Demographic and clinical profile of idiopathic pulmonary fibrosis patients in Spain: the SEPAR National Registry

被引：36

作者：

Fernandez-Fabrellas, Estrella ^{[1
]}

Molina-Molina, Maria ^{[2
]}

Soriano, Joan B. ^{[3
]}

Rodriguez Portal, Jose Antonio ^{[4
]}

Ancochea, Julio ^{[3
]}

Valenzuela, Claudia ^{[3
]}

Xaubet, Antoni ^{[5
]}

Aburto Barrenetxea, M. ^{[6
]}

Alfageme Michavila, I ^{[7
]}

Bollo de Miguel, E. ^{[8
]}

Cano, E. ^{[9
]}

Casanova Espinosa, A. ^{[10
]}

Castillo Villegas, D. ^{[11
]}

Figuerola Mendal, J. A. ^{[12
]}

Garcia Sevila, R. ^{[13
]}

Gaudo Navarro, J., I ^{[14
]}

Gomez Carrera, L. ^{[15
]}

Gonzalez, J. M. ^{[16
]}

Herrera Lara, S. ^{[17
]}

Laporta Hernandez, R. ^{[18
]}

Marin Gonzalez, M. ^{[19
]}

Nieto Barbero, M. A. ^{[20
]}

Portillo, K. ^{[21
]}

Romero, A. D. ^{[22
]}

Sanchez Simon-Talero, R. ^{[23
]}

Sancho Chust, J. N. ^{[24
]}

Sellares Torres, J. ^{[25
]}

Soler Sempere, M. J. ^{[26
]}

Sauleda Roig, J. ^{[27
]}

Tomas Lopez, L. ^{[28
]}

Villanueva Montes, M. ^{[29
]}

机构：

[1] Consorcio Hosp Gen Univ, Pulmonol Dept, Valencia, Spain

[2] Hosp Univ Bellvitge, Resp Dept, Interstitial Dis Unit, Carrer Feixa Llarga S-N, Barcelona 08907, Spain

[3] Hosp Univ La Princesa, Pulmonol Dept, Madrid, Spain

[4] Hosp Univ Virgen del Rocio, Pulmonol Dept, Seville, Spain

[5] Hosp Clin Barcelona, Pulmonol Dept, Barcelona, Spain

[6] Hosp Galdakao Usansolo, Bizkaia, Spain

[7] Hosp Univ Virgen de Valme, Seville, Spain

[8] Complejo Asistencial Univ Leon, Leon, Spain

[9] Hosp Univ Lucus Augusti, Lugo, Spain

[10] Hosp Univ Henares, Madrid, Spain

[11] Hosp Santa Creu & Sant Pau, Barcelona, Spain

[12] Hosp Clin Univ Lozano Blesa, Zaragoza, Spain

[13] Hosp Gen Univ Alicante, Alicante, Spain

[14] Hosp Univ Ramon y Cajal, Madrid, Spain

[15] Hosp Univ La Paz, Madrid, Spain

[16] Hosp Univ Salamanca, Salamanca, Spain

[17] Hosp Univ Doctor Peset, Valencia, Spain

[18] Hosp Univ Puerta Hierro Majadahonda, Madrid, Spain

[19] Hosp Clin Univ Valencia, Valencia, Spain

[20] Hosp Clin San Carlos, Madrid, Spain

[21] Hosp Badalona Germans Trias & Pujol, Barcelona, Spain

[22] Hosp Univ Virgen de las Nieves, Granada, Spain

[23] Hosp Univ Nuestra Senora Perpetuo Socorro, Albacete, Spain

[24] Hosp Univ St Joan Alacant, Alicante, Spain

[25] Hosp Clin Barcelona, Inst Torax, Barcelona, Spain

[26] Hosp Gen Univ Elche, Alicante, Spain

[27] Hosp Univ Son Espases, Palma De Mallorca, Illes Balears, Spain

[28] Hosp Univ Araba, Txagorritxu, Alaba, Spain

[29] Hosp San Agustin, Asturias, Spain

来源：

RESPIRATORY RESEARCH | 2019年 / 20卷 / 1期

关键词：

Anti-fibrotic treatment; Idiopathic pulmonary fibrosis; National registry; Spain; SEPAR; FORCED VITAL CAPACITY; DIAGNOSIS; DISEASE; PIRFENIDONE; SURVIVAL; INDEX; PATHOGENESIS; GUIDELINES; SYSTEM; CT;

D O I：

10.1186/s12931-019-1084-0

中图分类号：

R56 [呼吸系及胸部疾病];

学科分类号：

摘要：

BackgroundLittle is known on the characteristics of patients diagnosed with idiopathic pulmonary fibrosis (IPF) in Spain. We aimed to characterize the demographic and clinical profile of IPF patients included in the IPF National Registry of the Spanish Respiratory Society (SEPAR).MethodsThis is a prospective, observational, multicentre and nationwide study that involved 608 IPF patients included in the SEPAR IPF Registry up to June 27th, 2017, and who received any treatment for their disease. IPF patients were predominantly males, ex-smokers, and aged in their 70s, similar to other registries.ResultsUpon inclusion, meanSD predicted forced vital capacity was 77.6%+/- 19.4, diffusing capacity for carbon monoxide was 48.5%+/- 17.7, and the 6-min walk distance was 423.5m +/- 110.4. The diagnosis was mainly established on results from the high-resolution computed tomography in the proper clinical context (55.0% of patients), while 21.2% of patients required invasive procedures (surgical lung biopsy) for definitive diagnosis. Anti-fibrotic treatment was prescribed in 69.4% of cases, 51.5% pirfenidone and 17.9% nintedanib, overall with a good safety profile.Conclusions The SEPAR IPF Registry should help to further characterize current characteristics and future trends of IPF patients in Spain and compare/pool them with other registries and cohorts.

引用

页数：10

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