copyright national jewish health 2020©

Copyright National Jewish Health 2020©

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This educational activity is supported by an independent educational grant from Boehringer

Ingelheim Pharmaceuticals, Inc.


AccreditationAccreditation StatementNational Jewish Health is accredited by the Accreditation Council for Continuing Medical Education (ACCME) to provide continuing medical education for physicians.

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Faculty IntroductionsRebecca C. Keith, MDAssociate Professor Department of MedicineDivision of Pulmonary, Critical Care & Sleep MedicineInterstitial Lung Disease ProgramNational Jewish HealthDenver, CO

Kevin R. Flaherty, MDProfessor of Internal MedicineMichigan Medicine Pulmonary Clinic | TaubmanCenterUniversity of MichiganAnn Arbor, MI

Kevin Brown, MDProfessor and ChairDepartment of MedicineNational Jewish HealthDenver, CO


Disclosures• Kevin Brown, MD discloses grants from NHLBI and the Open Source Imaging Consoritum(OSIC), personal fees from Biogen, Galecto, Third Pole, Galapagos, Boehringer Ingelheim, Theravance, Lifemax, Pliant, Blade Therapeutics, Huitai Biomedicine, Lilly, Dispersol, DevProBiopharma, and Humanetics outside the submitted work; .

• Kevin Flaherty, MD discloses that he is a consultant for Bellerophon, Boehringer Ingelheim, Respivant, and Shionogi.

• Rebecca Keith, MD discloses that she is a speaker for Boehringer Ingelheim and Genetech.• Faculty, Planners and Reviewers:Michael Mohning, MD, Amen Sergew, MD, Andrea Harshman, MHA, CHCP, CMP‐HC, Mandy Comeau, Meghan Brenner, MA and the patient in the video have no relevant financial relationships to report.


Learning Objectives• Define Progressive Fibrosing ILD (PF‐ILD), identify conditions

associated with PF‐ILD, and describe the impact of PF‐ILD on symptoms, pulmonary function, and mortality.

• Describe best practices for the early and accurate diagnosis of PF‐ILD and the follow‐up of patients with ILDs who are at risk for developing PF‐ILD.

• Discuss evidence‐based strategies and emerging therapies for the pharmacologic treatment of PF‐ILD.


Chapter 1: Pathophysiology of chronic fibrosingILDs with progressive phenotype

Kevin Brown, MDProfessor and Chair

Department of MedicineNational Jewish Health

Denver, CO


The Universe of Fibrosing Interstitial Lung Disease


The outcome of HP differs from UIP

Selman et al, Am Rev Respir Dis 1993


UIP pattern fibrosis has the worst prognosis

Bjoraker JA. Am J Resp Crit Care Med, 1998


IPF

Not IPF

IPF


IPFIPF

f‐NSIPf‐NSIP

CTD‐ILDCTD‐ILD

SarcoidSarcoid

ARDSARDSDrug‐inducedDrug‐induced

HypersensitivityPneumonitis

HypersensitivityPneumonitis

UnclassifiableUnclassifiable

OccupationalOccupational


The Process


Not IPF

Identifiable cause/association?

HRCT pattern

Surgical lung biopsy

IPF

UIP pattern

Not UIP

YesNo

Is it IPF?

UIP pattern

Not UIP


The Problems



Is it IPF?


Not IPF


HRCT pattern


IPF

UIP pattern

Not UIP

YesNo

Is it IPF?

UIP pattern

Not UIP


Not IPF


HRCT pattern


IPF

UIP pattern

Not UIP

YesNo

Is it IPF?

UIP pattern

Not UIP

Probable UIPIndeterminate for UIPAlternative diagnosis

UIPProbable UIP

Indeterinnate for UIPAlternative diagnosis


The Importance of Disease Behavior


Survival in IPF and fibrotic NSIP

Jegal et al, Am J Respir Crit Care Med 2005

P=0.007


Baseline Predictors of Survival



Predictors of Survival at 6 Months



AE‐PF Clinical Context and Prognosis

Huie et al, Respirology 2010

Underlying Diagnosis

IPFCTD‐ILDUIP pattern

RAUCTD

NSIP patternUCTDSScDermatomyositis

LIPDrug‐induced ILDUnclassified


The Concept


Academic context

Wells AU Eur Respir J 2018


Scientific context

Wells AU Eur Respir J 2018


Testing the hypothesis


FVC decline was similar in INBUILD and INPULSIS

Brown KK Eur Respir J 2020


FVC decline was similar across subgroups

Brown KK Eur Respir J 2020


IPF

f‐NSIPf‐NSIP

CTD‐ILDCTD‐ILD

Fibrotic HP

Fibrotic HP

SarcoidSarcoid

ARDSARDS

IPAFIPAF

UnclassifiableUnclassifiable

OccupationalOccupational

Drug‐induce

d

Drug‐induce

d


Patient Perspective Video


Chapter 2: Best practices to make an early and accurate diagnosis of ILD with progressive

