Latin American Registry of Idiopathic Pulmonary Fibrosis (REFIPI): Clinical Characteristics, Evolution and Treatment

• Fabián Caro ^[7] ; Ivette Buendía-Roldán ^[1] ; Lorena Noriega-Aguirre ^[8] ; María L. Alberti ^[7] ; Alexandre Amaral ^[9] ; Guillermo Arbo ^[10] ; Santiago Auteri ^[7] ; Aníbal Bermúdez ^[11] ; Pablo Curbelo ^[2] ; Manuel de Jesús Díaz Verduzco ^[12] ; Isabel De la Fuente ^[3] ; Juan I. Enghelmayer ^[13] ; Martin Fernández ^[7] ; Matías Florenzano ^[14] ; Fernando Guillen ^[15] ; Ronaldo Kairalla ^[4] ; Yuri Liberato ^[16] ; Carlos Matiz ^[17] ; Mayra Mejía ^[1] ; Viviana Moyano ^[11] ; Alfredo Pachas ^[18] ; Silvia V. Escotorin ^[19] ; Gabriela Tabaj ^[20] ; Esther Tavera ^[21] ; Alvaro Undurraga ^[14] ; Brenda Varela ^[5] ; José Luis Velazquez ^[6] ; Moises Selman ^[1]
  1. [1] Instituto Nacional de Enfermedades Respiratorias

     Instituto Nacional de Enfermedades Respiratorias

     México

     
  2. [2] Hospital Maciel

     Hospital Maciel

     Uruguay

     
  3. [3] Hospital Dipreca

     Hospital Dipreca

     Santiago, Chile

     
  4. [4] Universidade de São Paulo

     Universidade de São Paulo

     Brasil

     
  5. [5] Hospital Alemán

     Hospital Alemán

     Argentina

     
  6. [6] Hospital San Juan de Dios

     Hospital San Juan de Dios

     Santiago, Chile

     
  7. [7] Hospital de Rehabilitación Respiratoria “María Ferrer”, Buenos Aires, Argentina
  8. [8] Centro de Diagnóstico y Tratamiento de Enfermedades Respiratorias (CEDITER), Panamá
  9. [9] División de Neumología, Instituto de Curazao (InCor), Hospital de Clínicas, Universidad de San Pablo, San Pablo, Brasil
  10. [10] Centro Médico Santa Julia, Asunción, Paraguay
  11. [11] Hospital Italiano de Córdoba, Córdoba, Argentina
  12. [12] Hospital Regional Cárdenas de la Vega de Sinaloa, Sinaloa, México
  13. [13] Hospital de Clínicas José de San Martín Martin, Buenos Aires, Argentina
  14. [14] Instituto Nacional del Tórax, Santiago, Chile
  15. [15] Hospital Dr. Belisario Domínguez, Ciudad de México, México
  16. [16] Hospital Belén de Trujillo, Trujillo, Peru
  17. [17] Fundación Santa Fe de Bogotá, Bogota, Colombia
  18. [18] Centro de Enfermedades Respiratorias Neumología Peruana, Lima, Peru
  19. [19] El Carmen de Salta Sanatorium, Salta, Argentina
  20. [20] Hospital del Tórax Dr. Antonio A. Cetrángolo, Buenos Aires, Argentina
  21. [21] Clínica Universitaria Unión Médica del Norte, República Dominicana

  Mostrar afiliaciones +
• Localización: Archivos de bronconeumología: Organo oficial de la Sociedad Española de Neumología y Cirugía Torácica SEPAR y la Asociación Latinoamericana de Tórax ( ALAT ), ISSN 0300-2896, Vol. 58, Nº. 12 (December), 2022, págs. 794-801
• Idioma: inglés
• Texto completo no disponible (Saber más ...)
• Resumen

  • 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, an average reduction of FVC (70.9%) and DLCO (53.7%) was 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.