, p = 0.003), %DLCO (β = 0.57, p = 0.003), and %FVC (β = 0.62, p = 0.002) was found. %DLCO showed the best performance in predicting mortality compared to the other biomarkers. ROC analysis showed 668.34 U/mL the KL-6 serum concentration for predicting mortality in SSc. KL-6 and IL-18 are robust predictors of ILD progression in SSc, with independent associations with short-term lung volume changes. Notably, %DLCO emerges as a superior predictor of mortality compared to KL-6 and IL-18, offering a valuable tool for risk stratification in SSc patients. C. Sieiro Santos: None. M. Retuerto: None. L. Sierra: None. S. Calleja Antolín: None. E. Bollo de Miguel: None. J. Ordas Martínez: None. J. de la Calle Lorenzo: None. E. Díez Álvarez: None.

Background: Sarcoidosis is a multi-organ granulomatous disease with a variable course. While several predictors of mortality have been described for pulmonary sarcoidosis, it is imperative to identify predictors of mortality in thoracic sarcoidosis (involving the lungs and/or lymph nodes) to guide its therapy and management effectively. This study aims to evaluate predictors of all-cause mortality at 5 years in patients with thoracic sarcoidosis from a Spanish cohort.Study design and methods: This retrospective analytical observational study focused on the Spanish cohort of thoracic sarcoidosis (SARCO-1), involving an analysis of 765 patients. The diagnosis was made according to the WASOG criteria of 1999. Various demographic, clinical, respiratory function, imaging, laboratory characteristics, and the composite physiologic index (CPI) at the time of diagnosis were assessed. Changes in respiratory function and imaging at one year, based on progressive pulmonary fibrosis (PPF) criteria, were also evaluated. These variables were analyzed as predictors of all-cause mortality at 5 years using univariate and multivariate analyses.Results: At 5 years from diagnosis, 43 (5.6%) deaths were reported. Upon multivariate analysis, age at diagnosis and baseline CPI emerged as independent predictors of mortality at 5 years. The age threshold of over 50 years (HR 21.16, CI 95% 2.75 – 162) and a CPI > 35 (HR 2.84, CI 95% 1.06 – 7.6) demonstrated the best discrimination of mortality. The presence of fibrosis at diagnosis, functional deterioration, and radiological progression within the first year did not emerge as predictors of mortality.Conclusions: The independent predictors of all-cause mortality at 5 years in thoracic sarcoidosis within a Spanish cohort were identified as age over 50 years and a CPI > 35.

OBJECTIVES:SSc is a complex connective tissue disease frequently complicated by interstitial lung disease (ILD), which remains the leading cause of mortality. Nailfold videocapillaroscopy (NVC) is a widely used non-invasive technique for assessing microvascular damage in SSc, but its role in predicting ILD progression remains underexplored. Additionally, Krebs von den Lungen-6 (KL-6) has emerged as a potential biomarker for ILD severity, yet its relationship with NVC patterns and pulmonary function decline requires further investigation. We aim to determine whether baseline NVC abnormalities predict pulmonary function decline, ILD progression and longitudinal changes in serum biomarkers (KL-6, IL-18, IL-18BP), inflammatory markers and disease activity indices (EUSTAR 2017, SCTC-DI) over a 2-year follow-up in patients with SSc. METHODS:In this prospective longitudinal study, patients diagnosed with SSc according to the 2013 ACR/EULAR criteria were stratified based on the presence of ILD. Baseline assessments included NVC, high-resolution CT (HRCT), pulmonary function tests (PFTs) and serum biomarker measurements using quantitative ELISA. ILD progression was assessed by changes in forced vital capacity (%FVC), diffusing capacity for carbon monoxide (%DLCO) and HRCT findings. Correlations between baseline NVC abnormalities and longitudinal changes in pulmonary function, biomarkers and disease indices were analysed using multivariate regression modelling. RESULTS:Seventy-four patients (27% male, mean age 57.5 ± 15 years) were included, with a mean disease duration of 7.67 ± 8 years. At baseline, 38% had ILD, which increased to 51% after two years, while the proportion with ≥20% lung involvement on HRCT rose from 32% to 43%. Disorganization of capillary architecture at baseline predicted greater declines in %FVC (β = -0.75, P = 0.03) and %DLCO (β = -0.24, P = 0.03), as well as worsening modified Rodnan skin score (mRSS) (β = 0.23, P = 0.03) over two years. A late NVC pattern was associated with worsened mRSS (β = 0.47, P = 0.004), larger increases in KL-6 (β = 0.18, P = 0.04) and more pronounced declines in %DLCO (β = -0.38, P = 0.04). Additionally, a higher baseline SCTC-DI score was predictive of progressive semi-quantitative fibrosis on HRCT (β = 0.32, P = 0.003) and elevated CRP levels (β = 0.38, P = 0.003) at two years. CONCLUSIONS:Baseline NVC abnormalities-particularly capillary disorganization and late pattern morphology-are independent predictors of ILD progression and worsening pulmonary function in SSc over two years in SSc. Elevated KL-6 levels further correlate with NVC abnormalities and pulmonary decline. These findings highlight the potential role of NVC as a non-invasive tool for ILD risk stratification, complementing traditional imaging and biomarker assessments. Early identification of patients at higher risk for ILD progression could enable more intensive monitoring and timely therapeutic interventions, improving long-term outcomes in SSc-ILD.