phenotype and manage over timeRebecca C. Keith, MDAssociate Professor

Department of MedicineDivision of Pulmonary, Critical Care & Sleep Medicine

Interstitial Lung Disease ProgramNational Jewish Health

Denver, CO


What is Interstitial Lung Disease?

Diverse group of disorders (>150) that result inflammation/scarring of the pulmonary parenchyma

Similar presentationProgressive dyspnea and dry coughAbnormal pulmonary physiologyAbnormal CXR and/or HRCT

https://www.pulmonaryfibrosis.org/docs/default‐source/patient‐information‐guides/2017‐pff‐patient‐information‐guide.pdf?sfvrsn=0


Cottin V, Hirani NA, Hotchkin DL, et al. Presentation, diagnosis and clinical course of the spectrum of progressive‐fibrosing interstitial lung diseases. EurRespir Rev 2018; 27: 180076 [https://doi. org/10.1183/16000617.0076‐2018].


When to suspect ILD?

If a patient has exertional dyspnea and/or chronic cough• Perform a physical examination, listen carefully for basilar

crackles• Examine the hands and joints for evidence of autoimmune

disease and/or clubbing• Order PFTs, restrictive pattern• Order a chest radiograph• Perform an informal hallway walk test; ≥ 3% is abnormal

If any of these suggest ILD, order a High Resolution CT

Reynen K, et al. N Engl J Med. 2000;343(17):1235.


I diagnosed ILD!

Now which ILD is it?


IPF Not IPF

Patient has ILD

Ragu G, et. al. Diagnosis of Idiopathic Pulmonary Fibrosis An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Car Med. 2018. Vol 198. 5


Making the diagnosis in ILD requires that you become a detective:

• Full History

• Physical Exam

• Pulmonary physiology

• Radiography

• +/‐ Surgical lung biopsy


CLUES

• “Typical” presentation for IPF• Usual age >50 years of age• Most with subacute dyspnea—insidious onset“I first noticed I was breathless playing with my grandchildren 2 years ago, but I thought I was just out of shape.”

• +/– Dry cough• +/– Fatigue/low stamina

• Identify symptoms pointing to other diagnosis• Rheumatologic illness

• Eyes, Skin, Joints, Muscles, Raynaud’s phenomenon, dry mouth/eyes


Findings Suggestive of Connective Tissue Disease

Rheumatoid Arthritis

Systemic Sclerosis

Sjögrenʼs Syndrome

Dermatomyositis/ Polymyositis

Symmetric arthritis/small joints• Morning stiffness

Subcutaneous nodules

Smoker

Raynaud’s after 40 years of age

Esophageal dysmotility

Skin • Tightening• Red spots

(telangiectasias)

Dry eyes/mouth

New onset dental caries

Proximal muscle weaknessRashes• Heliotrope• Shawl sign • Gottron’s papules• Erythroderma

Rough skin on hands, ie, “mechanic’s hands”


Occupational/Environmental History

• Occupation—asbestos, coal, silica, beryllium• Hobbies (avocational exposures)• Home environment

• Hot tubs (indoor/enclosed)• Basement shower• Swimming pools • Free‐standing humidifiers• Cooling systems (swamp cooler) • Water damage to home• Down comforter/pillow• Birds• Moldy hay


Physical Examination• Physician Exam

• Chest

• Crackles, excursion

• Cardiac

• Murmurs, P2 , RV heave/lift

• Joints

• Skin

• Serologies

• ANA, RF/anti‐CCP,

• Additional serologies depending on the clinical presentation:

• Scl‐70, SSA/SSB, anti‐Jo‐1/myositis panel, RNP, ANCA, D‐dimer, IgG subclasses, anti‐centromere antibodies, anti‐Th/To, DS‐DNA

• In the evaluation of hypersensitivity pneumonitis...

