Predictors of death or lung transplant after a diagnosis of idiopathic pulmonary fibrosis: insights from the IPF-PRO Registry

被引：44

作者：

Snyder, Laurie ^{[1
,2
]}

Neely, Megan L. ^{[1
,2
]}

Hellkamp, Anne S. ^{[1
]}

O'Brien, Emily ^{[1
]}

de Andrade, Joao ^{[3
]}

Conoscenti, Craig S. ^{[4
]}

Leonard, Thomas ^{[4
]}

Bender, Shaun ^{[4
]}

Gulati, Mridu ^{[5
]}

Culver, Daniel A. ^{[6
]}

Kaner, Robert J. ^{[7
]}

Palmer, Scott ^{[1
,2
]}

Kim, Hyun Joo ^{[8
]}

Asi, Wael ^{[9
]}

Baker, Albert ^{[10
]}

Beegle, Scott ^{[11
]}

Belperio, John A. ^{[12
]}

Condos, Rany ^{[13
]}

Cordova, Francis ^{[14
]}

Dilling, Daniel ^{[15
]}

Flaherty, Kevin ^{[16
]}

Glassberg, Marilyn ^{[17
]}

Guntupalli, Kalpalatha ^{[18
]}

Gupta, Nishant ^{[19
]}

Case, Amy Hajari ^{[20
]}

Hotchkin, David ^{[21
]}

Huie, Tristan ^{[22
]}

Kaner, Robert ^{[23
]}

Kim, Hyun ^{[8
]}

Kreider, Maryl ^{[24
]}

Lancaster, Lisa ^{[25
]}

Lasky, Joseph ^{[26
]}

Lederer, David ^{[27
]}

Lee, Doug ^{[28
,29
]}

Liesching, Timothy ^{[30
]}

Lipchik, Randolph ^{[31
]}

Lobo, Jason ^{[32
]}

Mageto, Yolanda ^{[33
]}

Menon, Prema ^{[34
]}

Morrison, Lake ^{[2
]}

Namen, Andrew ^{[35
]}

Oldham, Justin ^{[36
]}

Raj, Rishi ^{[37
]}

Ramaswamy, Murali ^{[38
]}

Russell, Tonya ^{[39
]}

Sachs, Paul ^{[40
]}

Safdar, Zeenat ^{[41
]}

Sigal, Barry ^{[42
,43
]}

Silhan, Leann ^{[44
]}

Strek, Mary ^{[45
]}

机构：

[1] Duke Clin Res Inst, Durham, NC 27705 USA

[2] Duke Univ, Med Ctr, Durham, NC 27710 USA

[3] Univ Alabama Birmingham, Birmingham, AL USA

[4] Boehringer Ingelheim Pharmaceut Inc, 90 E Ridge POB 368, Ridgefield, CT 06877 USA

