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Article: Clinical Overview of Progressive Fibrotic Interstitial Lung Disease.

Case, Amy Hajari

2022 Volume 9, Page(s) 858339

Abstract: Interstitial lung diseases (ILD) on the whole have variable prognoses, but there are those which manifest with fibrosis and are characterized by disease progression. Chief among these is idiopathic pulmonary fibrosis, but other ILDs, including autoimmune ...

Abstract	Interstitial lung diseases (ILD) on the whole have variable prognoses, but there are those which manifest with fibrosis and are characterized by disease progression. Chief among these is idiopathic pulmonary fibrosis, but other ILDs, including autoimmune ILD and chronic hypersensitivity pneumonitis, may have a progressive fibrotic phenotype also. A usual interstitial pneumonia pattern of lung involvement is a prominent risk factor for such a course, suggesting shared fibrotic pathways that may be targeted by antifibrotic therapies. This brief review describes ILDs that are most commonly fibrotic, shared risk factors for development of PF-ILD, and evidence for antifibrotic use in their management.
Language	English
Publishing date	2022-03-15
Publishing country	Switzerland
Document type	Journal Article ; Review
ZDB-ID	2775999-4
ISSN	2296-858X
ISSN	2296-858X
DOI	10.3389/fmed.2022.858339
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Article ; Online: Practice Patterns for Screening and Treating Interstitial Lung Disease-related Pulmonary Hypertension at Specialty Care Centers in the United States.

Khan, Sarah L / Danoff, Sonye K / Kulkarni, Tejaswini / Reichuber, Janell / Shifren, Adrian / Shlobin, Oksana A / Thavarajah, Krishna / Warrior, Krishnan / Case, Amy Hajari

Annals of the American Thoracic Society

2024

Language	English
Publishing date	2024-04-16
Publishing country	United States
Document type	Journal Article
ZDB-ID	2717461-X
ISSN	2325-6621 ; 1943-5665 ; 2325-6621
ISSN (online)	2325-6621 ; 1943-5665
ISSN	2325-6621
DOI	10.1513/AnnalsATS.202402-141RL
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Article ; Online: Defining the pathway to timely diagnosis and treatment of interstitial lung disease: a US Delphi survey.

Case, Amy Hajari / Beegle, Scott / Hotchkin, David L / Kaelin, Thomas / Kim, Hyun Joo / Podolanczuk, Anna J / Ramaswamy, Murali / Remolina, Carlos / Salvatore, Mary M / Tu, Conan / de Andrade, Joao A

BMJ open respiratory research

2023 Volume 10, Issue 1

Abstract: Introduction: Timely diagnosis of interstitial lung disease (ILD) is limited by obstacles in the current patient pathway. Misdiagnosis and delays are common and may lead to a significant burden of diagnostic procedures and worse outcomes. This Delphi ... ...

Abstract	Introduction: Timely diagnosis of interstitial lung disease (ILD) is limited by obstacles in the current patient pathway. Misdiagnosis and delays are common and may lead to a significant burden of diagnostic procedures and worse outcomes. This Delphi survey aimed to identify consensus on the key steps that facilitate the patient journey to an accurate ILD diagnosis and appropriate management in the US. Methods: A modified Delphi analysis was conducted, comprising three online surveys based on a comprehensive literature search. The surveys spanned five domains (guidelines, community screening, diagnosis, management and specialist referral) and were completed by a panel of US physicians, including primary care physicians and pulmonologists practising in community or academic settings. A priori definitions of consensus agreement were median scores of 2-3 (agree strongly/agree), with an IQR of 0-1 for questions on a 7-point Likert scale from -3 to 3, or ≥80% agreement for binary questions. Results: Forty-nine panellists completed the surveys and 62 statements reached consensus agreement. There was consensus agreement on what should be included in the primary care evaluation of patients with suspected ILD and the next steps following workup. Regarding diagnosis in community pulmonology care, consensus agreement was reached on the requisition and reporting of high-resolution CT scans and the appropriate circumstances for holding multidisciplinary discussions. Additionally, there was consensus agreement on which symptoms and comorbidities should be monitored, the frequency of consultations and the assessment of disease progression. Regarding specialist referral, consensus agreement was reached on which patients should receive priority access to ILD centres and the contents of the referral package. Conclusions: These findings clarify the most common issues that should merit further evaluation for ILD and help define the steps for timely, accurate diagnosis and appropriate collaborative specialty management of patients with ILD.
MeSH term(s)	Humans ; Lung Diseases, Interstitial/diagnosis ; Lung Diseases, Interstitial/therapy ; Comorbidity ; Surveys and Questionnaires ; Diagnostic Errors ; Physicians
Language	English
Publishing date	2023-11-24
Publishing country	England
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	2736454-9
ISSN	2052-4439 ; 2052-4439
ISSN (online)	2052-4439
ISSN	2052-4439
DOI	10.1136/bmjresp-2022-001594
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Article ; Online: Associations between resources and practices of ILD centers and outcomes in patients with idiopathic pulmonary fibrosis: data from the IPF-PRO Registry.

