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Original Article
Uncorrected Proof. Available online 18 June 2022
Latin American Registry of Idiopathic Pulmonary Fibrosis (REFIPI): Clinical Characteristics, Evolution and Treatment
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Fabián Caroa,
Corresponding author
fabiancarodoc@gmail.com

Corresponding authors.
, Ivette Buendía-Roldánb, Lorena Noriegac, Laura M. Albertia, Alexandre Amarald, Guillermo Arboe, Santiago Auteria, Aníbal Bermúdezf, Pablo Curbelog, Manuel de Jesús Díaz Verduzcoh, Isabel De la Fuentei, Juan I. Enghelmayerj, Martin Fernándeza, Matías Florenzanok, Fernando Guillenl, Ronaldo Kairallad, Yuri Liberatom, Carlos Matizn, Mayra Mejíab, Viviana Moyanof, Alfredo Pachaso, Silvia V. Escotorinp, Gabriela Tabajq, Esther Taverar, Alvaro Undurragak, Brenda Varelas, José Luis Velazquezt, Moises Selmanb,
Corresponding author
ivettebu@yahoo.com.mx

Corresponding authors.
, the REFIPI study group
a Hospital de Rehabilitación Respiratoria “María Ferrer”, Buenos Aires, Argentina
b Instituto Nacional de Enfermedades Respiratorias, Mexico City, Mexico
c Centro de Diagnóstico y Tratamiento de Enfermedades Respiratorias (CEDITER), Mexico
d División de neumología, Instituto de Curazao (InCor), Hospital de Clínicas, Universidad de san Pablo, San Pablo, Brazil
e Centro Médico Santa Julia, Asunción, Paraguay
f Hospital Italiano de Córdoba, Cordoba, Argentina
g Hospital Maciel, Montevideo, Uruguay
h Hospital Regional Cárdenas de la Vega de Sinaloa, Sinaloa, Mexico
i Hospital Dipreca, Santiago, Chile
j Hospital de Clínicas José de San Martín Martin, Buenos Aires, Argentina
k Instituto Nacional del Tórax, Santiago, Chile
l Hospital Dr. Belisario Domínguez, Mexico City, Mexico
m Hospital Belén de Trujillo, Trujillo, Peru
n Fundación Santa Fe de Bogotá, Bogota, Colombia
o Centro de Enfermedades Respiratorias Neumología Peruana, Lima, Peru
p El Carmen de Salta Sanatorium, Salta, Argentina
q Hospital del Tórax Dr. Antonio A. Cetrángolo, Buenos Aires, Argentina
r Clínica Universitaria Unión Médica del Norte, Dominican Republic
s Hospital Alemán, Buenos Aires, Argentina
t Hospital San Juan de Dios, Santiago, Chile
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Abstract
Introduction

Idiopathic pulmonary fibrosis (IPF) is a progressive, irreversible and frequently fatal disease. Currently there are national and multinational registries in Europe, United States, Australia and China to better understand the magnitude of the problem and the characteristics of the IPF patients. However, there are no national or regional registries in Latin America, so the objective of this study was to carry out a Latin American registry that would allow the identification of IPF patients in our region.

Methodology

A system consisting of 3 levels of control was designed, ensuring that patients met the diagnostic criteria for IPF according to international guidelines ATS/ERS/ALAT/JRS 2011. Demographic, clinical, serological, functional, tomographic, histological and treatment variables were recorded through a digital platform.

Results

761 IPF patients from 14 Latin American countries were included for analysis, 74.7% were male, with a mean age of 71.9+8.3 years. In general there was a long period of symptoms before definitive diagnosis (median 1 year). In functional tests, a FVC of 70.9% and DLCO 53.7% were detected. 72% received at least one antifibrotic drug (pirfenidone or nintedanib) and 11.2% of the patients had an acute exacerbation, of which 38 (45.2%) died from this cause.

Conclusions

Like other registries, we found that there is difficulty in the recognition and excessive delay in the diagnosis of IPF in Latin America. Most of the patients in REFIPI received antifibrotics; these were well tolerated and associated with fewer adverse events than those reported in clinical trials.

Keywords:
Idiopathic pulmonary fibrosis
Latin American

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