Our Alliance
Mission of the ILD Alliance
To unite, empower, and support individuals and organizations in advancing awareness, research, and care for those impacted by Interstitial Lung Disease.
Connect to ILD experts in your region
Partner with trusted Mash experts in your region to unlock your potential and achieve your goals.
ILD EXPERTS
Corinne Young
MSN, FNP-C, FCCP
Corinne is a Nurse Practitioner who began working in pulmonary disease in California in 2005, and since 2011, she has worked in a private pulmonary practice in Colorado Springs, Colorado. Corinne is currently one of 10 NPs nationwide to become a Fellow of the American College of Chest Physicians. Involvement and the representation of advanced practice providers in the pulmonary world has been one of her priorities, so that Corinne founded and is the President of the Association of Pulmonary Advanced Practice Providers (APAPP). She also works closely with the American College of Chest Physicians’ CHEST programs and serves on the Interprofessional Team Network, Clinical Research Network, and on the Executive Programing Committee. Additionally, Corinne serves on the American Board of Internal Medicine Pulmonary Disease Board.
Mary DeCardenas
MPAS, PA-C
Mary DeCardenas, MPAS, PA-C, is a board-certified Physician Assistant in the Department of Internal Medicine - Pulmonary Division at UT Southwestern Medical Center, specializing in Interstitial Lung Disease. Ms. DeCardenas completed her undergraduate degree in Psychology and English literature from the University of Texas. She then earned a Master of Physician Assistant Studies from UT Southwestern in 2003 and completed the PA Fellowship in Rheumatology at UT Southwestern and the North Texas VA Medical Center in 2005. After years of practice in Rheumatology, Ms DeCardenas joined the Pulmonary Division in 2022.
Erica Ludtke
NP
Erica A. Ludtke, NP, is a Nurse Practitioner specializing in gerontology at Vanderbilt University Medical Center in Nashville, Tennessee. She earned her Master of Science in Nursing from Vanderbilt University School of Medicine in 2020 and is board-certified as an Adult-Gerontology Primary Care Nurse Practitioner by the American Association of Nurse Practitioners. At Vanderbilt's Interstitial Lung Disease Center, she provides comprehensive care for patients with pulmonary conditions, emphasizing personalized treatment plans and patient education.
Michelle Vega
MSN, CRNP, FNP-BC
Michelle Vega-Olivo, MSN, CRNP, FNP-BC, is a Nurse Practitioner at the Temple Lung Center in Philadelphia, Pennsylvania. She specializes in advanced lung diseases, including interstitial lung disease and idiopathic pulmonary fibrosis. Michelle earned her Master of Science in Nursing from Temple University and is a board-certified Family Nurse Practitioner.
Shannon Benesh
NP
Shannon Benesh, NP, is a dedicated Nurse Practitioner at Columbia University, New York. She specializes in delivering patient-centered care and applies her clinical expertise to support optimal health outcomes, emphasizing education and comprehensive treatment strategies.
Jennifer Fitzgibons
MSN, APN
Jennifer FitzGibbons is an advanced practice nurse with over 20 years of experience in various patient care settings, and has coordinated numerous clinical trials in neurology, oncology, and Women’s Health. She received her training at the University of Pennsylvania and Yale University, and she is certified by the American Nurses Credentialing Center. Jennifer joined the Tulane Medical School Department of Pulmonology and Critical Care Medicine in 2021, after relocating to New Orleans with her family. She is particularly interested in patient education and wellness, expanding access to advanced clinical research, and improving quality of life of patients with pulmonary fibrosis and other chronic conditions.
Heather Bachert
MSN, NP
Heather D. Bachert, MSN, NP, is a board-certified Adult Health Nurse Practitioner affiliated with Henry Ford Health in Detroit, Michigan. She earned her Master of Science in Nursing from Wayne State University in 2013 and has over 25 years of experience in nursing. Heather specializes in pulmonary care and serves as the support group leader for the Interstitial Lung Disease Support Group at Henry Ford Hospital.
Kelley Brant
NP
Kelley A. Brant is a board-certified family nurse practitioner specializing in pulmonology at Intermountain Medical Center's Schmidt Chest Clinic in Murray, Utah. She earned her Master of Science in Nursing from the University of South Alabama in 2021. Kelley is dedicated to providing comprehensive pulmonary care, focusing on patient education and individualized treatment plans. She is affiliated with Intermountain Medical Center and Riverton Hospital.