de Andrade, Joao A / Kulkarni, Tejaswini / Neely, Megan L / Hellkamp, Anne S / Case, Amy Hajari / Culver, Daniel A / Guntupalli, Kalpalatha / Bender, Shaun / Conoscenti, Craig S / Snyder, Laurie D

Respiratory research

2022 Volume 23, Issue 1, Page(s) 3

Abstract: Background: Performance benchmarks for the management of idiopathic pulmonary fibrosis (IPF) have not been established. We used data from the IPF-PRO Registry, an observational registry of patients with IPF managed at sites across the US, to examine ... ...

Abstract	Background: Performance benchmarks for the management of idiopathic pulmonary fibrosis (IPF) have not been established. We used data from the IPF-PRO Registry, an observational registry of patients with IPF managed at sites across the US, to examine associations between the characteristics of the enrolling sites and patient outcomes. Methods: An online survey was used to collect information on the resources, operations, and self-assessment practices of IPF-PRO Registry sites that enrolled ≥ 10 patients. Site variability in 1-year event rates of clinically relevant outcomes, including death, death or lung transplant, and hospitalization, was assessed. Models were adjusted for differences in patient case mix by adjusting for known predictors of each outcome. We assessed whether site-level heterogeneity existed for each patient-level outcome, and if so, we investigated potential drivers of the heterogeneity. Results: All 27 sites that enrolled ≥ 10 patients returned the questionnaire. Most sites were actively following > 100 patients with IPF (70.4%), had a lung transplant program (66.7%), and had a dedicated ILD nurse leader (77.8%). Substantial heterogeneity was observed in the event rates of clinically relevant outcomes across the sites. After controlling for patient case mix, there were no outcomes for which the site variance component was significantly different from 0, but the p-value for hospitalization was 0.052. Starting/completing an ILD-related quality improvement project in the previous 2 years was associated with a lower risk of hospitalization (HR 0.60 [95% CI 0.44, 0.82]; p = 0.001). Conclusions: Analyses of data from patients with IPF managed at sites across the US found no site-specific characteristics or practices that were significantly associated with clinically relevant outcomes after adjusting for patient case mix. Trial registration ClinicalTrials.gov, NCT01915511. Registered 5 August 2013, https://clinicaltrials.gov/ct2/show/NCT01915511.
MeSH term(s)	Aged ; Female ; Follow-Up Studies ; Hospitalization/statistics & numerical data ; Humans ; Idiopathic Pulmonary Fibrosis/surgery ; Lung Transplantation/statistics & numerical data ; Male ; Middle Aged ; Registries ; Retrospective Studies ; Surveys and Questionnaires
Language	English
Publishing date	2022-01-07
Publishing country	England
Document type	Journal Article ; Multicenter Study ; Observational Study
ZDB-ID	2041675-1
ISSN	1465-993X ; 1465-993X
ISSN (online)	1465-993X
ISSN	1465-993X
DOI	10.1186/s12931-021-01921-7
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Article ; Online: Implementation of guideline recommendations and outcomes in patients with idiopathic pulmonary fibrosis: Data from the IPF-PRO registry.

de Andrade, Joao A / Kulkarni, Tejaswini / Neely, Megan L / Hellkamp, Anne S / Case, Amy Hajari / Guntupalli, Kalpalatha / Bender, Shaun / Conoscenti, Craig S / Snyder, Laurie D

Respiratory medicine

2021 Volume 189, Page(s) 106637

Abstract: Background: Few data are available on the extent to which clinical practice is aligned with international guidelines for the management of idiopathic pulmonary fibrosis (IPF). We investigated the extent to which management guidelines for IPF have been ... ...