• Serum precipitins have limited sensitivity and specificity.


Physical Examination• Physician Exam

• Chest• Crackles, excursion• Cardiac• Murmurs, P2 , RV heave/lift• Joints• Skin

• Serologies• ANA, RF/anti‐CCP, • Additional serologies depending on the clinical presentation:• Scl‐70, SSA/SSB, anti‐Jo‐1/myositis panel, RNP, ANCA, D‐dimer, IgG subclasses, anti‐centromere

antibodies, anti‐Th/To, DS‐DNA• In the evaluation of hypersensitivity pneumonitis...

• Serum precipitins have limited sensitivity and specificity.


Physical Examination – The Hand


PhysiologyAbnormalities are dependent upon lung compartment affected:

Restrictive ObstructiveMixedIsolated gas exchange abnormalities

Lynch et al. Imaging of Diffuse Lung Disease. 2000

Normal Obstructivepattern

Restrictivepattern

VC

VC

VC

RV

RV

FRC

FRC

FRC


Evaluation of Gas Exchange• ABG/SpO2

• Exercise oximetry• Resting SpO2 might be normal

• 6‐minute walk test• Nocturnal oximetry

“ILD protocol” HRCT • No IV contrast

• Supine and prone

• Inspiratory and expiratory images

• Reconstruction algorithm: 1–1.5 mm thick

Radiologic Evaluation


HRCT UIP Pattern• Subpleural, basal

• Posterior

• Reticular abnormalities

• Honeycombing

• Traction bronchiectasis

• Absence of other features • Peribronchiolar predominance

• Extensive ground glass

• Micronodules

• Cysts

• Mosaic attenuation

• Consolidation

PPV of 90–100% for UIP pattern on pathology


Alveolar Lavage and Pathology

• Bronchoscopy is of limited diagnostic value in patients with IPF but may be helpful in other disorders.• Sarcoidosis• Lymphangitic carcinomatosis• Hypersensitivity Pneumonitis

• Surgical Lung Biopsy• In the absence of a “classic” or probably HRCT UIP pattern• Thoracoscopic, multiple lobes should be considered based on the clinical

context


Idiopathic Pulmonary Fibrosis

• > 50 years old• Men > Women• smoking is a risk factor• Exertional dyspnea• +/‐ Cough or fatigue• NO extrapulmonary features• Presence of UIP pattern on HRCT imaging in those patients without a surgical lung biopsy


Connective Tissue Disease‐ILD

Fischer A and Du Bois R. Lancet 2012

• More common in younger women• All have associated ILD• More common in RA, SSc and PM/DM• All can present with lung disease first• Prognosis generally better than IPF• Most commonly NSIP and UIP


NSIP: Non‐Specific Interstitial Pneumonia

• Subpleural, basal predominance• Subpleural reticular changes• Traction bronchiectasis• Ground‐glass opacities• Absence of honeycombing• Subpleural sparing

Belloli EA, Beckford R, Hadley R, Flaherty KR. Idiopathic non‐specific interstitial pneumonia. Respirology. 2016 Feb;21(2):259‐68. doi: 10.1111/resp.12674. Epub 2015 Nov 13. PMID: 26564810


Hypersensitivity Pneumonitis

• Imaging with:• Lobular areas with decreased

attenuation/mosaic attenuation• Centrilobular nodules• Upper lung zone predominance• Chronic HP looks like UIP

• Relevant Exposure History• Birds, mold, and others

• Bronchoscopy can be helpful• Lymphocytic alveolar lavage• Tbbx

Magee AL, Montner SM, Husain A, Adegunsoye A, Vij R, Chung JH. Imaging of Hypersensitivity Pneumonitis. Radiol Clin North Am. 2016 Nov;54(6):1033‐1046.


Sarcoidosis

• Rare granulomatous disease of unclear etiology

• Genetic predisposition• HLA type

• Can affect multiple organs including the lung

• About 20% of patients can develop fibrotic lung disease

Patterson, Karen C, & Strek, Mary E. (2013). Pulmonary fibrosis in sarcoidosis. Clinical features and outcomes. Annals of the American Thoracic Society, 10(4), 362‐370.


How do you know ILD is becoming progressive?


Difficult to Predict an Individuals Disease Course

Ley B, Collard HR, King TE Jr. Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2011; 183:431.