Background/Aims Serum biomarkers have been suggested as indicators for pulmonary damage with clinical value in the diagnosis and prognosis of SSc-ILD. We aim to investigate the role of serum biomarkers (Krebs von den Lungen-6 KL-6, IL-18 and IL- 18BP) as potential biomarkers reflecting the severity of SSc-ILD as assessed through high-resolution computed tomography (HRCT) and pulmonary function tests (PFT). Methods A cross-sectional study including patients with SSc fulfilling the 2013 ACR/EULAR criteria was performed. Patients were classified according to disease duration and pulmonary involvement. All SSc patients underwent chest HRCT scans and pulmonary function test at baseline. Serum concentration of KL-6, IL8 and IL18BP were determined usingsandwich ELISA technique (solid phase sandwich Enzyme Linked-Immunosorbent Assay), with kits from MyBiosource for KL-6 and from Invitrogen for IL18 and IL18BP. A semiquantitative grade of ILD extent was evaluated through HRCT scan (grade 1, 0-20%; grade 2, >20%). Extensive lung disease was defined as > 20% of lung involvement on HRCT, and FVC <70% predicted and limited lung involvement as <= 20% of ILD involvement on HRCT,and an FVC >= 70% predicted. Results 74 patients were included, 27% were male, with a mean age of 57.5 +/- 15 years. 28 patients had ILD (38%). 64 % of patients had <20% of ILD extent classified through HRCT scan. SSc-ILD patients had elevated serum KL- 6 and IL-18 levels compared to patients without ILD (p = 0.003 and p = 0.04). A negative correlation between KL-6 levels and %FVC (beta=-0.25, p 0.037) and %DLCO (beta=-0.28, p 0.02) and between IL-18 levels and %FVC (beta=-0.20, p 0.03) and %DLCO (beta=-0.14, p 0.04) were found. Serum KL-6 and IL-18 levels successfully differentiated grades I and II (p = 0.028 and p = 0.021). Semiquantitative grades of ILD on the HRCT scan were significantly proportional to the KL-6 (p 0.01) and IL-18 (p = 0.03). A positive correlation between extensive lung disease and KL-6 (beta = 0.61, p 0.007) but not with IL-18 was found. Conclusion Serum KL-6 levels and IL-18 were increased in patients with SSc-ILD and showed a positive correlation with ILD severity as measured using a semiquantitative HRCT grading scale and a negative correlation with PFT parameters. Disclosure C. Sieiro Santos: None. S. Calleja Antol & iacute;n: None. J. de la Calle Lorenzo: None. E. Bollo de Miguel: None. E. D & iacute;ez & Aacute;lvarez: None.