Jamie Lederer
CRNP, MSN
Jamie L. Lederer, MSN, CRNP, is a board-certified acute care nurse practitioner specializing in pulmonary medicine at the Harron Lung Center, part of the Perelman Center for Advanced Medicine at the University of Pennsylvania in Philadelphia. She earned her Master of Science in Nursing from the University of Pennsylvania School of Nursing in 2013. Jamie is affiliated with the Hospital of the University of Pennsylvania and is a key member of the Interstitial Lung Disease (ILD) and Sarcoidosis Program. Her clinical expertise includes managing chronic lung diseases, interstitial lung disease, pulmonary fibrosis, and pulmonary sarcoidosis. She is dedicated to providing comprehensive, patient-centered care, utilizing the latest advancements in pulmonary medicine to enhance patient outcomes. Ms. Lederer is also involved in patient education and support, serving as the lead coordinator for the Pulmonary Fibrosis Foundation Care Center at the University of Pennsylvania.
Annalise Calypso
CRNP
Annlise Calypso is a certified registered nurse practitioner specializing in pulmonology at Johns Hopkins Medicine in Baltimore, Maryland. She earned her Master of Science in Nursing from Simmons University in 2017. Annlise is affiliated with Johns Hopkins Hospital and Johns Hopkins Bayview Medical Center, where she provides comprehensive care for patients with respiratory conditions.
Felicia Mackey
MSN, FNP-BC, PCCN, CCRN
Felicia Mackey, MSN, FNP-BC, RN, PCCN, CCRN, is a board-certified Family Nurse Practitioner specializing in pulmonary and sleep medicine at Pulmonary Medicine Associates in Sacramento, California. She earned her Master of Science in Nursing with a Family Nurse Practitioner focus from Sonoma State University, following a Bachelor of Science in Nursing from California State University, Sacramento. Her foundational nursing education includes an Associate Degree in Nursing from Sacramento City College. Felicia holds multiple certifications, including Progressive Care Certified Nurse and Critical Care Registered Nurse, and is a member of the California Association of Nurse Practitioners and the American Association of Critical-Care Nurses. She is dedicated to partnering with patients to achieve their health goals, aiming for long, healthy, and happy lives.
Rebekah Edwards
DNP, FNP-C
Rebekah Edwards, DNP, FNP-C, is a board-certified family nurse practitioner at the Norton Thoracic Institute, St. Joseph’s Hospital and Medical Center in Phoenix, Arizona. She earned her Doctor of Nursing Practice from the University of Arizona College of Medicine in 2014. Rebekah specializes in managing advanced and complex lung diseases, with clinical interests in interstitial lung diseases, alpha-1 antitrypsin deficiency, and lymphangioleiomyomatosis (LAM). She is dedicated to holistic care and enhancing the quality of life for patients with advanced lung conditions. She is a member of the American Academy of Nurse Practitioners, the American Thoracic Society, Sigma Theta Tau (a nursing honor society), and an allied health member of the American College of Chest Physicians. Rebekah is affiliated with St. Joseph's Hospital and Medical Center, Yavapai Regional Medical Center, and Chandler Regional Medical Center.
Lanier O'Hare
CRNP
Lanier O'Hare is a board-certified nurse practitioner specializing in pulmonology at UAB Hospital in Birmingham, Alabama. She earned her Bachelor of Nursing in 1999 and her Doctor of Philosophy in 2022 from the University of Alabama at Birmingham. As an assistant professor in the Division of Pulmonary, Allergy, and Critical Care Medicine, her clinical focus includes interstitial lung disease (ILD), pulmonary hypertension (PH), and sarcoidosis. She is also involved in palliative care for connective tissue disease-associated ILD.
Kathy Lindell
PhD, RN, ATSF, FAAN
Kathleen Lindell is a nurse scientist and educator specializing in pulmonary fibrosis. She is an associate professor at the Medical University of South Carolina (MUSC) College of Nursing. Dr. Lindell is dedicated to advancing pulmonary care through research, education, and interdisciplinary collaboration, focusing on improving outcomes and quality of life for patients with pulmonary diseases.
Katie Rosen
RN, MSN, ANP-C
Katherine "Katie" Rosen, MSN, ANP-C, is a board-certified adult nurse practitioner specializing in pulmonary medicine at National Jewish Health in Denver, Colorado. She earned her Master of Science in Nursing in 2012 and has over 12 years of experience in the field. Katie has a particular interest in interstitial lung diseases (ILD) and leads the pulmonary fibrosis support group at National Jewish Health. She is dedicated to improving the quality of life for her patients through comprehensive care and patient education. In addition to her clinical practice, Katie is affiliated with several hospitals in the Denver area, including National Jewish Health, Saint Joseph Hospital, and the University of Colorado Hospital Authority. She is committed to providing compassionate and personalized care to patients with complex pulmonary conditions.
Kelsey Cramer
MSN, AGACNP-BC, DNP
Kelsey Cramer is a board-certified adult-gerontology acute care nurse practitioner specializing in pulmonary medicine at Froedtert & the Medical College of Wisconsin. She focuses on interstitial lung disease (ILD), pulmonary hypertension (PH), and sarcoidosis. Kelsey earned her MSN and DNP from Rush University and is dedicated to delivering patient-centered care, education, and support for those managing complex lung conditions.