No Single Criteria for Defining ProgressionINBUILD [9] PIRFENIDONE [10] RELIEF [11] Cottin V, et al [4]

Timeframe 24 months 6 months 6 - 24 months 24 months

Progression criteria

a relative decline in the FVC of at least 10% of the predicted value, or

an absolute decline in %FVC >5%, or

an absolute annualized decline in %FVC ≥5%*

a relative decline in the FVC of at least 10％, or

a relative decline in the FVC of 5% to less than 10% of the predicted value and worsening of respiratory symptoms, or

significant symptomatic worsening

a relative decline in FVC ≥5–<10% and worsening of respiratory symptoms, or

a relative decline in the FVC of 5% to less than 10% of the predicted value and an increased extent of fibrosis on HRCT, or

a relative decline in FVC ≥5–<10% and an increased extent of fibrosis on HRCT, or

worsening of respiratory symptoms and an increased extent of fibrosis

a relative decline in the DLCO of at least 15%

1. Cottin V, Hirani NA, Hotchkin DL, Nambiar AM, Ogura T, Otaola M, Skowasch D, Park JS, Poonyagariyagorn HK, Wuyts W, Wells AU. Presentation, diagnosis and clinicalcourse of the spectrum of progressive-fibrosing interstitial lung diseases. Eur Respir Rev 2018; 27: 180076.2. Flaherty KR, Wells AU, Cottin V, Devaraj A, Walsh SLF, Inoue Y, Richeldi L, Kolb M, Tetzlaff K, Stowasser S, Coeck C, Clerisme-Beaty E, Rosenstock B, Quaresma M,Haeufel T, Goeldner RG, Schlenker-Herceg R, Brown KK; INBUILD Trial Investigators. Nintedanib in Progressive Fibrosing Interstitial Lung Diseases. N Engl J Med. 2019; 381:1718-27.3. Maher TM, Corte TJ, Fischer A, Kreuter M, Lederer DJ, Molina-Molina M, Axmann J, Kirchgaessler KU, Samara K, Gilberg F, Cottin V. Pirfenidone in patients withunclassifiable progressive fibrosing interstitial lung disease: a double-blind, randomised, placebo-controlled, phase 2 trial. Lancet Respir Med 2020; 8: 147-57.4. Jürgen Behr, Petra Neuser, Antje Prasse, Michael Kreuter, Klaus Rabe, Carmen Schade-Brittinger, Jasmin Wagner, Andreas Günther. Exploring Efficacy and Safety of OralPirfenidone for Progressive, non-IPF Lung Fibrosis (RELIEF) - A Randomized, Double-Blind, Placebo-Controlled, Parallel Group, Multi-Center, Phase II Trial. BMC Pulm Med2017; 17: 122.


INBUILD

• A relative decline in the FVC of at least 10% of the predicted value• A relative decline in the FVC of 5% to less than 10% of the predicted value

and either• Worsening of respiratory symptoms or• An increased extent of fibrosis on HRCT

• Worsening of respiratory symptoms and an increased extent of fibrosis on HRCT


PROGRESSIVE FIBROSING ILD – (PF‐ILD)

Consider individualized drivers of disease progression in a given disease state as well as in an individual.


Whiteboard Animation


Key points

• ILDs are a diverse group of disorders that can have similar clinical presentation

• Differentiating the etiology of the ILD is important in determining prognosis and therapy

• Close patient monitoring is key to determining if an ILD is becoming progressive

• Thoughtful consideration of potential drivers of progression in an individual patient or disease process can help guide next therapeutic steps


Chapter 3: Evidence‐based strategies and emerging therapies for the pharmacologic

treatment of PF‐ILDKevin R. Flaherty, MD

Professor of Internal MedicineMichigan Medicine Pulmonary Clinic | Taubman Center

University of MichiganAnn Arbor, MI


PF ‐ ILD

IPF

NSIP

CTD‐ILD

UC

HP

Connective tissue disease‐associated ILD4

•Systemic sclerosis•Rheumatoid arthritis•Polymyositis/ dermatomyositis•Sjögren’s syndrome•Systemic lupus erythematosus•Mixed connective tissue disease•Anti‐synthetase syndrome

Hypersensitivity pneumonitis (HP)5• Delayed allergic reaction• Environmental exposures

• Microbes• Animal/plant proteins• Chemicals

Unclassifiable (UC)2,3• About 10% ‐ 20% of ILD

cases• Reasons for UC

• No biopsy• Overlapping histology• Discrepancy between

imaging, clinical, and histological features

• Uncertain etiology

Non‐specific interstitial pneumonias • 14%‐36% of chronic fibrosing

idiopathic interstitial pneumonia1

1. Traila D et al. J Int Med Res.2018;46(1):448‐456; 2. Leung SC et al. Respirol Case Rep. 2015;3(3):85‐88; 3. Hyldgaard C et al. Respirology. 2017;22(3):494‐500; 4. Castelino FV et al. Arthritis Res Ther. 2010;12(4):213; 5. Riario Sforza GG et al. Clin Mol Allergy. 2017;15:6.