Abstract	Background: Few data are available on the extent to which clinical practice is aligned with international guidelines for the management of idiopathic pulmonary fibrosis (IPF). We investigated the extent to which management guidelines for IPF have been implemented in the US IPF-PRO Registry and associations between implementation of guidelines and clinical outcomes. Methods: We assessed the implementation of eight recommendations in clinical practice guidelines within the 6 months after enrollment: visit to a specialized clinic; pulmonary function testing; use of oxygen in patients with resting hypoxemia and exercise-induced hypoxemia; referral for pulmonary rehabilitation; treatment of gastro-esophageal reflux disease; initiation of anti-fibrotic therapy; referral for lung transplant evaluation. An implementation score was calculated as the number of recommendations achieved divided by the number for which the patient was eligible. Associations between implementation score and outcomes were analyzed using logistic regression and Cox proportional hazards models. Results: Among 727 patients, median (Q1, Q3) implementation score was 0.6 (0.5, 0.8). Patients with an implementation score >0.6 had greater disease severity than those with a lower score. Implementation was lowest for referral for pulmonary rehabilitation (19.5%) and lung transplant evaluation (22.3%). In unadjusted models, patients with higher implementation scores had a greater risk of death, death or lung transplant, and hospitalization, but no significant associations were observed in adjusted models. Conclusions: Management guidelines were more likely to be implemented in patients with IPF with greater disease severity. When adjusted for disease severity, no association was found between implementation of management guidelines and clinical outcomes.
MeSH term(s)	Guideline Adherence ; Hospitalization/statistics & numerical data ; Humans ; Idiopathic Pulmonary Fibrosis/mortality ; Idiopathic Pulmonary Fibrosis/therapy ; Lung Transplantation/statistics & numerical data ; Oxygen Inhalation Therapy ; Referral and Consultation/statistics & numerical data ; Registries ; Respiratory Function Tests ; Severity of Illness Index
Language	English
Publishing date	2021-09-29
Publishing country	England
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	1003348-8
ISSN	1532-3064 ; 0954-6111
ISSN (online)	1532-3064
ISSN	0954-6111
DOI	10.1016/j.rmed.2021.106637
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Article ; Online: Sustained Clinical Benefits of Spiration Valve System in Patients with Severe Emphysema: 24-Month Follow-Up of EMPROVE.

Annals of the American Thoracic Society

2023 Volume 21, Issue 2, Page(s) 251–260

Abstract: Rationale: ...

Abstract	Rationale:
MeSH term(s)	Humans ; Quality of Life ; Follow-Up Studies ; Bronchoscopy ; Treatment Outcome ; Pulmonary Emphysema ; Emphysema ; Forced Expiratory Volume ; Dyspnea/etiology ; Pulmonary Disease, Chronic Obstructive/complications
Language	English
Publishing date	2023-11-10
Publishing country	United States
Document type	Randomized Controlled Trial ; Multicenter Study ; Journal Article
ZDB-ID	2717461-X
ISSN	2325-6621 ; 1943-5665 ; 2325-6621
ISSN (online)	2325-6621 ; 1943-5665
ISSN	2325-6621
DOI	10.1513/AnnalsATS.202306-520OC
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Article ; Online: Associations between Patient-reported Outcomes and Death or Lung Transplant in Idiopathic Pulmonary Fibrosis. Data from the Idiopathic Pulmonary Fibrosis Prospective Outcomes Registry.

Case, Amy Hajari / Hellkamp, Anne S / Neely, Megan L / Bender, Shaun / Dilling, Daniel F / Gulati, Mridu / Hotchkin, David L / Huie, Tristan J / Lancaster, Lisa / Snyder, Laurie D / Conoscenti, Craig S / Palmer, Scott M

Annals of the American Thoracic Society

2020 Volume 17, Issue 6, Page(s) 699–705

Abstract: Rationale: ...

Abstract	Rationale:
MeSH term(s)	Aged ; Disease Progression ; Exercise ; Female ; Humans ; Idiopathic Pulmonary Fibrosis/diagnosis ; Idiopathic Pulmonary Fibrosis/mortality ; Idiopathic Pulmonary Fibrosis/surgery ; Lung Transplantation/mortality ; Lung Transplantation/trends ; Male ; Patient Reported Outcome Measures ; Proportional Hazards Models ; Prospective Studies ; Registries ; Surveys and Questionnaires ; United States/epidemiology
Language	English
Publishing date	2020-02-05
Publishing country	United States
Document type	Journal Article ; Observational Study ; Research Support, Non-U.S. Gov't
ZDB-ID	2717461-X
ISSN	2325-6621 ; 1943-5665 ; 2325-6621
ISSN (online)	2325-6621 ; 1943-5665
ISSN	2325-6621
DOI	10.1513/AnnalsATS.201906-437OC
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Article ; Online: Smartphone-Guided Self-prone Positioning vs Usual Care in Nonintubated Hospital Ward Patients With COVID-19: A Pragmatic Randomized Clinical Trial.

Chest

2022 Volume 162, Issue 4, Page(s) 782–791

Abstract: Background: Safe, effective, and easily implementable treatments that reduce the progression of respiratory failure in COVID-19 are urgently needed. Despite the increased adoption of prone positioning during the pandemic, the effectiveness of this ... ...