Cori Fratelli
MSN, FNP-C
Cori Fratelli, MSN, FNP-C, is a board-certified family nurse practitioner specializing in pulmonary medicine at National Jewish Health in Denver, Colorado. She earned her Bachelor of Arts in Biochemistry from the University of Colorado Boulder, followed by a Bachelor of Science in Nursing from Regis University. She then completed her Master of Science in Nursing at Georgetown University. Cori's clinical focus includes pulmonary fibrosis, sleep disorders, and other respiratory conditions. She has a unique background in both basic science and clinical research, with past research involving asthma, airway hyperresponsiveness, cystic fibrosis, lung cancer, COPD, and interstitial lung disease. This experience informs her interest in personalized medicine. She is a member of the American Academy of Nurse Practitioners and serves as a guest lecturer in the Family Nurse Practitioner Program at Georgetown University. Cori is dedicated to providing comprehensive, personalized care to her patients, utilizing the latest advancements in pulmonary medicine.
Lori Reed
NP
Lori Reed is a board-certified nurse practitioner specializing in pulmonology and critical care at Piedmont Physicians Pulmonary and Sleep Medicine in Atlanta, GA. She earned her BSN from Georgia Southern University and MSN from Georgia State University. With years of experience in cardiac care, Lori focuses on delivering compassionate, efficient care while educating patients about their conditions. Outside of work, she enjoys supporting the UGA Bulldogs, exercising, and spending time with her family.
Jenny Williams
PA-C
Jenny Williams, PA-C, is a board-certified physician assistant specializing in pulmonary disease and critical care medicine at UCHealth's Interstitial Lung Disease Clinic on the Anschutz Medical Campus in Aurora, Colorado. She earned her Bachelor of Arts in Integrated Physiology and Psychology with an emphasis in Neuroscience from the University of Colorado Boulder, followed by a Master of Science in Physician Assistant Studies from DeSales University. Jenny is dedicated to providing comprehensive care to patients with interstitial lung diseases, utilizing the latest advancements in pulmonary medicine.
Jessica Glennie
MSN, APRN
Jessica Glennie, MSN, APRN, is a board-certified nurse practitioner specializing in pulmonary medicine at the Cleveland Clinic in Cleveland, Ohio. She earned her Master of Science in Nursing from Kent State University in 2014. Jessica's clinical interests include interstitial lung diseases such as idiopathic pulmonary fibrosis, rheumatic lung diseases, chronic hypersensitivity pneumonitis, smoking-related interstitial lung diseases, drug-related interstitial lung disease, and pulmonary alveolar proteinosis. She is dedicated to providing comprehensive care to her patients, utilizing the latest advancements in pulmonary medicine.
ILD Alliance Educational Events
CME EVENT
2nd Annual APAPP National Conference
June 26 @ 12:00 pm - June 28 @ 12:00 pm CST
Join us for the 2nd Annual APAPP National Conference in Nashville Tenessee!..
CME EVENT
6th Annual RhAPP National Conference
September 25 @ 12:00 pm - September 27 @ 12:00 pm MST
Course Overview: Rheumatology Advanced Practice Providers (RhAPP) is an initiative dedicated to developing educational programs, providing professi..
ILD Digital Learning Center
Podcasts
Podcasts
Understanding MAC with Jennifer Faber- Gerling
At December 10, 2024
30:30 m
In this episode, Corinne Young interviews Jennifer Faber- Gerling about mycobacterium avian complex (MAC). They discuss the bacterium itself, the rise in cases, screening and diagnosis, treatment and side effects, reinfection versus reactivation, and symptom management. Jennifer emphasized the importance of close surveillance, collaboration with a multidisciplinary team, and individualized care for patients with MAC.
Idiopathic Shortness of Breath Part 1 Using CPET
At December 10, 2024
26:37 m
National Jewish Health’s NP, Cori Fratelli, shares with us how she and her colleagues utilize CPET and other testing in patients with unexplained shortness of breath.
Idiopathic Shortness of Breath part 2 Dysfunctional Breathing Clinic
At December 10, 2024
29:41 m
Nurse practitioner Cori Fratelli from National Jewish Health discusses breathing pattern disorders, related to unexplained dyspnea. Cori advocates to consider these disorders, discusses treatment methods like breath retraining, inspiratory muscle training, and emphasizes the need for more research and specialized clinics.
Deep Dive Into ILD Therapeutics
At December 18, 2024
37:59 m
Check out this podcast recording from the 2024 APAPP Conference in Vegas!