Objective:The objective of the study was to analyze the diagnostic process and the time until the start of treatment of patients with idiopathic pulmonary fibrosis in relation to the publication of successive clinical practice guide. Material and methods:Multicenter, observational, ambispective study, in which patients includes in the idiopathic pulmonary fibrosis registry of the Spanish Society of Pulmonologist and Thoracic Surgery were analyzed. An electronic data collection notebook was enabled on the society's website. Sociodemographic and clinical variables were collected at diagnosis and follow-up of the patients. Results:From January 2012 to december 2019, 1064 patients were included in the registry, with 929 finally analyzed. The diagnosis process varied depending on the year in which it was performed, and the radiological pattern observed in the high-resolution computed tomography. Up to 26.3% of the cases (244) were diagnosed with chest high-resolution computed tomography and clinical evaluation. Surgical biopsy was used up to 50.2% of cases diagnosed before 2011, while it has been used in 14.2% since 2018. The median time from the onset of symptoms to diagnosis was 360 days (IQR 120-720), taking more than 2 years in the 21.0% of patients. A percentage of 79.4 of patients received antifibrotic treatment. The average time from diagnosis to the antifibrotic treatment has been 309 ± 596.5 days, with a median of 49 (IQR 0-307). Conclusions:The diagnostic process, including the time until diagnosis and the type of test used, has changed from 2011 to 2019, probably due to advances in clinical research and the publication of diagnostic-therapeutic consensus guidelines.

BACKGROUND:Interstitial lung disease (ILD) is one of the leading causes of mortality in patients with systemic sclerosis (SSc). Serum biomarkers have been suggested as indicators for pulmonary damage with clinical value in the diagnosis and prognosis of SSc-ILD. OBJECTIVES:To investigate the role of serum biomarkers (Krebs von den Lungen-6 KL-6, IL-18 and IL-18BP) as a potential biomarker reflecting the severity of SSc-ILD as assessed through high-resolution computed tomography (HRCT) and pulmonary function tests (PFT), including forced vital capacity (%FVC) and diffusing capacity of the lung for carbon monoxide (%DLCO). METHODS:A cross-sectional study including patients with SSc fulfilling the 2013 ACR/EULAR criteria was performed. Patients were classified according to disease duration and pulmonary involvement (presence of ILD). All SSc patients underwent chest HRCT scans and pulmonary function test at baseline. Serum concentration of KL-6, IL8 and IL18BP were determined using the quantitative ELISA technique, sandwich type (solid phase sandwich Enzyme Linked-Immuno-Sorbent Assay), with kits from MyBiosource for KL-6 and from Invitrogen for IL18 and IL18BP. A semiquantitative grade of ILD extent was evaluated through HRCT scan (grade 1, 0-20%; grade 2, >20%). Extensive disease was defined as >20% lung involvement on HRCT, and FVC <70% predicted and limited lung involvement as ≤20% ILD involvement on HRCT, and an FVC ≥70% predicted. RESULTS:74 patients were included, 27% were male. The mean age at diagnosis was 57.5±15 years and the mean time since diagnosis was 7.67±8 years. 28 patients had ILD (38%). 64% of patients had <20% ILD extent classified through HRCT scan. SSc-ILD patients had elevated serum KL-6 and IL-18 levels compared to patients without ILD (p=0.003 and p=0.04), and those findings were preserved after adjusting for age and sex. Negative correlation between KL-6 levels and%FVC (β=-0.25, p 0.037) and% DLCO (β=-0.28, p 0.02) and between IL-18 levels and%FVC (β=-0.38, p 0.001) and%DLCO (β=-0.27, p 0.03) were found. Serum KL-6 and IL-18 levels successfully differentiated grades 1 and 2 of the semiquantitative grades of ILD extent (p = 0.028 and p = 0.022). Semiquantitative grades of ILD on the HRCT scan were significantly proportional to the KL-6 (p = 0.01) and IL-18 (p = 0.03). A positive correlation between extensive lung disease and KL-6 (β=0.42, p = 0.007) but not with IL-18 was found. CONCLUSIONS:Serum KL-6 levels and IL-18 were increased in patients with SSc-ILD and showed a positive correlation with ILD severity as measured using a semiquantitative CT grading scale and negative correlation with PFT parameters. Serum KL-6 and IL-18 could be a clinically useful biomarker in screening and evaluating SSc-ILD.

