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Interstitial Lung Disease: An Overview Justin Oldham, MD MS Assistant Professor of Medicine Director, Interstitial Lung Disease Program University of California at Davis Sacramento VA Medical Center

Interstitial Lung Disease: An Overview · 2019. 5. 13. · Interstitial LungDisease: An Overview Justin Oldham, MD MS Assistant Professor of Medicine Director,Interstitial LungDisease

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Page 1: Interstitial Lung Disease: An Overview · 2019. 5. 13. · Interstitial LungDisease: An Overview Justin Oldham, MD MS Assistant Professor of Medicine Director,Interstitial LungDisease

Interstitial Lung Disease:An Overview

Justin Oldham, MD MSAssistant Professor of MedicineDirector, Interstitial Lung Disease ProgramUniversity of California at DavisSacramento VA Medical Center

Page 2: Interstitial Lung Disease: An Overview · 2019. 5. 13. · Interstitial LungDisease: An Overview Justin Oldham, MD MS Assistant Professor of Medicine Director,Interstitial LungDisease

Disclosures

• Grants to study Genomic Predictors of IPF Outcomes (NIH) and protein markers of non-IPF ILD outcomes (ACCP)

• Grant to study early ILD detection• UC-Davis Gordon Wong endowment

• IPF Consulting/Speaking• Genentech

• Boehringer Ingelheim

Page 3: Interstitial Lung Disease: An Overview · 2019. 5. 13. · Interstitial LungDisease: An Overview Justin Oldham, MD MS Assistant Professor of Medicine Director,Interstitial LungDisease

Objectives

• Provide a framework for how the interstitial lung diseases (ILD) are organized

• Review the key components of the ILD evaluation

Page 4: Interstitial Lung Disease: An Overview · 2019. 5. 13. · Interstitial LungDisease: An Overview Justin Oldham, MD MS Assistant Professor of Medicine Director,Interstitial LungDisease

What is interstitial lung disease?

Page 5: Interstitial Lung Disease: An Overview · 2019. 5. 13. · Interstitial LungDisease: An Overview Justin Oldham, MD MS Assistant Professor of Medicine Director,Interstitial LungDisease

Inflammatory ILDs

Non-specific Interstitial Pneumonia Organizing Pneumonia

Kligerman et al.Radiographics 2009 Travis et al. AJRCCM 2013

Common in autoimmune ILD

Page 6: Interstitial Lung Disease: An Overview · 2019. 5. 13. · Interstitial LungDisease: An Overview Justin Oldham, MD MS Assistant Professor of Medicine Director,Interstitial LungDisease

Fibrotic ILDs

Peripheral fibrosis (common in idiopathic pulmonary fibrosis)

Central fibrosis (common in chronic hypersensitivity pneumonitis)

Oldham et al. Res Med 2014Selman et al. AJRCCM 2012

Page 7: Interstitial Lung Disease: An Overview · 2019. 5. 13. · Interstitial LungDisease: An Overview Justin Oldham, MD MS Assistant Professor of Medicine Director,Interstitial LungDisease

Inflammatory ILDs

Cellular non-specific Interstitial Pneumonia Organizing Pneumonia

Travis et al. AJRCCM 2013

Usually indicates autoimmune ILD

Page 8: Interstitial Lung Disease: An Overview · 2019. 5. 13. · Interstitial LungDisease: An Overview Justin Oldham, MD MS Assistant Professor of Medicine Director,Interstitial LungDisease

Fibrotic ILDs

Usual Interstitial Pneumonia (usually indicates IPF)

Oldham et al. Res Med 2014Selman et al. AJRCCM 2012

Airway-centric fibrosis (usually indicates chronic hypersensitivity pneumonitis

Page 9: Interstitial Lung Disease: An Overview · 2019. 5. 13. · Interstitial LungDisease: An Overview Justin Oldham, MD MS Assistant Professor of Medicine Director,Interstitial LungDisease