News
News
2023 ACR/CHEST Guideline: Screening & Monitoring ILD in Systemic Autoimmune Rheumatic Diseases
"2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) Guideline for the Screening and Monitoring of Interstitial Lung Disease in People with Systemic Autoimmune Rheumatic Diseases" Arthritis Rheumatol. 2024 Aug;76(8):1201-1213. doi: 10.1002/art.42860.Epub 2024 Jul 8. Sindhu R Johnson 1, Elana J Bernstein 2, Marcy B Bolster 3, Jonathan H Chung 4, Sonye K Danoff 5, Michael D George 6, Dinesh Khanna 7, Gordon Guyatt 8, Reza D Mirza 8, Rohit Aggarwal 9, Aberdeen Allen Jr 10, Shervin Assassi 11, Lenore Buckley 12, Hassan A Chami 5, Douglas S Corwin 13, Paul F Dellaripa 14, Robyn T Domsic 9, Tracy J Doyle 14, Catherine Marie Falardeau 15, Tracy M Frech 16, Fiona K Gibbons 3, Monique Hinchcliff 12, Cheilonda Johnson 6, Jeffrey P Kanne 17, John S Kim 18, Sian Yik Lim 19, Scott Matson 20, Zsuzsanna H McMahan 5, Samantha J Merck 21, Kiana Nesbitt 22, Mary Beth Scholand 23, Lee Shapiro 24, Christine D Sharkey 17, Ross Summer 25, John Varga 7, Anil Warrier 26, Sandeep K Agarwal 27, Danielle Antin-Ozerkis 12, Bradford Bemiss 28, Vaidehi Chowdhary 12, Jane E Dematte D'Amico 28, Robert Hallowell 3, Alicia M Hinze 29, Patil A Injean 30, Nikhil Jiwrajka 6, Elena K Joerns 31, Joyce S Lee 32, Ashima Makol 29, Gregory C McDermott 14, Jake G Natalini 33, Justin M Oldham 7, Didem Saygin 9, Kimberly Showalter Lakin 34, Namrata Singh 35, Joshua J Solomon 36, Jeffrey A Sparks 14, Marat Turgunbaev 37, Samera Vaseer 38, Amy Turner 37, Stacey Uhl 39, Ilya Ivlev 39 Affiliations • 1 University of Toronto, Schroeder Arthritis Institute, Toronto Western Hospital, Mount Sinai Hospital, Toronto, Ontario, Canada. • 2 Columbia University Vagelos College of Physicians and Surgeons, Columbia University Irving Medical Center, New York City. • 3 Massachusetts General Hospital, Boston. • 4 The University of Chicago Medicine, Chicago, Illinois. • 5 Johns Hopkins University School of Medicine, Baltimore, Maryland. • 6 University of Pennsylvania, Philadelphia. • 7 University of Michigan, Ann Arbor. • 8 McMaster University, Hamilton, Ontario, Canada. • 9 University of Pittsburgh, Pittsburgh, Pennsylvania. • 10 Parlin, New Jersey. • 11 University of Texas Health Science Center at Houston. • 12 Yale School of Medicine, New Haven, Connecticut. • 13 St. Luke's University Health Network, Bethlehem, Pennsylvania. • 14 Brigham and Women's Hospital, Boston, Massachusetts. • 15 Kissimmee, Florida. • 16 Vanderbilt University Medical Center, Nashville, Tennessee. • 17 University of Wisconsin School of Medicine and Public Health, Madison. • 18 University of Virginia School of Medicine, Charlottesville. • 19 Hawaii Pacific Health, Aiea. • 20 University of Kansas Medical Center, Kansas City. • 21 Saint Francis Health System, Tulsa, Oklahoma. • 22 Lansdowne, Pennsylvania. • 23 University of Utah, Salt Lake City. • 24 Albany Medical College, Albany, New York. • 25 Thomas Jefferson University Hospital, Philadelphia, Pennsylvania. • 26 Millennium Physicians, Huntsville, Texas. • 27 Baylor College of Medicine, Houston, Texas. • 28 Northwestern University, Chicago, Illinois. • 29 Mayo Clinic, Rochester, Minnesota. • 30 Cedars-Sinai, Los Angeles, California. • 31 UT Southwestern Medical Center, Dallas, Texas. • 32 University of Colorado Denver, Aurora. • 33 New York University Langone Health, New York City. • 34 Hospital for Special Surgery, Weill Cornell Medicine, New York City. • 35 University of Washington, Seattle. • 36 National Jewish Health, Denver, Colorado. • 37 American College of Rheumatology, Atlanta, Georgia. • 38 University of Oklahoma, Oklahoma City. • 39 ECRI, Center for Clinical Evidence and Guidelines, Plymouth Meeting, Pennsylvania. Abstract Objective: We provide evidence-based recommendations regarding screening for interstitial lung disease (ILD) and the monitoring for ILD progression in people with systemic autoimmune rheumatic diseases (SARDs), specifically rheumatoid arthritis, systemic sclerosis, idiopathic inflammatory myopathies, mixed connective tissue disease, and Sjögren disease. Methods: We developed clinically relevant population, intervention, comparator, and outcomes questions related to screening and monitoring for ILD in patients with SARDs. A systematic literature review was performed, and the available evidence was rated using the Grading of Recommendations, Assessment, Development, and Evaluation methodology. A Voting Panel of interdisciplinary clinician experts and patients achieved consensus on the direction and strength of each recommendation. Results: Fifteen recommendations were developed. For screening people with these SARDs at risk for ILD, we conditionally recommend pulmonary function tests (PFTs) and high-resolution computed tomography of the chest (HRCT chest); conditionally recommend against screening with 6-minute walk test distance (6MWD), chest radiography, ambulatory desaturation testing, or bronchoscopy; and strongly recommend against screening with surgical lung biopsy. We conditionally recommend monitoring ILD with PFTs, HRCT chest, and ambulatory desaturation testing and conditionally recommend against monitoring with 6MWD, chest radiography, or bronchoscopy. We provide guidance on ILD risk factors and suggestions on frequency of testing to evaluate for the development of ILD in people with SARDs. Conclusion: This clinical practice guideline presents the first recommendations endorsed by the American College of Rheumatology and American College of Chest Physicians for the screening and monitoring of ILD in people with SARDs.
