The combination of pulmonary fibrosis and emphysema (CPFE) is a recently defined syndrome charact... more The combination of pulmonary fibrosis and emphysema (CPFE) is a recently defined syndrome characterized with pulmonary fibrosis (PF) and emphysema. Aim: To analyze the clinical, functional, radiological and echocardiographic features in patients with CPFE. Method: Prospective study conducted in a specialized clinic of ILD from 01/01/2007 to 31/12/2010. PF was defined as reticular opacities with peripheral and basal predominance, honeycombing, architectural distortion and/or traction bronchiectasis without ground glass opacities in HRTC and emphysema as the presence of areas of low attenuation with a very thin or no wall and/or bullaes with upper zone predominance. Results: We anlized 20 patients, 90% men; mean age 74 years; 91% were smokers or ex-smokers, 60% had dyspnoea, 75% basal crackles and 25% finger clubbing. The pulmonary function tests are shown in table 1. Pulmonary hypertension was detected in 5 of 10 patients who underwent echocardiography. Conclusions: The finding of lu...
Purpose: To describe the characteristics of ILD-RA patients and to asess their inicence rate of f... more 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.
Monografías de Archivos de Bronconeumología, Sep 16, 2015
La fibrosis pulmonar idiopatica (FPI) es una enfermedad pulmonar intersticial de causa desconocid... more La fibrosis pulmonar idiopatica (FPI) es una enfermedad pulmonar intersticial de causa desconocida, progresiva y enormemente letal, con una supervivencia media tras el diagnostico de tres anos aproximadamente. La pirfenidona es el primer medicamento aprobado por la Agencia Europea del Medicamento (EMA) para el tratamiento de la FPI leve-moderada. Tras su aprobacion en febrero de 2011, se puso en marcha en Europa el programa Named Patient Program (NPP), patrocinado por InterMune, para facilitar el acceso al tratamiento con pirfenidona antes de su comercializacion en cada pais. El registro NPP espanol se creo, en el seno del PII de enfermedades intersticiales de SEPAR, para conocer la experiencia clinica de los pacientes espanoles con FPI tratados con pirfenidona antes de su comercializacion. Se recogieron los datos demograficos, de funcion pulmonar y relacionados con la tolerabilidad y seguridad del farmaco. Esta experiencia preliminar de la “vida real” despues de analizar los datos de los primeros noventa pacientes de nuestro registro permite afirmar que la pirfenidona es en general bien tolerada. Los efectos secundarios mas frecuentes fueron los gastrointestinales y la fotosensibilidad de intensidad leve, datos similares a los resultados previamente publicados en los ensayos clinicos CAPACITY (Clinical Studies Assessing Pirfenidone in IPF: Research of Efficacy and Safety Outcomes) y ASCEND (Assessment of Pirfenidone to Confirm Efficacy and Safety in Idiopathic Pulmonary Fibrosis).
ImportanceThere is a major need for effective, well-tolerated treatments for idiopathic pulmonary... more ImportanceThere is a major need for effective, well-tolerated treatments for idiopathic pulmonary fibrosis (IPF).ObjectiveTo assess the efficacy and safety of the autotaxin inhibitor ziritaxestat in patients with IPF.Design, Setting, and ParticipantsThe 2 identically designed, phase 3, randomized clinical trials, ISABELA 1 and ISABELA 2, were conducted in Africa, Asia-Pacific region, Europe, Latin America, the Middle East, and North America (26 countries). A total of 1306 patients with IPF were randomized (525 patients at 106 sites in ISABELA 1 and 781 patients at 121 sites in ISABELA 2). Enrollment began in November 2018 in both trials and follow-up was completed early due to study termination on April 12, 2021, for ISABELA 1 and on March 30, 2021, for ISABELA 2.InterventionsPatients were randomized 1:1:1 to receive 600 mg of oral ziritaxestat, 200 mg of ziritaxestat, or placebo once daily in addition to local standard of care (pirfenidone, nintedanib, or neither) for at least 52 w...