What types of ILD we see on a regular basisInterstitial Lung Disease

Etiology Known Etiology Unknown (aka idiopathic) Unclassifiable

Autoimmune disease- RA, SSc, Sjogrens, IIM

Environmental ILD- Hypersensitivity pneumonitis

Occupational ILD- Asbestosis/Silicosis

Drug-induced ILD- Amio/MTX/Chemo

Smoking-related- Desquamative interstitial pneumonia- Respiratory bronchiolitis-ILD

Chronic Fibrosing- Idiopathic pulmonary fibrosis- Idiopathic NSIP

None of the above

Page 10: Interstitial Lung Disease: An Overview · 2019. 5. 13. · Interstitial LungDisease: An Overview Justin Oldham, MD MS Assistant Professor of Medicine Director,Interstitial LungDisease

Fibrosis

Inflammation

Interstitial Lung Disease

Inflammatory Predominant ILDsAutoimmune ILD (most)

Hypersensitivity Pneumonitis (early)Cryptogenic Organizing Pneumonia

Drug-induced ILD

Fibrotic Predominant ILDsUIP due to Scleroderma or RA

Hypersensitivity pneumonitis (late)Idiopathic pulmonary fibrosis

Asbestosis

Page 11: Interstitial Lung Disease: An Overview · 2019. 5. 13. · Interstitial LungDisease: An Overview Justin Oldham, MD MS Assistant Professor of Medicine Director,Interstitial LungDisease

The ILD Evaluation

Goals

• Standardized work-up to improve diagnostic accuracy

• Avoid unnecessary lung biopsy

• Diagnose early in the disease course

• Treat the disease early to improve outcomes

Page 12: Interstitial Lung Disease: An Overview · 2019. 5. 13. · Interstitial LungDisease: An Overview Justin Oldham, MD MS Assistant Professor of Medicine Director,Interstitial LungDisease

The ILD Evaluation

Family history of ILD? – familial IPF

Environmental history (Birds, mold)? - HP

New medication? - Chemo/Amio/MTX

History

Smoking history? – smoking-ILDs

Joint pain/swelling, rash, muscle weakness, skin tightening, dysphagia? - CTD-ILD

Job exposures? - asbestosis, silicosis

Laboratory work-up - Autoimmune serologies

High-resolution CT Scan

Physical Exam- Autoimmune features?- Crackles? Location?

Early graying, bone marrow abnormality, liver disease? – short telomere-related ILD

Page 13: Interstitial Lung Disease: An Overview · 2019. 5. 13. · Interstitial LungDisease: An Overview Justin Oldham, MD MS Assistant Professor of Medicine Director,Interstitial LungDisease

The ILD Evaluation

History unrevealingPhysical exam non-specific

Laboratory work-up negativeChest CT scan non-diagnostic

Unclassifiable ILD

Surgical Lung Biopsy- Must have sufficient lung function- Largely safe, but small and finite risk of death and

exacerbation

Page 14: Interstitial Lung Disease: An Overview · 2019. 5. 13. · Interstitial LungDisease: An Overview Justin Oldham, MD MS Assistant Professor of Medicine Director,Interstitial LungDisease

The ILD Evaluation - PFT

• Helps characterize physiology

– Forced vital capacity (FVC)

– Diffusion capacity (DLCO)

• Can assist with prognostication

– Baseline values

– Longitudinal change over time

Ley et al. Ann Int Med 2012

Page 15: Interstitial Lung Disease: An Overview · 2019. 5. 13. · Interstitial LungDisease: An Overview Justin Oldham, MD MS Assistant Professor of Medicine Director,Interstitial LungDisease

• Generally of limited use with a few notable exceptions• Hypersensitivity pneumonitis – cellular analysis