Potential of PDE4B Inhibitors for Treating ILD Associated with Autoimmune Diseases
PMID: 39212123 Abstract: Patients with autoimmune disease-related interstitial lung disease may develop pulmonary fibrosis, which may become progressive. Progressive pulmonary fibrosis (PPF) is associated with poor outcomes. Antifibrotic therapies have shown efficacy as treatments for PPF in patients with autoimmune diseases, but new treatments are needed to slow or halt disease progression. Phosphodiesterases (PDEs) are enzymes that mediate the hydrolysis of cyclic adenosine monophosphate (cAMP) and cyclic guanosine monophosphate (cGMP). Pre-clinical data suggest that preferential inhibition of PDE4B has the potential to slow the progression of pulmonary fibrosis by inhibiting inflammatory and fibrotic pathways, with a lower risk of gastrointestinal adverse events than associated with pan-PDE4 inhibitors. Nerandomilast (BI 1015550) is a preferential PDE4 inhibitor that has demonstrated anti-inflammatory and antifibrotic effects in pre-clinical studies. In a phase II trial in patients with idiopathic pulmonary fibrosis, nerandomilast (given alone or on top of background antifibrotic therapy) prevented a decrease in lung function over 12 weeks with an acceptable safety and tolerability profile. The phase III FIBRONEER-ILD trial is evaluating the efficacy and safety of nerandomilast, given alone or on top of nintedanib, in patients with PPF, including PPF associated with autoimmune diseases. In this article, we review the potential of PDE4B inhibition in the treatment of ILD associated with autoimmune diseases, including the pre-clinical and early clinical data available to date.
The Risk of Lung Cancer in RA and RA-Associated Interstitial Lung Disease
Arthritis Rheumatol. 2024 Dec;76(12):1730-1738. doi: 10.1002/art.42961. Epub 2024 Aug 16. Rebecca T Brooks 1, Brent Luedders 2, Austin Wheeler 2, Tate M Johnson 2, Yangyuna Yang 2, Punyasha Roul 2, Apar Kishor Ganti 2, Namrata Singh 3, Brian C Sauer 4, Grant W Cannon 4, Joshua F Baker 5, Ted R Mikuls 2, Bryant R England 2 Affiliations • 1 Mayo Clinic, Rochester, Minnesota. • 2 The Department of Veterans Affairs Nebraska-Western Iowa Health Care System and the University of Nebraska Medical Center, Omaha. • 3 University of Washington, Seattle. • 4 Salt Lake City Department of Veterans Affairs and the University of Utah. • 5 Corporal Michael J. Crescenz Department of Veterans Affairs and the University of Pennsylvania, Philadelphia. Abstract Objective: We aimed to evaluate lung cancer risk in patients with rheumatoid arthritis (RA) and RA-interstitial lung disease (ILD). Methods: We performed a retrospective, matched cohort study of RA and RA-ILD within the Veterans Health Administration (VA) between 2000 and 2019. Patients with RA and RA-ILD were identified with validated administrative-based algorithms, then matched (up to 1:10) on age, gender, and VA enrollment year to individuals without RA. Lung cancers were identified from a VA oncology database and the National Death Index. Conditional Cox regression models assessed lung cancer risk adjusting for race, ethnicity, smoking status, Agent Orange exposure, and comorbidity burden among matched individuals. Several sensitivity analyses were performed. Results: We matched 72,795 patients with RA with 633,937 patients without RA (mean age 63 years; 88% male). Over 4,481,323 patient-years, 17,099 incident lung cancers occurred. RA was independently associated with an increased lung cancer risk (adjusted hazard ratio [aHR] 1.58 [95% confidence interval (CI) 1.52-1.64]), which persisted in never smokers (aHR 1.65 [95% CI 1.22-2.24]) and in those with incident RA (aHR 1.54 [95% CI 1.44-1.65]). Compared to non-RA controls, prevalent RA-ILD (n = 757) was more strongly associated with lung cancer risk (aHR 3.25 [95% CI 2.13-4.95]) than RA without ILD (aHR 1.57 [95% CI 1.51-1.64]). Analyses of both prevalent and incident RA-ILD produced similar results (RA-ILD vs non-RA aHR 2.88 [95% CI 2.45-3.40]). Conclusion: RA was associated with a >50% increased risk of lung cancer, and those with RA-ILD represented a particularly high-risk group with an approximate three-fold increased risk. Increased lung cancer surveillance in RA, and especially RA-ILD, may be a useful strategy for reducing the burden posed by the leading cause of cancer death.
