Evaluating Patients for Potential ILD

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Interstitial Lung Disease – Insights in ILD

Interstitial Lung Disease Conditions – Insights in ILD

150+ ILDs

Many ILDs share similar symptoms, physiology, and radiologic findings¹

ILD, interstitial lung disease.
1. Raghu G et al. Clin Chest Med. 2004;25(3):409-419.

Signs and Symptoms of ILD – Insights in ILD

Signs and symptoms shared by most ILDs include^2-6:

Dyspnea
Cough
Fatigue
Restrictive pattern on PFTs^a
Diffuse lung infiltration on chest imaging

^aPatients can have other pathologies that could result in mixed or sometimes pure obstructive patterns
PFT, pulmonary function test
1. Raghu G et al. Clin Chest Med 2004;25(3):409-419. 2. Raghu G et al. Am J Respir Crit Care Med 2011;183(6):788-824. 3. Van Manen MJG et al. Eur Respir Rev. 2016;25:278-286. 4. European Respiratory Society. Interstitial Lung Diseases in European Lung White Book. 256-269. 5. Cosgrove G et al.
BMC Pulm Med 2018; 18:9. 6. Nathan S et al. Paed Respir Rev. 2015;16(4):219-224.

Signs and Symptoms Mistaken – Insights in ILD

ILDs may be mistaken for more common disorders, such as^1,2:

Pulmonary
Bronchitis
Pneumonia
COPD
Asthma

Cardiac
CHF
Cardiac Ischemia

Psychogenic Conditions

CHF, congestive heart failure; COPD, chronic obstructive pulmonary disease.
1. Karnani NG et al. Am Fam Physician. 2005;71(8):1529-1537. 2. Cosgrove G et al. BMC Pulm Med 2018; 18:9.

Interstitial Lung Disease Misdiagnosis – Insights in ILD

In one study of patients with ILD, 55% of respondents had at least 1 misdiagnosis and 38% had 2 or more misdiagnoses

75% consulted 3 or more physicians

68% of respondents stated that consulting a specialist with expertise in ILD was the most important factor in obtaining an accurate diagnosis

34% had a delay of 2 or more years in diagnosis

Misdiagnosis affected amount of time spent with friends and family, and some patients reported that it influenced their decision to retire or apply for disability

1. Cosgrove G et al. BMC Pulm Med. 2018; 18:9.

Causes of ILDs – Insights in ILD

Some ILDs are associated with infection, exposure to dust or other particles, or an underlying genetic predisposition¹
The cause may also be unknown (idiopathic interstitial pneumonias)¹

Used with permission from the American Thoracic Society. http://www.thoracic.org/patients/patient-resources/resources/idiopathic-pulmonary-fibrosis.pdf
1. Raghu G et al. Clin Chest Med. 2004;25(3):409-419.

ILDs Are Classified by the Originating Factor – Insights in ILD

ILD^a
Exposure-related
Systemic rheumatic disease-related*
Idiopathic
Sarcoidosis
Other

ILDs often share dyspnea and cough as presenting symptoms, but the underlying associated disease will also produce additional signs and symptoms^2

aAdapted from Ryerson et al. 2013.
*Systemic rheumatic disease is also called connective tissue disease and collagen-vascular disease.
1. Ryerson CJ et al. Curr Opin Pulm Med. 2013;19(5):453-459. 2. Raghu G et al. Clin Chest Med. 2004;25(3):409-419.