Flaherty KR et al. N Engl J Med 2019;381:1718-27.


INBUILD Study Design


*Randomisation was stratified by HRCT pattern (UIP-like fibrotic pattern or other fibrotic patterns) based on central review.†Visits occurred every 16 weeks until end of treatment. bid, twice daily; UIP, usual interstitial pneumonia.

RScreening

Open‐label nintedanib

(INBUILD‐ON)

Double‐blind

Nintedanib 150 mg bid

Placebo Placebo

Nintedanib 150 mg bid

1:1*

Visit 1 2 3 4 5 6 7 8 9

523624126420Week

PART A PART B†

Primary endpoint assessed


INBUILD Study – Key Inclusion Criteria

• Physician‐diagnosed ILD other than IPF• Features of diffuse fibrosing lung disease (reticular abnormality with traction bronchiectasis, with or without honeycombing) of >10% extent on HRCT performed ≤12 months prior to screening, confirmed by central review

• FVC ≥45% predicted• DLco ≥30%–<80% predicted



INBUILD Study – Criteria for Progression

• Patients were required to meet ≥1 of the following criteria for ILD progression in the 24 months before screening, despite management:

• Relative decline in FVC ≥10% predicted• Relative decline in FVC ≥5–<10% predicted and worsened respiratory symptoms

• Relative decline in FVC ≥5–<10% predicted and increased extent of fibrosis on HRCT

• Worsened respiratory symptoms and increased extent of fibrosis on HRCT



INBUILD Study – Baseline Characteristics


Nintedanib (n=332)

Placebo(n=331)

Age, years, mean (SD) 65.2 (9.7) 66.3 (9.8)

Male, n (%) 179 (53.9) 177 (53.5)

Body mass index, kg/m2, mean (SD) 28.1 (5.1) 28.4 (5.5)

Current or former smoker, n (%) 169 (50.9) 169 (51.1)

Race, n (%)*

White 242 (72.9) 246 (74.3)

Asian 83 (25.0) 80 (24.2)

Black/African‐American 5 (1.5) 5 (1.5)

American Indian/Alaska Native/Native Hawaiian/other Pacific Islander 1 (0.3) 0

UIP‐like fibrotic pattern on HRCT, n (%) 206 (62.0) 206 (62.2)

FVC, % predicted, mean (SD) 68.7 (16.0) 69.3 (15.2)

DLco, % predicted, mean (SD)* 44.4 (11.9) 47.9 (15.0)

K‐BILD questionnaire total score, mean (SD) 52.5 (11.0) 52.3 (9.8)


INBUILD Study – Types of ILD Enrolled


Hypersensitivity pneumonitis

Autoimmune ILDs*

Unclassifiable IIP

Other ILDs†

Idiopathic non‐specific interstitial pneumonia


INBUILD Study – Change in FVC


-80.8

-187.8

-82.9

-211.1

-79.0

-154.2

-250

-200

-150

-100

-50

0

Difference: 107.0 mL/year(95% CI: 65.4, 148.5);

p<0.001Relative reduction: 57%


p<0.001Relative reduction: 61%


nominal p=0.014Relative reduction: 49%

Nintedanib (n=332)

Placebo (n=331)

Nintedanib (n=206)

Placebo (n=206)

Nintedanib (n=126)

Placebo (n=125)

Overall populationUIP‐like fibrotic pattern on HRCT

Other fibrotic patterns on HRCT

Adju

sted

mea

n (S

E) a

nnua

l rat

e of

de

clin

e in

FVC

(mL/

year

)


INBUILD Study – Adverse Events


Nintedanib(n=332)

Placebo(n=331)

Diarrhea 222 (66.9) 79 (23.9)

Nausea 96 (28.9) 31 (9.4)

Bronchitis 41 (12.3) 47 (14.2)