RA-ILD: Update on Prevalence, Risk Factors, Pathogenesis, and Therapy
Curr Rheumatol Rep. 2024 Sep 25. doi: 10.1007/s11926-024-01155-8. Online ahead of print. Daniel I Sullivan 1, Dana P Ascherman 2 Affiliations • 1Division of Pulmonary, Allergy, Critical Care, and Sleep Medicine, Department of Medicine, University of Pittsburgh, UPMC Montefiore Hospital, 3459 Fifth Ave, NW 628, Pittsburgh, PA, 15213, USA. dsulliva@pitt.edu. • 2Division of Rheumatology and Clinical Immunology, Department of Medicine, Abstract Purpose of review: Rheumatoid arthritis is frequently complicated by interstitial lung disease (RA-ILD), an underappreciated contributor to excess morbidity and mortality. The true prevalence of RA-ILD is difficult to define given the variability in diagnostic criteria used. The lack of standardized screening methods, an incomplete understanding of disease pathogenesis, and dearth of validated biomarkers have limited the development of controlled clinical trials for this disease. Recent findings: Numerous studies have focused on clinical, radiographic, genetic, molecular, and/or serologic markers of disease severity as well as risk of disease progression. In addition to defining valuable clinical biomarkers, these studies have provided insights regarding the pathogenesis of RA-ILD and potential therapeutic targets. Additional studies involving immunomodulatory and/or anti-fibrotic agents have assessed new therapeutic options for different stages of RA-ILD. RA-ILD continues to be a major contributor to the increased morbidity and mortality associated with RA. Advancements in our understanding of disease pathogenesis at a molecular level are necessary to drive the development of more targeted therapy.
ANCHOR-RA: Multi-National Cross-Sectional Study on ILD Screening in Rheumatoid Arthritis Patients
Design of ANCHOR-RA: a multi-national cross-sectional study on screening for interstitial lung disease in patients with rheumatoid arthritis BMC Rheumatol. 2024 May 21;8(1):19. doi: 10.1186/s41927-024-00389-4. Jeffrey A Sparks 1, Philippe Dieudé 2, Anna-Maria Hoffmann-Vold 3 4, Gerd R Burmester 5, Simon Lf Walsh 6, Michael Kreuter 7, Christian Stock 8, Steven Sambevski 9, Margarida Alves 9, Paul Emery 10 Affiliations • 1 Division of Rheumatology, Inflammation, and Immunity, Brigham and Women's Hospital, Harvard Medical School, 60 Fenwood Road, #6016U, Boston, MA, 02115, USA. jsparks@bwh.harvard.edu. • 2 Department of Rheumatology, Bichat-Claude Bernard University Hospital, Assistance Publique-Hôpitaux de Paris, INSERM UMR1152, University of Paris, Paris, France. • 3 Department of Rheumatology, Oslo University Hospital, University of Zurich, Oslo, Norway. • 4 Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland. • 5 Department of Rheumatology and Clinical Immunology, Charité - Universitätsmedizin Berlin, Berlin, Germany. • 6 National Heart and Lung Institute, Imperial College London, London, UK. • 7 Center for Pulmonary Medicine, Departments of Pneumology, Critical Care & Sleep Medicine, Mainz University Medical Center and of Pulmonary, Marienhaus Clinic Mainz, Mainz, Germany. • 8 Boehringer Ingelheim Pharma GmbH & Co. KG, Ingelheim am Rhein, Germany. • 9 Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany. • 10 NIHR Leeds Biomedical Research Centre, Institute of Rheumatic and Musculoskeletal Medicine, Leeds Teaching Hospitals NHS Trust and Leeds, University of Leeds, Leeds, UK. Abstract Background: Patients with rheumatoid arthritis (RA) are at risk of developing interstitial lung disease (ILD), which is associated with high mortality. Screening tools based on risk factors are needed to decide which patients with RA should be screened for ILD using high-resolution computed tomography (HRCT). The ANCHOR-RA study is a multi-national cross-sectional study that will develop a multivariable model for prediction of RA-ILD, which can be used to inform screening for RA-ILD in clinical practice. Methods: Investigators will enrol consecutive patients with RA who have ≥ 2 of the following risk factors for RA-ILD: male; current or previous smoker; age ≥ 60 years at RA diagnosis; high-positive rheumatoid factor and/or anti-cyclic citrullinated peptide (titre > 3 x upper limit of normal); presence or history of certain extra-articular manifestations of RA (vasculitis, Felty's syndrome, secondary Sjögren's syndrome, cutaneous rheumatoid nodules, serositis, and/or scleritis/uveitis); high RA disease activity in the prior 12 months. Patients previously identified as having ILD, or who have had a CT scan in the prior 2 years, will not be eligible. Participants will undergo an HRCT scan at their local site, which will be assessed centrally by two expert radiologists. Data will be collected prospectively on