Progression and Survival – Insights in ILD

Many ILDs have high rates of morbidity and mortality¹
ILDs are commonly observed in patients of all ages but more frequently affect adults¹
Most unretired patients with ILD are unable to work due to breathlessness upon exertion²
Patients with occupational or exposure-related ILD should avoid further exposure and transfer to another job²

1. Nathan S et al. Paed Respir Rev. 2015;16(4):219-224. 2. Eur Respir Society White Book ILD

Evaluating Patients for Potential ILD – Insights in ILD

Checklist for Evaluating Patients – Insights in ILD

Comprehensive patient history
Comprehensive physical exam
Pulmonary function testing
Laboratory analyses
Imaging
Other modalities (as indicated)

1. Raghu G et al. Clin Chest Med. 2004;25(3):409-419.

A Comprehensive History aids in ILD diagnosis – Insights in ILD

Past Medical History^1,2

Gastroesophageal reflux disease
Cancer, chemotherapy, and/or radiation therapy
Cardiac arrhythmias and amiodarone use
Prostate, urinary, kidney infections, and nitrofurantoin use

1. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824. 2. Lederer DJ. http://www.pfdoc.org/2014/07/a-pulmonary-fibrosis-primer-for-doctors.html. Accessed December 5, 2017. 3. Schwaiblmair M et al. Open Respir Med J. 2012;6:63-74. 4. Camus P. The drug-induced respiratory disease website. 2012. Pneumotox website. www.pneumotox.com. Accessed December 12, 2017. 5. Garcia-Sancho C et al. Respir Med. 2011;105(12):1902-1907.

A Comprehensive History of ILDs – Insights in ILD

Current and Past Chronic Medications^3,4

Cancer, chemotherapy, and/or radiation therapy
Amiodarone use
Nitrofurantoin use

A Comprehensive History of ILDs – Insights in ILD

Exposure History¹

Tobacco use
Drug use
Occupational
Environmental
Avocational

A Comprehensive History of ILDs – Insights in ILD

Family History^1-3

Pulmonary fibrosis
ILD
Sarcoidosis
Autoimmune disease
Oxygen use

Specific Sounds Can Help Determine ILDs – Insights in ILD

Both anterior and posterior auscultation are necessary for detecting sounds of ILDs, particularly posterior basilar auscultation^1,2
Patients may exhibit signs of ILD with concomitant sounds of other pulmonary complications (eg, wheeze)²
Careful auscultation in areas 6 and 7 should be performed when ILD is suspected¹
- Auscultating areas 4 and 5 (anterior) can be more difficult to access on women, but they are also important to examine. These areas may be more accessible when a patient is lying down

1. Lederer DJ. http://www.pfdoc.org/2014/07/a-pulmonary-fibrosis-primer-for-doctors.html. Accessed December 5, 2017.
2. Sarkar M et al. Ann Thorac Med. 2015;10(3):158-68.

Posterior
Anterior
1
2
3
4
5
6
7
2
3
4
5
6
7
0
1

Specific Sounds Can Help Determine ILDs – Insights in ILD

Crackles

Many ILDs exhibit fine, bibasilar crackles^1,2
However, crackles are not specific to ILDs. The timing and location of the crackles can vary, which further distinguishes the specific cause, such as COPD, pneumonia, asbestosis, chronic bronchitis, and pulmonary edema secondary to heart failure¹
Crackles may occur alongside other breath sounds, providing further clues to the potential cause(s) of dyspnea and/or cough^1,3,4

1. Sarkar M et al. Ann Thorac Med. 2015;10(3):158-168. 2.Cottin V et al. Eur Respir J. 2012;40(3):519-521. 3.Cottin V et al. Eur Respir J. 2005;26:586-593. 4. Castelino FV et al. Arthr Res Ther. 2010;12:213.

Undergoing Pulmonary Function Tests – Insights in ILD

Wheezes
Squeaks
Pops
Rhonchi

Sounds heard at initial inspiration and those heard primarily at the tops of the lungs should prompt investigation of causes other than ILDs, although these can also be characteristic of some forms of ILD^1,2
Thorough auscultation of all regions is the best way to ensure that the signs of all potential explanations of dyspnea and/or cough are explored²

1. Kritek P, Choi MK. In: Longo DL, Fauco AS, Kasper DL et al., eds. Harrison’s Principles of Internal Medicine. 18th ed. New York, NY: McGraw-Hill Companies, Inc. 2012:2094-2101. 2. Sarkar M et al. Ann Thorac Med 2015;10(3):158-168.17

