interstitial lung disease: an overview · 2019. 5. 13. · interstitial lungdisease: an overview...
TRANSCRIPT
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Interstitial Lung Disease:An Overview
Justin Oldham, MD MSAssistant Professor of MedicineDirector, Interstitial Lung Disease ProgramUniversity of California at DavisSacramento VA Medical Center
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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
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Objectives
• Provide a framework for how the interstitial lung diseases (ILD) are organized
• Review the key components of the ILD evaluation
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What is interstitial lung disease?
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Inflammatory ILDs
Non-specific Interstitial Pneumonia Organizing Pneumonia
Kligerman et al.Radiographics 2009 Travis et al. AJRCCM 2013
Common in autoimmune ILD
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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
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Inflammatory ILDs
Cellular non-specific Interstitial Pneumonia Organizing Pneumonia
Travis et al. AJRCCM 2013
Usually indicates autoimmune ILD
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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
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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
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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
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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
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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
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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
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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
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• 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
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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
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pulmonary.fibrosis.org
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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
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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
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Appropriate ILD Therapy is Critical
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Oldham et al. Res Med 2016
autoimmune-ILD: steroids and immunosuppressive therapy
probably helpful
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Morisset et al. Chest 2017
Environmental ILD: steroids and immunosuppressive therapy probably helpful
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Raghu et al. NEJM 2012
IPF: steroids and immunosuppressive therapy
harmful
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IPF: anti-fibrotic therapy likely helpful
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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
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Thank You!