SLIDE 1
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
SLIDE 2 CSIM Annual Meeting 2019
Aaron LeBlanc: Approach to ILD – October 4th, 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
- f 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
SLIDE 3 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
GSK
Attended a COPD expert network
Speakers bureau member Payment from a commercial (including gifts or other consideration ‘in kind’ compensation)
GSK, AZ
Compensation for other learning activities (OLAs) for
Grant(s) or an honorarium Patent for a product referred to or marketed by a commercial Investments in a pharmaceutical
- rganization, medical devices
- r communications firm.
Participating or participated in a trial
SLIDE 4
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
CSIM Annual Meeting 2019
SLIDE 5 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
SLIDE 6 QUICK SNAPPER 2:
- What percentage of patients with ILD remain unclassifiable?
A. 20% B. 30% C. 40% D. 50%
SLIDE 7 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
SLIDE 8 CRACKLING CASES
- 80M - Inpatient Consult for SOBOE
- 76F – Outpatient Consult for CT abnormalities
- 58M – Outpatient Consult for recurrent respiratory infections
SLIDE 9
CASE #1
Case courtesy of Dr Yi-Jin Kuok, Radiopaedia.org, rID: 17341
SLIDE 10 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?
SLIDE 11
CASE #2
Case courtesy of Dr Mohammad Taghi Niknejad, Radiopaedia.org, rID: 61075
SLIDE 12 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?
SLIDE 13
CASE #3
Case courtesy of Melbourne Uni Radiology Masters, Radiopaedia.org, rID: 38919
SLIDE 14 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
SLIDE 15
DIFFERENTIAL DIAGNOSIS
ILD Fibrosis UIP IPF
SLIDE 16
DIFFERENTIAL - ETIOLOGY
Idiopathic Pneumoconiosis HP Smoking CTD Drugs Misc IPF iNSIP Asbestosis Silicosis CWP Mold Birds RB-ILD DIP LCH RA Scleroderma DM Lupus Sjogren’s MCTD IPAF MTX Amio Nitrofuratoin Sarcoidosis
SLIDE 17
DRUG-INDUCED ILD
www.pneumotox.com
SLIDE 18
DIFFERENTIAL - IMAGING
Upper Zone Predominance Lower Zone Predominance HP Sarcoidosis RB-ILD Silicosis Ankylosis Spondylitis IPF iNSIP Asbestosis CTD
SLIDE 19 DIAGNOSIS - IPF
- CT Findings:
- Supleural, Basal Predominance
- Honeycombing +/- Traction Bronchiectasis
SLIDE 20
SLIDE 21 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
SLIDE 22 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
SLIDE 23 THE CASE CONTINUES TO CRACKLE
- What is your diagnosis?
- What is your management plan at this time?
SLIDE 24 IPF TREATMENT
- Comprehensive care
- Anti-fibrotic therapy
SLIDE 25 COMPREHENSIVE CARE
- Pulmonary Rehabilitation
- Consider oxygen therapy
- Lung Transplantation Referral
- Vaccinations
- Reflux Management
SLIDE 26 IPF TREATMENT
Drug Nintedanib Pirfenidone Patients studied Age: 67 FVC: 80% DLCO: 47% Age: 68 FVC: 68% DLCO: 44% Dosing 150 mg po BID 801 mg po TID Change in baseline FVC (mL) -95.1 vs. -205 (p < 0.001)
- 95.3 vs. -205 (p < 0.001)
- 235 vs. -428 (p < 0.001)
Significant Decline in FVC (Greater than 10%) 29.4% vs 43.1% (p < 0.001) 30.4% vs. 36.1 % (p = 0.18) 16.5% v. 31.8% (p < 0.001) Adverse events Diarrhea, Nausea Weight Loss LFT Elevation GI upset Rash LFT elevation Richeldi et al., NEJM 2014 King et al., NEJM 2014
SLIDE 27
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
SLIDE 28 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
SLIDE 29
Case courtesy of Dr Wayland Wang, Radiopaedia.org, rID: 50749
SLIDE 30 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?
SLIDE 31
ACUTE EXACERBATION
SLIDE 32 ACUTE EXACERBATION
- Therapy – unclear
- Personal recommendations:
- Treat any identified underlying causes
- Continue anti-fibrotic therapy
- Consider moderate dose steroids
SLIDE 33 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
SLIDE 34
FUTURE DIRECTIONS
Flaherty et al., NEJM 2019
SLIDE 35
QUESTIONS?