PFTs Help Differentiate Respiratory Defects – Insights in ILD

PFTs allow the physician to³:
- Evaluate lung function
- Differentiate amon pulmonary disorders
- Guide initial management strategies
Pulmonary function can be assessed using spirometry parameters such as^1,4,5:
- Lung volumes (FEV₁, FVC)
- Gas exchange (DL_CO)
- Dyspnea on exertion (6MWT)
- A quicker way to assess dyspnea on exertion is to use the hallway or staircase

6MWT, 6-minute walk test; DL_co,diffusing capacity of the lungs for carbon monoxide; FEV₁,forced expiratory volume in 1 minute; FVC, forced vital capacity.
1. Lederer DJ. http://www.pfdoc.org/2014/07/a-pulmonary-fibrosis-primer-for-doctors.html. Accessed November 17,2017. 2. Meyer KC. Trans Respir Med. 2014;2:4. 3. Ranu H et al. Ulster Med J. 2011;80(2):84-90. 4. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824. 5. Ley B et al. Am J Respir Crit Care Med. 2011;183(4):431-440.

PFTs Help Differentiate Pulmonary Disorders – Insights in ILD

Restrictive defect²

TLC is low
Low FVC
Higher FEV₁/FVC ratio
Flows are higher than expected at a given lung volume

Obstructive defect²

TLC is normal
Normal FVC
Lower FEV₁
Lower FEV₁/FVC ratio
Flows are lower than expected over the entire volume range

Restrictive defects are common in ILDs¹

Used with permission from Pellegrino R et al. Eur Respir J. 2005;26(5):948-968.
TLC, total lung capacity.
1. Ranu H et al. Ulster Med J. 2011;80(2):84-90. 2. Pellegrino R et al. Eur Respir J. 2005;26(5):948-968.

Other Investigations for ILDs – Insights in ILD

FVC
FEV₁
FEV₁/FVC
DL_co
TLC

Obtaining complete PFTs is important because it allows determination of the nature of the respiratory defect1

1. Ranu H et al. Ulster Med J. 2011;80(2):84-90. 2. Lin H et al. J Thorac Dis. 2015;7(4):767-779.

Laboratory Workup – Insights in ILD

Laboratory workups
Chest X-ray
HRCT
Lung biopsy
BAL

BAL, bronchoalveolar lavage; HRCT, high resolution computed tomography.
1. Jo HE et al. Respirology. 2016;21(8):1438-1444. 2. Vij R et al. Chest. 2013;143(3):814-824. 3. Meyer KC et al. Eur Respir J. 2011;38:761-769.

Get Chest X-rays to Rule Out Other Causes – Insights in ILD

Basic blood tests (eg, CBC) are generally a first step in evaluating a patient, although many ILDs will not have specific abnormalities¹
Some ILDs – particularly those associated with systemic rheumatic diseases^a – will often exhibit characteristic antibody signatures²

^aSystemic rheumatic diseases have also been called connective tissue disease or collagen-vascular diseases.
CBC, complete blood count.
1. Bradley B et al. Thorax. 2008; 63(suppl V):v1-v58. 2. Vij R et al. Chest. 2013;143(3):814-824.

HRCTs Are Essential to Diagnosing ILDs – Insights in ILD

Chest X-rays cannot solely diagnose most ILDs, but they can help rule out other causes of dyspnea, including heart disease, pneumonia, collapsed lung, emphysema, and lung cancer^1,2
When possible, previous chest X-rays should be included in the evaluation to determine whether the disease process is acute or chronic³

If an X-ray report states ILD, interstitial findings, or reticulation, the patient should be referred to a pulmonologist for an HRCT scan and further workup³

1. National Heart, Lung, and Blood Institute. Chest X Ray. http://www.nhlbi.nih.gov/health/health-topics/topics/cxray/whoneeds. Accessed December 11, 2017. 2. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824. 3. Meyer KC. Trans Respir Med. 2014;2:4.