• Asbestosis – cellular analysis, biopsy

• Drug toxicity – cellular analysis, biopsy

• Potential emerging utility in bronchoscopic biopsy to diagnose IPF

The ILD Evaluation - Bronchoscopy

Page 16: Interstitial Lung Disease: An Overview · 2019. 5. 13. · Interstitial LungDisease: An Overview Justin Oldham, MD MS Assistant Professor of Medicine Director,Interstitial LungDisease

Travis et al. AJRCCM 2013

The ILD Evaluation – Multi-disciplinary Discussion

MDDPulmonologist

Chest Radiologist Pulmonary pathologist

Consider ILD center referral for all patients with ILD

Page 17: Interstitial Lung Disease: An Overview · 2019. 5. 13. · Interstitial LungDisease: An Overview Justin Oldham, MD MS Assistant Professor of Medicine Director,Interstitial LungDisease

pulmonary.fibrosis.org

Page 18: Interstitial Lung Disease: An Overview · 2019. 5. 13. · Interstitial LungDisease: An Overview Justin Oldham, MD MS Assistant Professor of Medicine Director,Interstitial LungDisease

The ILD Center Experience

• Standardized evaluation

• Multi-disciplinary discussion

• Co-morbidity assessment and treatment

• Pulmonary rehabilitation referral

• Assessment for supplemental oxygen needs

• ILD support group

• ILD therapeutics

• Clinical trials

Page 19: Interstitial Lung Disease: An Overview · 2019. 5. 13. · Interstitial LungDisease: An Overview Justin Oldham, MD MS Assistant Professor of Medicine Director,Interstitial LungDisease

An accurate diagnosis is critical

Oldham et al. ERJ 2016

CTD-ILD

IPF

>50% of patients diagnosed with autoimmune ILD will live >10 years

<50% of patients diagnosed with IPF will live >5 years

Page 20: Interstitial Lung Disease: An Overview · 2019. 5. 13. · Interstitial LungDisease: An Overview Justin Oldham, MD MS Assistant Professor of Medicine Director,Interstitial LungDisease

Appropriate ILD Therapy is Critical

Page 21: Interstitial Lung Disease: An Overview · 2019. 5. 13. · Interstitial LungDisease: An Overview Justin Oldham, MD MS Assistant Professor of Medicine Director,Interstitial LungDisease

Oldham et al. Res Med 2016

autoimmune-ILD: steroids and immunosuppressive therapy

probably helpful

Page 22: Interstitial Lung Disease: An Overview · 2019. 5. 13. · Interstitial LungDisease: An Overview Justin Oldham, MD MS Assistant Professor of Medicine Director,Interstitial LungDisease

Morisset et al. Chest 2017

Environmental ILD: steroids and immunosuppressive therapy probably helpful

Page 23: Interstitial Lung Disease: An Overview · 2019. 5. 13. · Interstitial LungDisease: An Overview Justin Oldham, MD MS Assistant Professor of Medicine Director,Interstitial LungDisease

Raghu et al. NEJM 2012

IPF: steroids and immunosuppressive therapy

harmful

Page 24: Interstitial Lung Disease: An Overview · 2019. 5. 13. · Interstitial LungDisease: An Overview Justin Oldham, MD MS Assistant Professor of Medicine Director,Interstitial LungDisease

IPF: anti-fibrotic therapy likely helpful

Page 25: Interstitial Lung Disease: An Overview · 2019. 5. 13. · Interstitial LungDisease: An Overview Justin Oldham, MD MS Assistant Professor of Medicine Director,Interstitial LungDisease

Summary

• ILD subtypes progress at highly variable rates

• You will help some ILD subtypes by prescribing steroids/immunosuppression

• You will hurt some ILD subtypes by prescribing steroids/immunosuppression

• An early and accurate diagnosis is critical

Page 26: Interstitial Lung Disease: An Overview · 2019. 5. 13. · Interstitial LungDisease: An Overview Justin Oldham, MD MS Assistant Professor of Medicine Director,Interstitial LungDisease

Thank You!