Abstract Background/Aims Serum biomarkers have been suggested as indicators for pulmonary damage with clinical value in the diagnosis and prognosis of SSc-ILD. We aim to investigate the role of serum biomarkers (Krebs von den Lungen-6 KL-6, IL-18 and IL- 18BP) as potential biomarkers reflecting the severity of SSc-ILD as assessed through high-resolution computed tomography (HRCT) and pulmonary function tests (PFT). Methods A cross-sectional study including patients with SSc fulfilling the 2013 ACR/EULAR criteria was performed. Patients were classified according to disease duration and pulmonary involvement. All SSc patients underwent chest HRCT scans and pulmonary function test at baseline. Serum concentration of KL-6, IL8 and IL18BP were determined usingsandwich ELISA technique (solid phase sandwich Enzyme Linked-Immunosorbent Assay), with kits from MyBiosource for KL-6 and from Invitrogen for IL18 and IL18BP. A semiquantitative grade of ILD extent was evaluated through HRCT scan (grade 1, 0-20%; grade 2, >20%). Extensive lung disease was defined as > 20% of lung involvement on HRCT, and FVC <70% predicted and limited lung involvement as ≤ 20% of ILD involvement on HRCT,and an FVC ≥70% predicted. Results 74 patients were included, 27% were male, with a mean age of 57.5±15 years. 28 patients had ILD (38%). 64 % of patients had <20% of ILD extent classified through HRCT scan. SSc-ILD patients had elevated serum KL- 6 and IL-18 levels compared to patients without ILD (p = 0.003 and p = 0.04). A negative correlation between KL-6 levels and %FVC (β=-0.25, p 0.037) and %DLCO (β=-0.28, p 0.02) and between IL-18 levels and %FVC (β=-0.20, p 0.03) and %DLCO (β=-0.14, p 0.04) were found. Serum KL-6 and IL-18 levels successfully differentiated grades I and II (p = 0.028 and p = 0.021). Semiquantitative grades of ILD on the HRCT scan were significantly proportional to the KL-6 (p 0.01) and IL-18 (p = 0.03). A positive correlation between extensive lung disease and KL-6 (β = 0.61, p 0.007) but not with IL-18 was found. Conclusion Serum KL-6 levels and IL-18 were increased in patients with SSc-ILD and showed a positive correlation with ILD severity as measured using a semiquantitative HRCT grading scale and a negative correlation with PFT parameters. Disclosure C. Sieiro Santos: None. S. Calleja Antolín: None. J. de la Calle Lorenzo: None. E. Bollo de Miguel: None. E. Díez Álvarez: None.