Abstract	Background: Safe, effective, and easily implementable treatments that reduce the progression of respiratory failure in COVID-19 are urgently needed. Despite the increased adoption of prone positioning during the pandemic, the effectiveness of this technique on progression of respiratory failure among nonintubated patients is unclear. Research question: What is the effectiveness of smartphone-guided self-prone positioning recommendations and instructions compared with usual care in reducing progression of respiratory failure among nonintubated patients with COVID-19? Study design and methods: Awake Prone Position for Early Hypoxemia in COVID-19 (APPEX-19) is a multicenter randomized clinical trial that randomized nonintubated adults with COVID-19 on < 6 L/min of supplemental oxygen to receive a smartphone-guided self-prone positioning intervention or usual care. The primary outcome was the composite of respiratory deterioration (an increase in supplemental oxygen requirement) or ICU transfer. Using a Bayesian statistical approach, the posterior probability of superiority within each treatment arm (superiority threshold 95%) was calculated. Results: The trial was stopped early for slow enrollment. A total of 293 participants were included in the modified intention-to-treat analysis (159 self-prone positioning intervention and 134 usual care). Among participants who self-reported body positioning (n = 139 [70 intervention, 69 usual care]), 71.4% in the intervention arm and 59.4% in the usual care arm attempted prone positioning. Thirty-one participants (posterior mean, 24.7%; 95% credible interval, 18.6-31.4) receiving usual care and 32 participants (posterior mean, 22.1%; 95% credible interval, 16.6-28.1) receiving the self-prone positioning intervention experienced the primary outcome; the posterior probability of superiority for the self-prone positioning intervention was 72.1%, less than the 95% threshold for superiority. Adverse events occurred in 26.9% of participants in the usual care arm and in 11.9% of participants in the intervention arm. Interpretation: Among nonintubated patients with COVID-19, smartphone-guided self-prone positioning recommendations and instructions did not promote strong adherence to prone positioning. Clinical trial registration: ClinicalTrials.gov; No.: NCT04344587; URL: www. Clinicaltrials: gov.
MeSH term(s)	Adult ; Bayes Theorem ; COVID-19 ; Hospitals ; Humans ; Oxygen ; Prone Position ; Respiratory Insufficiency/therapy ; SARS-CoV-2 ; Smartphone
Chemical Substances	Oxygen (S88TT14065)
Language	English
Publishing date	2022-05-18
Publishing country	United States
Document type	Journal Article ; Multicenter Study ; Randomized Controlled Trial ; Research Support, N.I.H., Extramural
ZDB-ID	1032552-9
ISSN	1931-3543 ; 0012-3692
ISSN (online)	1931-3543
ISSN	0012-3692
DOI	10.1016/j.chest.2022.05.009
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Article ; Online: Improving Lung Function in Severe Heterogenous Emphysema with the Spiration Valve System (EMPROVE). A Multicenter, Open-Label Randomized Controlled Clinical Trial.

Criner, Gerard J / Delage, Antoine / Voelker, Kirk / Hogarth, D Kyle / Majid, Adnan / Zgoda, Michael / Lazarus, Donald R / Casal, Roberto / Benzaquen, Sadia B / Holladay, Robert C / Wellikoff, Adam / Calero, Karel / Rumbak, Mark J / Branca, Paul R / Abu-Hijleh, Muhanned / Mallea, Jorge M / Kalhan, Ravi / Sachdeva, Ashutosh / Kinsey, C Matthew /

Lamb, Carla R / Reed, Michael F / Abouzgheib, Wissam B / Kaplan, Phillip V / Marrujo, Gregory X / Johnstone, David W / Gasparri, Mario G / Meade, Arturo A / Hergott, Christopher A / Reddy, Chakravarthy / Mularski, Richard A / Case, Amy Hajari / Makani, Samir S / Shepherd, Ray W / Chen, Benson / Holt, Gregory E / Martel, Simon

American journal of respiratory and critical care medicine

2019 Volume 200, Issue 11, Page(s) 1354–1362

Abstract: Rationale: ...

Abstract	Rationale:
MeSH term(s)	Aged ; Bronchi/physiopathology ; Female ; Forced Expiratory Volume ; Humans ; Inhalation ; Lung/physiopathology ; Male ; Prostheses and Implants/adverse effects ; Pulmonary Emphysema/physiopathology ; Pulmonary Emphysema/therapy ; Treatment Outcome
Language	English
Publishing date	2019-08-01
Publishing country	United States
Document type	Journal Article ; Multicenter Study ; Randomized Controlled Trial ; Research Support, Non-U.S. Gov't
ZDB-ID	1180953-x
ISSN	1535-4970 ; 0003-0805 ; 1073-449X
ISSN (online)	1535-4970
ISSN	0003-0805 ; 1073-449X
DOI	10.1164/rccm.201902-0383OC
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