Nasopharyngitis 44 (13.3) 40 (12.1)

Dyspnoea 36 (10.8) 44 (13.3)

Vomiting 61 (18.4) 17 (5.1)

Cough 33 (9.9) 44 (13.3)

Decreased appetite 48 (14.5) 17 (5.1)

Alanine aminotransferase increased 43 (13.0) 12 (3.6)

Progression of ILD 16 (4.8) 39 (11.8)

Weight decreased 41 (12.3) 11 (3.3)

Aspartate aminotransferase increased 38 (11.4) 12 (3.6)


Maher et al. Lancet Resp Med 2019


Phase II Trial of Pirfenidone in Patients with Progressive Fibrosing Unclassifiable ILD

• Inclusion criteria:• Unclassifiable ILD after multi‐disciplinary team discussion• Age 18–85 years• %FVC ≥45%• %DLco ≥30%• >10% fibrosis on HRCT within the previous 12 months• Progressive disease within the previous 6 months

• Absolute decline in FVC > 5% or• Symptomatic worsening not explained by other cause



Phase II Trial of Pirfenidone in Patients with Progressive Fibrosing Unclassifiable ILD


• Primary endpoint: • Change in FVC (mL) measured by daily home spirometry

• Secondary endpoints:• Change in FVC (mL and %), %DLco and 6MWD (measured at site visits)

• Change in UCSD SOBQ score, cough‐VAS score and SGRQ total score

• Progression‐free survival*• Safety:

• TEAEs, withdrawals and discontinuations


Baseline Characteristics


Characteristic*Pirfenidone(n=127)

Placebo(n=126)

Age at screening, years 70.0 (61.0, 76.0) 69.0 (63.0, 74.0)

Male, n (%) 70 (55.1) 69 (54.8)

Historical surgical lung biopsy, n (%) 40 (31.5) 48 (38.1)

%FVC 71.0 (59.0, 87.3) 71.5 (58.0, 88.0)

%DLco 44.6 (36.9, 53.5) 48.0 (38.4, 59.0)

6MWD, m 372.0 (303.0, 487.0) 395.0 (325.0, 472.0)

Dose intensity,† % 94.9 (78.5, 95.3)‡ 95.3 (93.6, 95.5)§


Primary Endpoint: Predicted FVC change from baseline to 24 week by home spirometry


CI, confidence interval.

FVC change from baseline at Week 24, mL

Pirfenidone (n=124)

Placebo (n=123)

Mean (95% CI) –17.9 (–311.7, 275.9)

116.6 (–451.9, 685.2)

Median (Q1, Q3) –87.7 (–338.1, 148.6)

–157.1 (–370.9, 70.1)

Analysis of the primary endpoint was impacted by high intra‐individual variability in home spirometry values and issues applying linear regression to

patients with a small number of readings collected in a short period of time


Secondary Endpoint: Predicted FVC change from baseline to 24 week by site spirometry


FVC change measured at site visits consistently favoured pirfenidone over placebo

Predicted mean (95% CI) change in FVC from baseline to Week 24:

• Pirfenidone: –17.8 (–62.6, 27.0) mL

• Placebo: –113.0 (–152.5, –73.6) mL

• Pirfenidone vs. placebo: 95.3 (35.9, 154.6) mL; P=0.002

• A rank ANCOVA model for %FVC for absolute change from baseline to last observed measurement yielded a P‐value of 0.038

ANCOVA, analysis of covariance.


Adverse Events


Summary of treatment‐related TEAEs known to be associated with pirfenidone, n (%)

Pirfenidone (n=127) Placebo (n=124)

GI disorder 60 (47.2) 32 (25.8)

Photosensitivity 10 (7.9) 2 (1.6)

Rash 13 (10.2) 9 (7.3)

Dizziness 10 (7.9) 4 (3.2)

Weight decrease 10 (7.9) 1 (0.8)

Fatigue 16 (12.6) 12 (9.7)


Patient with ILD

Multi-disciplinary approach to diagnosis

IPF

Discussionabout initiation of

anti-fibrotic medication

Non-IPF ILD

Initial treatment based on diagnosis eg:‐ CTD‐ILD consider immunosuppression‐ HP antigen avoidance

Improved/Stable

Discussion about initiation of anti-fibrotic medicationand/or escalate other treatment(s)

Monitor for response

yesno

Discussion to continue or decrease treatment

Monitor for progression


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