Patients with Dyspnea Should Have an HRCT – Insights in ILD

HRCT creates high-resolution stacked X-ray slices to visualize internal organs^1,2
HRCT allows physicians to^3,4:
- Differentiate features that help distinguish among various ILDs
- Assess progression of disease and to exclude concomitant processes such as emphysema, edema, or infection

1. FDA Medical X ray Imaging. http://www.fda.gov/RadiationEmittingProducts/RadiationEmittingProductsandProcedures/
MedicalImaging /MedicalX-rays/ucm115318.htm. Accessed December 11, 2017. 2. Verschakelen JA. Curr Opin Pulm Med. 2010;16(5):503-510.
3. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824. 4. Suh RD et al. Semin Respir Crit Care Med. 2006;27(6):623-633.

Lung Biopsies Can Add Additional Information – Insights in ILD

PLACEHOLDER - Faculty to provide image set of X-ray and HRCT

1. Walsh SL et al. Semin Respir Crit Care Med. 2014;35(1):129-144.

Bronchoalveolar Lavage Can Provide Crucial Information – Insights in ILD

Depending on the specific ILD suspected and the outcome of other evaluations, such as serologies and/or HRCT, lung biopsy may be recommended to accurately diagnose the patient’s condition^1,2
Types of lung biopsy procedures^3,4:
- Open lung biopsy
- Video-assisted thoracoscopic surgery
- Cryobiopsy

Patients with suspected acute exacerbations of ILD should not be referred for lung biopsy due to higher risk of mortality^1,5

1. Cottin V. Eur Respir J. 2016;48(5):1274-1277. 2. Lynch DA et al. Lancet Respir Med. 2018;6(20:138-153. 3. Tomassetti S et al. Am J Respir Crit Care Med. 2016;193(7):745-752. 4. Colby TV et al. Arch Pathol Lab Med. 2016;141(7):891-900. 5. Hutchinson JP et al. Am J Respir Crit Care Med. 2016;193(10):1161-1167

Diagnosing ILDs Takes a Diverse Team of HCPs – Insights in ILD

Performing BAL can rule out opportunistic infections and is useful in diagnosing hypersensitivity pneumonitis and sarcoidosis¹
However, BAL results are generally nonspecific (consistent with or suggestive of a given condition) rather than pathognomonic for ILD¹

~500%
The amount smoking increases cell count in BAL^2,3

1. Meyer KC et al. Eur Respir J. 2011;38:761-769. 2. Karimi R et al. PLoS One. 2012;7(3):e34232. 3. BAL Cooperative Group Steering Committee. Am Rev Respir Dis. 1990;141(5 Pt 2):S169-S202.

Early Referral to an ILD Center Can Improve Time – Insights in ILD

PCPs are often the first ones to recognize the initial signs of ILD and/or diseases that have ILD manifestations¹
Referral to a pulmonologist, rheumatologist, or other specialist may be required, depending on the presenting symptoms^1,2
Once pulmonary symptoms occur, patients should be evaluated and potentially referred to an ILD Center of Excellence¹

PCP, primary care physician.
1. Cosgrove G et al. BMC Pulm Med. 2018; 18:9. 2. Lynch DA et al. Lancet Respir Med. 2018;6(20:138-153.

Evaluating Patients for Potential ILD Summary – Insights in ILD

Multidisciplinary discussions are recommended for diagnosing ILDs^1-3
ILD centers have the resources and experience to manage a multidisciplinary approach to diagnosing ILDs^2,3

MDD, multidisciplinary discussion
1. Cosgrove G et al. BMC Pulm Med. 2018; 18:9. 2. Flaherty K et al. Am J Respir Crit Care Med. 2007;175(10):1054-1060. 3. Raghu G et al. Am J Respir Crit Care Med. 2011;183(6):788-824. 4. Lynch DA et al. Lancet Respir Med. 2018;6(20:138-153.