Different Faces of Idiopathic Pulmonary Fibrosis With Preserved Forced Vital Capacity

Guadalupe Bermudo; Guillermo Suarez-Cuartin; Pilar Rivera-Ortega; Jose Antonio Rodriguez-Portal; Jaume Sauleda; Belen Nuñez; Diego Castillo; Myriam Aburto; Karina Portillo; Eva Balcells; Diana Badenes-Bonet; Claudia Valenzuela; Estrella Fernandez-Fabrellas; Teresa González-Budiño; Esteban Cano; Orlando Acosta; Virginia Leiro-Fernández; Ana Romero; Lurdes Planas-Cerezales; Ana Villar; Amalia Moreno; Rosalia Laporta; Vanesa Vicens-Zygmunt; Jessica Shull; Tomàs Franquet; Patricio Luburich; Maria Molina-Molina

doi:10.1016/j.arbres.2021.03.018

Different Faces of Idiopathic Pulmonary Fibrosis With Preserved Forced Vital Capacity

Arch Bronconeumol. 2022 Feb;58(2):135-141. doi: 10.1016/j.arbres.2021.03.018. Epub 2021 Mar 27.

[Article in English, Spanish]

Authors

Guadalupe Bermudo¹, Guillermo Suarez-Cuartin¹, Pilar Rivera-Ortega², Jose Antonio Rodriguez-Portal³, Jaume Sauleda⁴, Belen Nuñez⁴, Diego Castillo⁵, Myriam Aburto⁶, Karina Portillo⁷, Eva Balcells⁸, Diana Badenes-Bonet⁸, Claudia Valenzuela⁹, Estrella Fernandez-Fabrellas¹⁰, Teresa González-Budiño¹¹, Esteban Cano¹², Orlando Acosta¹³, Virginia Leiro-Fernández¹⁴, Ana Romero¹⁵, Lurdes Planas-Cerezales¹⁶, Ana Villar¹⁷, Amalia Moreno¹⁸, Rosalia Laporta¹⁹, Vanesa Vicens-Zygmunt¹, Jessica Shull¹, Tomàs Franquet⁵, Patricio Luburich²⁰, Maria Molina-Molina²¹

Affiliations

¹ ILD Unit, Respiratory Department, University Hospital of Bellvitge, IDIBELL, University of Barcelona, Spain.
² ILD Unit, Respiratory Department, University Hospital of Bellvitge, IDIBELL, University of Barcelona, Spain; ILD Unit, Respiratory Department, Wythenshawe Hospital, Manchester University NHS Foundation Trust, UK.
³ Respiratory Department, University Hospital Virgen del Rocío, Sevilla, Spain.
⁴ Respiratory Department, University Hospital Son Espases, Mallorca, Spain.
⁵ Respiratory Department, University Hospital De la Santa Creu i Sant Pau, Barcelona, Spain.
⁶ Respiratory Department, Hospital de Galdakao, Bizkaia, Spain.
⁷ Respiratory Department, University Hospital Germans Trias i Pujol, Badalona, Spain.
⁸ Respiratory Department, University Hospital del Mar, Barcelona, Spain; Pompeu Fabra University (UPF), Barcelona, Spain; CIBER Enfermedades Respiratorias (CIBERES), Madrid, Spain.
⁹ Respiratory Department, University Hospital de la Princesa, Madrid, Spain.
¹⁰ Respiratory Department, University Hospital General de Valencia, Valencia, Spain.
¹¹ Respiratory Department, University Hospital Central de Asturias (HUCA), Oviedo, Spain.
¹² Respiratory Department, University Hospital Lucus Agusti, Lugo, Spain.
¹³ Respiratory Department, University Hospital of Canarias, Santa Cruz de Tenerife, Spain.
¹⁴ Respiratory Department, University Hospital Alvaro Cunqueiro, Vigo, Spain.
¹⁵ Respiratory Department, University Hospital Virgen de las Nieves, Granada, Spain.
¹⁶ ILD Unit, Respiratory Department, University Hospital of Bellvitge, IDIBELL, University of Barcelona, Spain; Respiratory Department, Hospital de Viladecans, Barcelona, Spain.
¹⁷ Respiratory Department, University Hospital Vall d'Hebrón, Barcelona, Spain.
¹⁸ Respiratory Department, University Hospital Parc Taulí, Sabadell, Spain.
¹⁹ Respiratory Department, University Hospital Puerta del Hierro, Majadahonda, Spain.
²⁰ ILD Unit. Radiology Department. University Hospital of Bellvitge, Barcelona, Spain.
²¹ ILD Unit, Respiratory Department, University Hospital of Bellvitge, IDIBELL, University of Barcelona, Spain; CIBER Enfermedades Respiratorias (CIBERES), Madrid, Spain. Electronic address: mariamolinamolina@hotmail.com.

PMID: 33895005
DOI: 10.1016/j.arbres.2021.03.018

Abstract

Introduction: Idiopathic pulmonary fibrosis (IPF) is progressive and irreversible. Some discrepancies about IPF staging exists, especially in mild phases. Forced vital capacity (FVC) higher than 80% has been considered early or mild IPF even for the design of clinical trials.

Methods: Spanish multicentre, observational, retrospective study of IPF patients diagnosed between 2012 and 2016, based on the ATS/ERS criteria, which presented FVC greater or equal 80% at diagnosis. Clinical and demographic characteristics, lung function, radiological pattern, treatment, and follow-up were analyzed.

Results: 225 IPF patients were included, 72.9% were men. The mean age was 69.5 years. The predominant high-resolution computed tomography (HRCT) pattern was consistent usual interstitial pneumonia (UIP) (51.6%). 84.7% of patients presented respiratory symptoms (exertional dyspnea and/or cough) and 33.33% showed oxygen desaturation below 90% in the 6min walking test (6MWT). Anti-fibrotic treatment was initiated at diagnosis in 55.11% of patients. Median FVC was 89.6% (IQR 17) and 58.7% of patients had a decrease of diffusion lung capacity for carbon monoxide (DLCO) below 60% of theoretical value; most of them presented functional progression (61.4%) and higher mortality at 3 years (20.45%). A statistically significant correlation with the 3-years mortality was observed between DLCO <60% and consistent UIP radiological pattern.

Conclusions: Patients with preserved FVC but presenting UIP radiological pattern and moderate-severe DLCO decrease at diagnosis associate an increased risk of progression, death or lung transplantation. Therefore, in these cases, preserved FVC would not be representative of early or mild IPF.

Keywords: Diagnóstico precoz; Diffuse interstitial lung disease; Early diagnosis; Enfermedad pulmonar intersticial difusa; Fibrosis pulmonar idiopática; Idiopathic pulmonary fibrosis; Mortalidad; Mortality; Prognosis; Pronóstico.