[5] Yale Sch Med, New Haven, CT USA

[6] Cleveland Clin, Cleveland, OH 44106 USA

[7] Weill Cornell Med, New York, NY USA

[8] Univ Minnesota, Minneapolis, MN USA

[9] Renovatio Clin, The Woodlands, TX USA

[10] Lynchburg Pulm Associates, Lynchburg, VA USA

[11] Albany Med Ctr, Albany, NY USA

[12] Univ Calif Los Angeles, Los Angeles, CA USA

[13] NYU Med Ctr, New York, NY 10016 USA

[14] Temple Univ, Philadelphia, PA 19122 USA

[15] Loyola Univ Hlth Syst, Maywood, IL USA

[16] Univ Michigan, Ann Arbor, MI 48109 USA

[17] Univ Miami, Miami, FL USA

[18] Baylor Coll Med, Houston, TX 77030 USA

[19] Univ Cincinnati, Med Ctr, Cincinnati, OH 45267 USA

[20] Piedmont Healthcare, Austell, GA USA

[21] Oregon Clin, Portland, OR USA

[22] Natl Jewish Hosp, Denver, CO USA

[23] Weill Cornell Med Coll, New York, NY USA

[24] Univ Penn, Philadelphia, PA 19104 USA

[25] Vanderbilt Univ, 221 Kirkland Hall, Nashville, TN 37235 USA

[26] Tulane Univ, New Orleans, LA 70118 USA

[27] Columbia Univ, Med Ctr, New York Presbyterian Hosp, New York, NY USA

[28] Wilmington Hlth, Wilmington, NC USA

[29] PMG Res, Wilmington, NC USA

[30] Lahey Clin Fdn, Burlington, MA USA

[31] Froedtert & Med Coll Wisconsin Community Phys, Milwaukee, WI USA

[32] Univ N Carolina, Chapel Hill, NC USA

[33] Baylor Univ, Med Ctr Dallas, Dallas, TX USA

[34] Vermont Lung Ctr, Colchester, VT USA

[35] Wake Forest Univ, Winston Salem, NC 27109 USA

[36] Univ Calif Davis, Sacramento, CA 95817 USA

[37] Stanford Univ, Stanford, CA 94305 USA

[38] PulmonIx LLC, Greensboro, NC USA

[39] Washington Univ, St Louis, MO 63110 USA

[40] Pulm Associates Stamford, Stamford, CT USA

[41] Houston Methodist Lung Ctr, Houston, TX USA

[42] Salem Chest, Winston Salem, NC USA

[43] Southeastern Clin Res Ctr, Winston Salem, NC USA

[44] UT Southwestern Med Ctr, Dallas, TX USA

[45] Univ Chicago, Chicago, IL 60637 USA

[46] Univ Louisville, Louisville, KY 40292 USA

[47] South Miami Hosp, South Miami, FL USA

[48] St Josephs Hosp, Phoenix, AZ USA

[49] Med Univ South Carolina, Charleston, SC 29425 USA

来源：

RESPIRATORY RESEARCH | 2019年 / 20卷

关键词：

FORCED VITAL CAPACITY; BODY-MASS INDEX; ACUTE EXACERBATION; RESPIRATORY HOSPITALIZATION; DISEASE PROGRESSION; CLINICAL-COURSE; SURVIVAL; MORTALITY; PREVALENCE; HRCT;

D O I：

10.1186/s12931-019-1043-9

中图分类号：

R56 [呼吸系及胸部疾病];

学科分类号：

摘要：

BackgroundIdiopathic pulmonary fibrosis (IPF) is a progressive disease with a variable clinical course and high mortality. We used data from a large national US registry of patients with IPF to investigate relationships between patient characteristics, including markers of disease severity, and mortality.MethodsThe analysis cohort comprised patients enrolled in the IPF-PRO Registry from its inception on 5 June 2014 to 26 October 2017. The primary criterion for inclusion in this registry is that patients must be diagnosed or confirmed withIPF at the enrolling centre within 6months. Associations between patient characteristics and markers of disease severity at enrolment and mortality outcomes were investigated using univariable, multivariable and adjustment models.ResultsAmong 662 patients enrolled, 111 patients died or had a lung transplant over a follow-up period of 30months. The probability of being free of both events at month 30 was 50.6% (95% CI: 40.0, 60.2). When patient characteristics and markers of disease severity were jointly examined in a multivariable analysis, oxygen use at rest (hazard ratio [HR] 2.44 [95% CI: 1.45, 4.10]), lower forced vital capacity (FVC) % predicted (HR 1.28 [95% CI: 1.10, 1.49] per 10% decrease) and diffusion capacity for carbon monoxide (DLco) % predicted (HR 1.25 [95% CI: 1.04, 1.51] per 10% decrease) were significantly associated with increased risk of death or lung transplant. The risk of death or lung transplant increased with increasing age in patients 62years old (HR 1.18 [95% CI: 0.99, 1.40] per 5-year increase), and decreased with increasing age in patients <62years old (HR 0.60 [95% CI: 0.39, 0.92] per 5-year increase).ConclusionsIn an observational US registry of patients with IPF, oxygen use at rest, lower FVC % predicted, and lower DLco % predicted were associated with risk of death or lung transplant. An audio podcast of the lead author discussing these data can be downloaded from: http://www.usscicomms.com/respiratory/snyder/IPF-PROsurvival1/.Trial registrationClinicalTrials.gov number: NCT01915511.