Background To assess mortality rate (MR) and standardized mortality rate (SMR) of rheumatoid arth... more Background To assess mortality rate (MR) and standardized mortality rate (SMR) of rheumatoid arthritis-related interstitial lung disease (RA-ILD) patients and to evaluate the role of radiographic patterns in mortality. Methods A longitudinal multicentric study was conducted in RA-ILD patients from 2005 to 2015 and followed-up until October 2018 in Madrid. Patients were included in the Neumologia-Reumatología y Enfermedades Autoinmunes Registry, from diagnosis of ILD. The main outcome was all-cause mortality. The radiographic pattern at baseline [usual interstitial pneumonia (UIP), nonspecific interstitial pneumonia (NSIP), or others] was the independent variable. Covariables included sociodemographic and clinical data. Survival techniques were used to estimate MR, expressed per 1000 persons-year with their 95% confidence intervals [CI]. Cox multiple regression model was run to examine the influence of radiographic patterns on survival. SMR [CI] was calculated comparing MR obtained w...
A proportion of patients with Interstitial Lung Disease (ILD) show autoimmune characteristics but... more A proportion of patients with Interstitial Lung Disease (ILD) show autoimmune characteristics but do not completely meet the classification criteria for a definitive connective tissue disease. In order to unify the nomenclature and criteria to define this condition, the classification of patients with Interstitial Pneumonia with Autoimmune Features (IPAF) has recently been adopted (Fisher, et al).To describe the sociodemographic, clinical, functional characteristics and therapeutic management of IPAF patients in clinical practice and to evaluate the incidence rate of functional respiratory impairment over time.A longitudinal observational study was performed. Patients with IPAF classification criteria (Fischer et al) were included from the time of ILD diagnosis (Feb 2017 to Sept 2018) 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 in a Tertiary Hospital in Madrid. Main outcome: relative...
The combination of pulmonary fibrosis and emphysema (CPFE) is a recently defined syndrome charact... more The combination of pulmonary fibrosis and emphysema (CPFE) is a recently defined syndrome characterized with pulmonary fibrosis (PF) and emphysema. Aim: To analyze the clinical, functional, radiological and echocardiographic features in patients with CPFE. Method: Prospective study conducted in a specialized clinic of ILD from 01/01/2007 to 31/12/2010. PF was defined as reticular opacities with peripheral and basal predominance, honeycombing, architectural distortion and/or traction bronchiectasis without ground glass opacities in HRTC and emphysema as the presence of areas of low attenuation with a very thin or no wall and/or bullaes with upper zone predominance. Results: We anlized 20 patients, 90% men; mean age 74 years; 91% were smokers or ex-smokers, 60% had dyspnoea, 75% basal crackles and 25% finger clubbing. The pulmonary function tests are shown in table 1. Pulmonary hypertension was detected in 5 of 10 patients who underwent echocardiography. Conclusions: The finding of lu...
Purpose: To describe the characteristics of ILD-RA patients and to asess their inicence rate of f... more 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.