demographic and RA-related characteristics, patient-reported outcomes, comorbidities and pulmonary function. The primary outcomes will be the development of a probability score for RA-ILD, based on a multivariable model incorporating potential risk factors commonly assessed in clinical practice, and an estimate of the prevalence of RA-ILD in the study population. It is planned that 1200 participants will be enrolled at approximately 30 sites in the USA, UK, Germany, France, Italy, Spain. Discussion: Data from the ANCHOR-RA study will add to the body of evidence to support recommendations for screening for RA-ILD to improve detection of this important complication of RA and enable early intervention. Trial registration: clinicaltrials.gov NCT05855109 (submission date: 3 May 2023).
Phase III Double-Blind RCT of BI 1015550 in Progressive Pulmonary Fibrosis Patients (FIBRONEER-ILD)
Design of a phase III, double-blind, randomised, placebo-controlled trial of BI 1015550 in patients with progressive pulmonary fibrosis (FIBRONEER-ILD) PMID: 37709661 Abstract: Introduction: Progressive pulmonary fibrosis (PPF) includes any diagnosis of progressive fibrotic interstitial lung disease (ILD) other than idiopathic pulmonary fibrosis (IPF). However, disease progression appears comparable between PPF and IPF, suggesting a similar underlying pathology relating to pulmonary fibrosis. Following positive results in a phase II study in IPF, this phase III study will investigate the efficacy and safety of BI 1015550 in patients with PPF (FIBRONEER-ILD). Methods and analysis: In this phase III, double-blind, placebo-controlled trial, patients are being randomised 1:1:1 to receive BI 1015550 (9 mg or 18 mg) or placebo twice daily over at least 52 weeks, stratified by background nintedanib use. Patients must be diagnosed with pulmonary fibrosis other than IPF that is progressive, based on predefined criteria. Patients must have forced vital capacity (FVC) ≥45% predicted and haemoglobin-corrected diffusing capacity of the lung for carbon monoxide ≥25% predicted. Patients must be receiving nintedanib for at least 12 weeks, or not receiving nintedanib for at least 8 weeks, prior to screening. Patients on stable treatment with permitted immunosuppressives (eg, methotrexate, azathioprine) may continue their treatment throughout the trial. Patients with clinically significant airway obstruction or other pulmonary abnormalities, and those using immunosuppressives that may confound FVC results (cyclophosphamide, tocilizumab, mycophenolate, rituximab) or high-dose steroids will be excluded. The primary endpoint is absolute change from baseline in FVC (mL) at week 52. The key secondary endpoint is time to the first occurrence of any acute ILD exacerbation, hospitalisation for respiratory cause or death, over the duration of the trial. Ethics and dissemination: The trial is being carried out in accordance with the ethical principles of the Declaration of Helsinki, the International Council on Harmonisation Guideline for Good Clinical Practice and other local ethics committees. The study results will be disseminated at scientific congresses and in peer-reviewed publications.
Phase III Double-Blind RCT of BI 1015550 in Idiopathic Pulmonary Fibrosis Patients (FIBRONEER-IPF)
Design of a phase III, double-blind, randomised, placebo-controlled trial of BI 1015550 in patients with idiopathic pulmonary fibrosis (FIBRONEER-IPF) PMID: 37597969 Abstract: IntroductionThere is an unmet need for new treatments for idiopathic pulmonary fibrosis (IPF). The oral preferential phosphodiesterase 4B inhibitor, BI 1015550, prevented a decline in forced vital capacity (FVC) in a phase II study in patients with IPF. This study design describes the subsequent pivotal phase III study of BI 1015550 in patients with IPF (FIBRONEER-IPF). Methods and analysis: In this placebo-controlled, double-blind, phase III trial, patients are being randomised in a 1:1:1 ratio to receive 9 mg or 18 mg of BI 1015550 or placebo two times per day over at least 52 weeks, stratified by use of background antifibrotics (nintedanib/pirfenidone vs neither). The primary endpoint is the absolute change in FVC at week 52. The key secondary endpoint is a composite of time to first acute IPF exacerbation, hospitalisation due to respiratory cause or death over the duration of the trial. Ethics and dissemination: The trial is being carried out in compliance with the ethical principles of the Declaration of Helsinki, in accordance with the International Council on Harmonisation Guideline for Good Clinical Practice and other local ethics committees. The results of the study will be disseminated at scientific congresses and in peer-reviewed publications. Trial registration number: NCT05321069.
