Interstitial Pulmonary Fibrosis A Deadly Disease. Said Chaaban, MD - - PowerPoint PPT Presentation
Interstitial Pulmonary Fibrosis A Deadly Disease. Said Chaaban, MD Assistant Professor of Medicine Pulmonary and Critical Care Medicine University of Kentucky August, 2018 Disclosure This presentation has no: Conflicts of interest
Said Chaaban, MD Assistant Professor of Medicine Pulmonary and Critical Care Medicine University of Kentucky August, 2018
“. . ..some kind of fibrosis. . .. . ..so I just kept going to the clinic and they did lung function. . ..which. . ..at the time. . ..were quite low- . . ..then he decided to refer me to Dr. X’s clinic and then we found out that I was too bad for the trial [Bridget]”
statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med. 2002;165(2):277-304.
Diffuse Parenchymal Lung Disease
DPLD of known cause e.g. drugs or association e.g. collagen vascular disease Idiopathic Interstitial Pneumonias Granulomatous DPLD e.g. sarcoidosis Other forms of DPLD e.g. LAM, HX etc. Idiopathic Pulmonary Fibrosis Desquamative Interstitial Pneumonia IIP other than Idiopathic pulmonary Fibrosis Respiratory bronchiolitis Interstitial lung disease Acute Interstitial Pneumonia Nonspecific interstitial Pneumonia (provisional) Cryptogenic Organizing pneumonia Lymphocytic interstitial Pneumonia
statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med. 2002;165(2):277-304.
Respir Med. 2014;2(7):566-72.
Respir Med. 2014;2(7):566-72.
1. Richeldi L, Collard HR, Jones MG. Idiopathic pulmonary fibrosis. Lancet. 2017 2. Taskar VS, Coultas DB. Is idiopathic pulmonary fibrosis an environmental disease?. Proc Am Thorac Soc. 2006;3(4):293-8. 3. Maher TM, Wells AU, Laurent GJ. Idiopathic pulmonary fibrosis: multiple causes and multiple mechanisms?. Eur Respir J. 2007;30(5):835-9. 4. Mora AL, Rojas M, Pardo A, Selman M. Emerging therapies for idiopathic pulmonary fibrosis, a progressive age-related disease. Nat Rev Drug Discov. 2017;16(11):755-772 5. Ghebre YT, Raghu G. Idiopathic Pulmonary Fibrosis: Novel Concepts of Proton Pump Inhibitors as Antifibrotic Drugs. Am J Respir Crit Care Med. 2016;193(12):1345-52.
“. . .it started with a cough’ ‘. . . ‘I was getting a bit breathless going upstairs’ being typical. From there on, the journey to diagnosis was variable, usually causing anxiety and frustration. Participants described a lengthy delay from being treated in primary care initially for asthma, COPD or recurrent chest infections before being referred to a local chest physician”
probability:
Less common
Health Technol Assess. 2015;19(20):i-xxiv, 1-336.
American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med. 2002;165(2):277-304.
Am J Respir Crit Care Med. 2011;183(6):788-824.
the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med. 2002;165(2):277-304.
recommendation)
antibody titer and pattern
Clinical Practice Guideline. Am J Respir Crit Care Med. 2015;192(2):e3-19.
ILD:
environmental exposures
UIP pattern on HRCT Specific combinations
lung biopsy pattern
Clinical Practice Guideline. Am J Respir Crit Care Med. 2015;192(2):e3-19.
Not IPF
No
1. Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788-824.
UIP Pattern (All Four Features) Subpleural, basal predominance Reticular abnormality Honeycombing with or without traction Bronchiectasis Absence of features listed as inconsistent with UIP pattern
1. Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788-824.
1. Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788-824.
Possible UIP Pattern (All Three Features) Inconsistent with UIP Pattern (Any of the Seven Features) Subpleural, basal predominance Upper or mid-lung predominance Reticular abnormality Peribronchovascular predominance Absence of features listed as inconsistent with UIP pattern Extensive ground glass abnormality (extent > reticular abnormality) Profuse micronodules (bilateral, predominantly upper lobes) Discrete cysts (multiple, bilateral, away from areas of honeycombing) Diffuse mosaic attenuation/air-trapping (bilateral, in three or more lobes) Consolidation in bronchopulmonary segment(s)/lobe(s)
1. Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788-824.
1. Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788-824.
statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med. 2002;165(2):277-304.
(counseling)
1. Hutchinson JP, Fogarty AW, Mckeever TM, Hubbard RB. In-Hospital Mortality after Surgical Lung Biopsy for Interstitial Lung Disease in the United States. 2000 to 2011. Am J Respir Crit Care Med. 2016;193(10):1161-7.
1. Dibardino DM, Haas AR, Lanfranco AR, Litzky LA, Sterman D, Bessich JL. High Complication Rate after Introduction of Transbronchial Cryobiopsy into Clinical Practice at an Academic Medical Center. Ann Am Thorac Soc. 2017;14(6):851-857.
1. Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788-824.
Diagnostic Gold Standard
Pathologists Physicians Radiologists
1. Flaherty KR, King TE, Raghu G, et al. Idiopathic interstitial pneumonia: what is the effect of a multidisciplinary approach to diagnosis?. Am J Respir Crit Care Med. 2004;170(8):904-10.
1. Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788-824.
“. . .its nothing more than stress and anxiety” “I’ve always been active, garden, DIY, you know I’d do anything around the house. I mean there was a little job there yesterday and I had to get Christine to (do it). . ..I sort of direct operations now, but physically do it, No’. [Jim]”
studies suggest a median survival time from 2 to 3 years from the time of diagnosis
1. Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788-824. 2. Richeldi L, Collard HR, Jones MG. Idiopathic pulmonary fibrosis. Lancet. 2017
(on the bike) before I went to work then I used to go out and do the same at night. . . and I’ve rode across the country in 3 days and 216 miles in 12 hours. . ...[Arthur]”
Baseline Factors Longitudinal Factors Level of dyspnea Increase in level of dyspnea DLCO < 40% Decrease in FVC by > 10% Desaturation < 88% during 6MWT Decrease in DLCO > 10% Extent of honey combing on HRCT Worsening fibrosis on HRCT Pulmonary Hypertension >50 m decrease on 6MWT 6MWT <250 m
1. Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788-824. 2. Brown AW, Nathan SD. The Value and Application of the 6-Minute-Walk Test in Idiopathic Pulmonary Fibrosis. Ann Am Thorac Soc. 2018;15(1):3-10.
1. Ley B, Collard HR, King TE. Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011;183(4):431-40.
Respiratory (77%) Non-respiratory (18%) Progression of IPF Cardiac Acute Exacerbations Septic Acute Lung Injury GI Pneumonia Stroke Cor Pulmonale Cancer
1. Ley B, Collard HR, King TE. Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011;183(4):431-40.
Nintedanib Pirfenidone FVC >50% FVC (50-90%) DLCO (30-79 %) DLCO (30-90%) Transaminitis Transaminitis Gastrointestinal (Diarrhea & nausea) Gastrointestinal (Dyspepsia & anorexia) Dermatological (photosensitivity)
“So it’s getting around I think is the big change. . ..actually doing jobs . . .. . .you’ve got to think before you do anything, if you want to. . .. . . just nip into the shops, you’ve gotta think ‘have I enough liquid oxygen? Have I filled the bottle? Have I done this? Have I done that? [Terry]” ‘. . ..when I shave, I put me (my) shaving cream on, I’ve got to take the
cause I can just lie down and use it’. [Frank]
but I can get around. . ..and I don’t seem to cough as much. [Terry]
without resting hypoxaemia. Respirology. 2017;22(5):957-964.
in this together. . ..like going up and down the stairs. . ...and I say to Christine when you come down will you bring me so and so.
care physicians and end-of-life planning should be discussed in the outpatient setting
necessary treatment priority
patient’s family
caregivers
. . ..it’s his only chance really, unless there is something else. . ... I just couldn’t come to terms with it. . ..I was getting panic attacks. . .two or three times a week. . .and used to break down. . .. cry lake a
[Geoffrey]
transplant life expectancy exceeds their current life expectancy without the transplant
pneumonitis(UIP)or fibrosing non-specific interstitial pneumonitis(NSIP),regardless of lung function
1. Weill D, Benden C, Corris PA, et al. A consensus document for the selection of lung transplant candidates: 2014--an update from the Pulmonary Transplantation Council of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant. 2015;34(1):1-15.
ClinicalTrials.gov
2017;16(11):755-772.