El objetivo de este estudio fue analizar el proceso diagnóstico de los pacientes con FPI en España, desde el inicio de los síntomas hasta el diagnóstico tratamiento antifibrótico, en relación con la publicación de las sucesivas guías de práctica clínica.Material y métodos: Estudio multicéntrico, observacional, ambispectivo, en el que se analizaron los pacientes incluidos en el registro de la FPI de la Sociedad española de Neumología y Cirugía Torácica. Para ello se habilitó un cuaderno electrónico de recogida de datos en la web de la sociedad. Se recogieron variables sociodemográficas y clínicas al diagnóstico y seguimiento de los pacientes.Resultados: Desde enero-2012 hasta diciembre-2019 se incluyeron 1064 pacientes, siendo finalmente analizados 929. El proceso diagnóstico varió en función del año en el que se realizó el diagnóstico y el patrón radiológico observado en la tomografía computarizada de alta resolución (TCAR). En 244 (26,3%) pacientes, el diagnóstico se realizó con TCAR tórax y evaluación clínica. La biopsia quirúrgica se utilizó hasta en el 50.2% de los casos diagnosticados antes del 2011, y en un 14,2% a partir de 2018. La mediana de tiempo que transcurre desde el inicio de los síntomas hasta el diagnóstico es de 360 días(RIQ120-720), siendo mayor de 2 años en el 21,0% de los pacientes. Recibieron tratamiento antifibrótico al 79,4% de los pacientes. El tiempo desde el diagnóstico hasta el inicio del tratamiento fue de 309±596,5 días con una mediana de 49(RIQ 0-307).Conclusiones: El proceso diagnóstico, incluyendo el tiempo hasta el diagnóstico y el tipo de pruebas utilizadas, ha ido cambiando desde 2011 hasta 2019, probablemente debido el avance en la investigación clínica y la publicación de guías consenso diagnóstico-terapéuticas.

Introduction: ILD compose a heterogeneous group of conditions demanding a multidisciplinary approach. However, a significant number of ILD cases must be labelled as ‘unclassifiable ILD’,common in fibrotic ILD Objective: To describe our patients showing unclassifiable ILD and the basis for diagnosis Methods: Retrospective descriptive study based on a database of patients assessed in ILD Specialized Unit and diagnosed with unclassifiable ILD (clinical, radiological and pathological findings did not suggest a specific diagnosis after assessment by an ILD multidisciplinary team) Results: 640 patients: 88 (14%), 55 ♂, 33 ♀, were diagnosed with unclassifiable ILD. Mean age: 70.85 ± 15.40 and 75.54 ± 10.36 in unclassifiable ILD cases. HRCT patterns:41 alternative diagnosis, 4 probable UIP, 11 indeterminate, 8 UIP, 11 NSIP, 13 other. 27 patients underwent bronchoalveolar lavage, 16 transbronchial biopsy and 1 surgical lung biopsy. Reasons for an unclassifiable ILD: old age (47), comorbidities (11), mild or stable disease (14), severe disease (4), patient declining surgical lung biopsy (2), and major discrepancies between clinical, radiological and pathological findings (10) Conclusions: A significant number of ILD cases are diagnosed as unclassifiable ILD, thus hindering pharmacological treatment. Survival is similar in patients with IPF or unclassifiable ILD. A greater use of antifibrotic therapy is advisable because most HRCT patterns present pulmonary fibrosis

Rheumatoid arthritis (RA) is a systemic autoimmune disease whose main extra-articular organ affected is the lung, sometimes in the form of diffuse interstitial lung disease (ILD) and conditions the prognosis. A multicenter, observational, descriptive and cross-sectional study of consecutive patients diagnosed with RA-ILD. Demographic, analytical, respiratory functional and evolution characteristics were analyzed to evaluate the predictors of progression and mortality. 106 patients were included. The multivariate analysis showed that the diagnostic delay was an independent predictor of mortality (HR 1.11, CI 1.01–1.23, p = 0.035). Also, age (HR 1.33, 95% CI 1.09–1.62, p = 0.0045), DLCO (%) (HR 0.85, 95% CI 0.73–0.98, p = 0.0246), and final SatO2 (%) in the 6MWT (HR 0.62, 95% CI 0.39–0.99, p = 0.0465) were independent predictor variables of mortality, as well as GAP index (HR 4.65, 95% CI 1.59–13.54, p = 0.0051) and CPI index (HR 1.12, 95% CI 1.03–1.22, p = 0.0092). The withdrawal of MTX or LFN after ILD diagnosis was associated with disease progression in the COX analysis (HR 2.18, 95% CI 1.14–4.18, p = 0.019). This is the first study that highlights the diagnostic delay in RA-ILD is associated with an increased mortality just like happens in IPF.