Trial of a Preferential Phosphodiesterase 4B Inhibitor for Idiopathic Pulmonary Fibrosis
Abstract: Background: Phosphodiesterase 4 (PDE4) inhibition is associated with antiinflammatory and antifibrotic effects that may be beneficial in patients with idiopathic pulmonary fibrosis. Methods: In this phase 2, double-blind, placebo-controlled trial, we investigated the efficacy and safety of BI 1015550, an oral preferential inhibitor of the PDE4B subtype, in patients with idiopathic pulmonary fibrosis. Patients were randomly assigned in a 2:1 ratio to receive BI 1015550 at a dose of 18 mg twice daily or placebo. The primary end point was the change from baseline in the forced vital capacity (FVC) at 12 weeks, which we analyzed with a Bayesian approach separately according to background nonuse or use of an antifibrotic agent. Results: A total of 147 patients were randomly assigned to receive BI 1015550 or placebo. Among patients without background antifibrotic use, the median change in the FVC was 5.7 ml (95% credible interval, –39.1 to 50.5) in the BI 1015550 group and –81.7 ml (95% credible interval, –133.5 to –44.8) in the placebo group (median difference, 88.4 ml; 95% credible interval, 29.5 to 154.2; probability that BI 1015550 was superior to placebo, 0.998). Among patients with background antifibrotic use, the median change in the FVC was 2.7 ml (95% credible interval, –32.8 to 38.2) in the BI 1015550 group and –59.2 ml (95% credible interval, –111.8 to –17.9) in the placebo group (median difference, 62.4 ml; 95% credible interval, 6.3 to 125.5; probability that BI 1015550 was superior to placebo, 0.986). A mixed model with repeated measures analysis provided results that were consistent with those of the Bayesian analysis. The most frequent adverse event was diarrhea. A total of 13 patients discontinued BI 1015550 treatment owing to adverse events. The percentages of patients with serious adverse events or severe adverse events were similar in the two trial groups. Conclusions: In this placebo-controlled trial, treatment with BI 1015550, either alone or with background use of an antifibrotic agent, prevented a decrease in lung function in patients with idiopathic pulmonary fibrosis. (Funded by Boehringer Ingelheim; 1305-0013 ClinicalTrials.gov number, NCT04419506.) Idiopathic pulmonary fibrosis (IPF) is a progressive, irreversible lung disease with high mortality.1,2 Currently, there are two approved antifibrotic drugs — nintedanib and pirfenidone — that slow, but do not stop, the progression of fibrosis.3-5 Therefore, there is a need for additional treatments that can be used alone or with existing antifibrotic therapies.6 Phosphodiesterase 4 (PDE4) inhibition is associated with antiinflammatory and antifibrotic properties.7,8 Preferential inhibition of the PDE4B subtype may be beneficial in the treatment of IPF because it may harness these properties9,10 and is also associated with a more acceptable safety profile than nonselective PDE4 inhibitors.7,10 IPF is a rare disease, which makes the recruitment of large numbers of patients in early-phase clinical trials a challenge. In this trial, we used Bayesian analysis11,12 to incorporate informative historical data from phase 2–4 clinical trials of nintedanib for the control groups. Consistent decreases in the forced vital capacity (FVC) that have been observed in the placebo groups of these trials3,4,13–16 make such an approach suitable for proof-of-concept studies of new drug candidates for the treatment of IPF. In this multicenter, randomized, double-blind, phase 2 trial, we investigated the efficacy and safety of BI 1015550, an oral preferential inhibitor of PDE4B,17 in patients with IPF according to background nonuse or use of an antifibrotic agent.
Testimonials
"The ILD Alliance of RhAPP & APAPP will provide our growing APP community with valuable resources to better educate and connect with ILD Experts based on your region."
Amanda Mixon
"At the end of the day, it is all about our patients. Having a place where we can interact and learn from other ILD Experts in our community will only make our day-to-day patient care stronger and more effective."
Jessica Glennie
"Thanks to the ILD Alliance, I was able to connect with another APP in my region to discuss a few questions I had."
Daric Mueller
"I listened to the "Deep Dive Into ILD Therapeutics" podcast with Jessica Glennie and Cori Fratelli on my way to work. Podcasts like these help us understand what options we have to best treat our patients."
Felicia Mackey
"Being able to connect with other APPs in your region who are in different specialties is invaluable! I hope there are more alliances like these in the future."
Jeannette Hart
