Canadian Society of Internal Medicine Annual Meeting 2019 Halifax, - PowerPoint PPT Presentation

Canadian Society of Internal Medicine Annual Meeting 2019 Halifax, NS Snap, Crackle, Pop: An Approach to ILD Aaron LeBlanc Respirology and Internal Medicine Dartmouth General Hospital Assistant Professor Dalhousie University

CSIM Annual Meeting 2019 The following presentation represents the views of the speaker at the time of the presentation. This information is meant for educational purposes, and should not replace other sources of information or your medical judgment. Learning Objectives: • Review the diagnosis and differential diagnosis of interstitial lung disease • Determine treatment strategies for acute flares • Recite updates on new therapies and their complications Aaron LeBlanc: Approach to ILD – October 4 th , 2019

CSIM Annual Meeting 2019 Conflict Disclosures Definition: A Conflict of Interest may occur in situations where the personal and professional interests of individuals may have actual, potential or apparent influence over their judgment and actions. “I have the following conflicts to declare” Company/Organization Details Advisory Board or equivalent Attended a COPD expert network GSK Speakers bureau member Payment from a commercial Compensation for other learning GSK, AZ (including gifts or other consideration activities (OLAs) for ‘ in kind ’ compensation) Grant(s) or an honorarium Patent for a product referred to or marketed by a commercial Investments in a pharmaceutical organization, medical devices or communications firm. Participating or participated in a trial

CSIM Annual Meeting 2019 Some of the drugs, devices, or treatment modalities mentioned in this presentation are: Nintendanib Pirfenidone I intend to make therapeutic recommendations for medications that have not received regulatory approval. Not applicable

QUICK SNAPPER 1 • The gold standard for diagnosis in Interstitial lung disease is: A. High-Resolution CT Scan B. Multi-Disciplinary Discussion C. Open Lung Biopsy D. Trans-bronchial cryobiopsy

QUICK SNAPPER 2: • What percentage of patients with ILD remain unclassifiable? A. 20% B. 30% C. 40% D. 50%

QUICK SNAPPER 3: • In the definition of an acute exacerbation of IPF, what is the maximum time of deterioration allowed prior to the diagnosis? A. 1 week B. 2 weeks C. 1 month D. 2 months

CRACKLING CASES • 80M - Inpatient Consult for SOBOE • 76F – Outpatient Consult for CT abnormalities 58M – Outpatient Consult for recurrent respiratory infections •

CASE #1 Case courtesy of Dr Yi-Jin Kuok, Radiopaedia.org, rID: 17341

CASE #1 • What pattern of ILD do the CT findings represent? • What are the three MOST LIKELY causes for this patient’s ILD? • What additional tests might you order? • Should this patient be referred for a lung biopsy?

CASE #2 Case courtesy of Dr Mohammad Taghi Niknejad, Radiopaedia.org, rID: 61075

CASE #2 • What TWO main CT patterns could the CT findings represent? • What are the THREE MOST likely causes for this patient’s ILD? • What additional tests might you order? • Should this patient be referred for a lung biopsy?

CASE #3 Case courtesy of Melbourne Uni Radiology Masters, Radiopaedia.org, rID: 38919

CASE #3 • Based on the CT findings, what are the THREE MOST likely causes? • What is the MOST LIKELY cause? • What is the MOST important step in management? • What should be the recommended initial route for biopsy? • List three pulmonary disease that can cause a mixed pattern of obstruction and restriction: • COPD or asthma + ILD • Hypersensitivity Pneumonitis • Sarcoidosis • Organizing Pneumonia

DIFFERENTIAL DIAGNOSIS ILD Fibrosis UIP IPF

DIFFERENTIAL - ETIOLOGY Idiopathic Pneumoconiosis HP Smoking CTD Drugs Misc IPF Asbestosis Mold RB-ILD RA MTX Sarcoidosis iNSIP Silicosis Birds DIP Scleroderma Amio CWP LCH DM Nitrofuratoin Lupus Sjogren’s MCTD IPAF