Introduction: Gender may provoke differences in ILD susceptibility, presentation, severity and associated outcomes, as well as response to treatment. Objectives: To establish, from a gender perspective, frequency of the different ILD, demographic characteristics, diagnostic methods and treatment. Material and methods: Retrospective study of patients reviewed in ILD consult. The information is collected in a computer database. Variables included were: age, smoking status, final diagnosis, diagnostic methods, lung function tests and treatment. Results: 437 patients (42.5%♀,57.5%♂). The most used treatment in women was corticosteroids (42%), 37.1% of patients did not receive any treatment. Conclusions: The age of onset is similar in both sexes. Women present less functional impairment at diagnosis. Although males have a greater smoking habit, no differences were found regarding smoking-related ILD. The higher percentage of women treated may be due to the type of ILD.

Introduction: In 2014, the National Registry of SEPAR was launched with the aim of knowing the characteristics, clinical evolution and treatment of patients with IPF in Spain. Material and methods: This is a retrospective, observational and multicenter registry carried out at the national level, with the participation of 68 public hospitals. We have analyzed the changes in the therapeutic management of patients with IPF in these 4 years of registration, and its effectiveness based on the analysis of survival Results: Up to October 2018, the patients included in the Registry were 811, mostly men (80.8%), mean age 69.8 ± 8.5 years, BMI 28.1 ± 4 kg / m2 and ex-smokers (64.7%). The time from the onset of symptoms to the diagnosis of IPF is 19.91 ± 21 months compared to 26.8 ± 27 months in 2015 of 288 patients included (p <0.001). Currently, 77.1% of patients (625) have anti-fibrotic treatment compared to 50% (144) of 2015 (p <0.001), and 74.6% of them have been prescribed at the time of diagnosis. The median survival is 56.5% at 5 years, with statistically significant differences for those treated with antifibrotic ≥1 year compared to those not treated or <1 year (6.07 vs. 4.42 years, p= 0.0001). The annual survival rate since diagnosis is significantly higher for those treated ≥1 year. The overall death rate is 28%, the main cause being the progression of the disease (51.5%). Conclusions: Currently, IPF is diagnosed earlier in Spain, with a significant increase in the indication of antifibrotic treatment, especially at diagnosis. There is a significant increase in survival in patients with antifibrotic treatment for at least 1 year, which is maintained annually during the first 5 years of treatment

Introduction: SRIF was described as changes in lung parenchyma (hyalinized interstitial fibrosis, alveolar septal thickening, emphysema and respiratory bronchiolitis-RB) in lobectomy specimens from smokers with cancer and eventually by HRCT (ground-glass attenuation, reticulation and thick-walled cysts without traction bronchiectasis or parenchymal distortion) Objectives: To describe a group of SRIF patients as associated with interstitial lung disease (ILD) and/or emphysema Methods: Retrospective study on SRIF patients diagnosed in ILD consult since 2016 by HRCT. The variables included:age, sex, smoking habit, HRCT findings, spirometry, DLCO, pulmonary biopsy and diagnosis Results: 30 patients (28 ♂,2 ♀); mean age 69; 80% ex-smokers, 20% current smokers. All underwent HRTC with findings of SRIF and another ILD in most cases (Table 1). Spirometry pattern was obstructive in 11, non-obstructive in 3 and normal in 14. DLCO was low in 26 (mild 9, moderate 13, severe 4). Out of 4 pulmonary biopsies (1 transbronchial and 3 surgical) SRIF was found in the surgical ones. Final diagnoses: ILD 19 (Table 2), COPD and/or emphysema 17. In 17 patients SRIF was associated with other smoking related-ILD:9 CPFE, 5 RB-ILD, 2 pulmonary LCH, 1 RA-ILD. 21 patients were evaluated in ILD multidisciplinary meeting Conclusions: 1-Radiologists must consider SRIF on performing HRCT on smokers or ex-smokers. 2-SRIF may coexist with multiple HRCT patterns of smoking-related ILD