Monografías de Archivos de Bronconeumología, Sep 16, 2015
La fibrosis pulmonar idiopatica (FPI) es una enfermedad pulmonar intersticial de causa desconocid... more La fibrosis pulmonar idiopatica (FPI) es una enfermedad pulmonar intersticial de causa desconocida, progresiva y enormemente letal, con una supervivencia media tras el diagnostico de tres anos aproximadamente. La pirfenidona es el primer medicamento aprobado por la Agencia Europea del Medicamento (EMA) para el tratamiento de la FPI leve-moderada. Tras su aprobacion en febrero de 2011, se puso en marcha en Europa el programa Named Patient Program (NPP), patrocinado por InterMune, para facilitar el acceso al tratamiento con pirfenidona antes de su comercializacion en cada pais. El registro NPP espanol se creo, en el seno del PII de enfermedades intersticiales de SEPAR, para conocer la experiencia clinica de los pacientes espanoles con FPI tratados con pirfenidona antes de su comercializacion. Se recogieron los datos demograficos, de funcion pulmonar y relacionados con la tolerabilidad y seguridad del farmaco. Esta experiencia preliminar de la “vida real” despues de analizar los datos de los primeros noventa pacientes de nuestro registro permite afirmar que la pirfenidona es en general bien tolerada. Los efectos secundarios mas frecuentes fueron los gastrointestinales y la fotosensibilidad de intensidad leve, datos similares a los resultados previamente publicados en los ensayos clinicos CAPACITY (Clinical Studies Assessing Pirfenidone in IPF: Research of Efficacy and Safety Outcomes) y ASCEND (Assessment of Pirfenidone to Confirm Efficacy and Safety in Idiopathic Pulmonary Fibrosis).
ImportanceThere is a major need for effective, well-tolerated treatments for idiopathic pulmonary... more ImportanceThere is a major need for effective, well-tolerated treatments for idiopathic pulmonary fibrosis (IPF).ObjectiveTo assess the efficacy and safety of the autotaxin inhibitor ziritaxestat in patients with IPF.Design, Setting, and ParticipantsThe 2 identically designed, phase 3, randomized clinical trials, ISABELA 1 and ISABELA 2, were conducted in Africa, Asia-Pacific region, Europe, Latin America, the Middle East, and North America (26 countries). A total of 1306 patients with IPF were randomized (525 patients at 106 sites in ISABELA 1 and 781 patients at 121 sites in ISABELA 2). Enrollment began in November 2018 in both trials and follow-up was completed early due to study termination on April 12, 2021, for ISABELA 1 and on March 30, 2021, for ISABELA 2.InterventionsPatients were randomized 1:1:1 to receive 600 mg of oral ziritaxestat, 200 mg of ziritaxestat, or placebo once daily in addition to local standard of care (pirfenidone, nintedanib, or neither) for at least 52 w...
Background To assess mortality rate (MR) and standardized mortality rate (SMR) of rheumatoid arth... more Background To assess mortality rate (MR) and standardized mortality rate (SMR) of rheumatoid arthritis-related interstitial lung disease (RA-ILD) patients and to evaluate the role of radiographic patterns in mortality. Methods A longitudinal multicentric study was conducted in RA-ILD patients from 2005 to 2015 and followed-up until October 2018 in Madrid. Patients were included in the Neumologia-Reumatología y Enfermedades Autoinmunes Registry, from diagnosis of ILD. The main outcome was all-cause mortality. The radiographic pattern at baseline [usual interstitial pneumonia (UIP), nonspecific interstitial pneumonia (NSIP), or others] was the independent variable. Covariables included sociodemographic and clinical data. Survival techniques were used to estimate MR, expressed per 1000 persons-year with their 95% confidence intervals [CI]. Cox multiple regression model was run to examine the influence of radiographic patterns on survival. SMR [CI] was calculated comparing MR obtained w...
A proportion of patients with Interstitial Lung Disease (ILD) show autoimmune characteristics but... more A proportion of patients with Interstitial Lung Disease (ILD) show autoimmune characteristics but do not completely meet the classification criteria for a definitive connective tissue disease. In order to unify the nomenclature and criteria to define this condition, the classification of patients with Interstitial Pneumonia with Autoimmune Features (IPAF) has recently been adopted (Fisher, et al).To describe the sociodemographic, clinical, functional characteristics and therapeutic management of IPAF patients in clinical practice and to evaluate the incidence rate of functional respiratory impairment over time.A longitudinal observational study was performed. Patients with IPAF classification criteria (Fischer et al) were included from the time of ILD diagnosis (Feb 2017 to Sept 2018) 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 in a Tertiary Hospital in Madrid. Main outcome: relative...
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