DRUG-INDUCED ILD www.pneumotox.com

DIFFERENTIAL - IMAGING Upper Zone Predominance Lower Zone Predominance HP IPF Sarcoidosis iNSIP RB-ILD Asbestosis Silicosis CTD Ankylosis Spondylitis

DIAGNOSIS - IPF • CT Findings: • Supleural, Basal Predominance • Honeycombing +/- Traction Bronchiectasis

MULTIDISCIPLINARY DISCUSSION (MDD) • Considered gold standard in the diagnosis of Interstitial Lung Disease • With MDD: • Significant reduction in unclassifiable ILD • Significant number of cases with a change in diagnosis • Cases with greatest change: • Idiopathic Pulmonary Fibrosis • Hypersensitivity Pneumonitis • Connective-tissue disease (CTD) related ILD Burge et al., Thorax , 2017 Chaudhuri et al., J. Clin Med , 2016 Jo et al., Respirology , 2016

THE CASE CONTINUES TO CRACKLE: • Repeat CT: • Subpleural and lower-lobe predominant reticular changes • Resolution of previously noted ground glass changes • Traction bronchiectasis and honeycombing similar to previous Case courtesy of Dr Yi-Jin Kuok, Radiopaedia.org, rID: 17341

THE CASE CONTINUES TO CRACKLE • What is your diagnosis? • What is your management plan at this time?

IPF TREATMENT • Comprehensive care • Anti-fibrotic therapy

COMPREHENSIVE CARE • Pulmonary Rehabilitation • Consider oxygen therapy • Lung Transplantation Referral • Vaccinations • Reflux Management

IPF TREATMENT Drug Nintedanib Pirfenidone Patients studied Age: 67 Age: 68 FVC: 80% FVC: 68% DLCO: 47% DLCO: 44% Dosing 150 mg po BID 801 mg po TID Change in baseline FVC (mL) -95.1 vs. -205 (p < 0.001) -235 vs. -428 (p < 0.001) -95.3 vs. -205 (p < 0.001) Significant Decline in FVC 29.4% vs 43.1% (p < 0.001) 16.5% v. 31.8% (p < 0.001) (Greater than 10%) 30.4% vs. 36.1 % (p = 0.18) Adverse events Diarrhea, Nausea GI upset Weight Loss Rash LFT Elevation LFT elevation Richeldi et al., NEJM 2014 King et al., NEJM 2014

SIDE EFFECTS Side Effect Management Liver Enzyme Elevation Monitor liver enzymes monthly x 6 months, then every 3 months after Decrease/discontinue drug if elevation occurs Rash Sun protective measures Decrease/discontinue drug if rash occurs GI Symptoms and/or Weight Loss Loperamide for diarrhea Decrease/Discontinue drug if symptoms occur

THE CASE POPS • 3 months later: • Presents to ED with 2 weeks of increased SOB, cough and new sputum production • No hemoptysis • Leg edema, but no orthopnea or PND • Mild respiratory distress • Pulse: 110 O2: 87% room air • Lungs: crackles throughout most lung fields on auscultation

Case courtesy of Dr Wayland Wang, Radiopaedia.org, rID: 50749

THE CASE POPS • Is this an acute exacerbation of IPF? • What further investigations would you like to order? • How will you manage this patient? • What will you do with her anti-fibrotic therapy?

ACUTE EXACERBATION

ACUTE EXACERBATION Therapy – unclear • • Personal recommendations: • Treat any identified underlying causes • Continue anti-fibrotic therapy • Consider moderate dose steroids

CONCLUSIONS • ILDs can be classified by clinical and radiographic criteria • MDD is the gold standard for diagnosis of ILD • IPF has specific anti-fibrotic therapy • Patients should be monitored for GI, hepatic and cutaneous side effects • Definition of exacerbation has been clarified – specific therapy is unclear

FUTURE DIRECTIONS Flaherty et al., NEJM 2019

QUESTIONS?