引用

页数：10

共 50 条

[1] Predictors of death or lung transplant after a diagnosis of idiopathic pulmonary fibrosis: insights from the IPF-PRO Registry
Laurie Snyder
Megan L. Neely
Anne S. Hellkamp
Emily O’Brien
Joao de Andrade
Craig S. Conoscenti
Thomas Leonard
Shaun Bender
Mridu Gulati
Daniel A. Culver
Robert J. Kaner
Scott Palmer
Hyun Joo Kim
[J]. Respiratory Research, 20
[2] Predictors of death or transplant in patients with idiopathic pulmonary fibrosis in the IPF-PRO Registry
Snyder, Laurie
Neely, Megan L.
Hellkamp, Anne
O'Brien, Emily
Flaherty, Kevin R.
De Andrade, Joao
Conoscenti, Craig
Leonard, Thomas
Bender, Shaun
Gulati, Mridu
Culver, Daniel A.
Kaner, Robert J.
Palmer, Scott
Kim, Hyun J.
[J]. EUROPEAN RESPIRATORY JOURNAL, 2018, 52
[3] Time to diagnosis of idiopathic pulmonary fibrosis in the IPF-PRO Registry
Snyder, Laurie D.
Mosher, Christopher
Holtze, Colin H.
Lancaster, Lisa H.
Flaherty, Kevin R.
Noth, Imre
Neely, Megan L.
Hellkamp, Anne S.
Bender, Shaun
Conoscenti, Craig S.
de Andrade, Joao A.
Whelan, Timothy P. M.
[J]. BMJ OPEN RESPIRATORY RESEARCH, 2020, 7 (01)
[4] Time to Diagnosis of Idiopathic Pulmonary Fibrosis in the IPF-PRO Registry
Snyder, L. D.
Mosher, C.
Holtze, C. H.
Lancaster, L. H.
Flaherty, K. R.
Noth, I.
Neely, M. L.
Hellkamp, A. S.
Bender, S.
Conoscenti, C. S.
De Andrade, J. A.
Whelan, T. P. M.
[J]. AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE, 2020, 201
[5] HOSPITALIZATIONS AND OUTCOMES IN PATIENTS WITH IDIOPATHIC PULMONARY FIBROSIS (IPF): INSIGHTS FROM THE IPF-PRO REGISTRY
Kim, Hyun
Neely, Megan
Janssen, Katherine
Adegunsoye, Ayodeji
Strek, Mary
Yow, Eric
Conoscenti, Craig
Leonard, Thomas
Snyder, Laurie
[J]. CHEST, 2020, 158 (04) : 1062A - 1063A
[6] Association of Circulating Proteins with Death or Lung Transplant in Patients with Idiopathic Pulmonary Fibrosis in the IPF-PRO Registry Cohort
Jamie L. Todd
Megan L. Neely
Robert Overton
Hillary Mulder
Jesse Roman
Joseph A. Lasky
Joao A. de Andrade
Mridu Gulati
Howard Huang
Thomas B. Leonard
Christian Hesslinger
Imre Noth
John A. Belperio
Kevin R. Flaherty
Scott M. Palmer
[J]. Lung, 2022, 200 : 11 - 18
[7] Survival in Patients with Idiopathic Pulmonary Fibrosis (IPF): Data from the IPF-PRO Registry
Palmer, S. M.
Snyder, L.
Neely, M. L.
Yow, E.
Bender, S.
Culver, D. A.
de Andrade, J.
Gulati, M.
Kaner, R. J.
Leonard, T.
O'Brien, E.
Pushlar, N.
Conoscenti, C. S.
[J]. AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE, 2018, 197
[8] A Multivariable Protein-Inclusive Predictor of Death or Lung Transplant in Patients with Idiopathic Pulmonary Fibrosis: Data from the IPF-PRO Registry
Todd, J. L.
Neely, M. L.
Mulder, H.
Lancaster, L. H.
White, E. S.
Hesslinger, C.
[J]. AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE, 2022, 205
[9] CHARACTERISTICS OF PATIENTS WITH IDIOPATHIC PULMONARY FIBROSIS (IPF) IN THE US: DATA FROM THE IPF-PRO REGISTRY
Culver, Daniel
Yow, Eric
Neely, Megan
Belperio, John
Bender, Shaun
De Andrade, Joao
Roman, Jesse
Whelan, Timothy
Palmer, Scott
Conoscenti, Craig
[J]. CHEST, 2018, 154 (04) : 397A - 398A
[10] Treatment Patterns in Patients with Idiopathic Pulmonary Fibrosis (IPF): Data from the IPF-PRO Registry
Salisbury, M. L.
Conoscenti, C. S.
Culver, D. A.
Yow, E.
Neely, M.
Bender, S.
Hartmann, N.
Palmer, S.
Leonard, T.
[J]. AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE, 2019, 199

← 1 2 3 4 5 →