A MULTIDISCIPLINARY APPROACH INVOLVING A CLINICAL, RADIOLOGICAL AND PATHOLOGICAL REVIEW IS RECOMMENDED FOR EARLY AND ACCURATE ILD DIAGNOSIS1,3–6
Example of a multidisciplinary team
It is recommended that all collected diagnostic information be evaluated by a multidisciplinary team experienced in ILD, which may either establish a diagnosis or discuss the indication for further diagnostic procedures such as thoracoscopic lung biopsy or transbronchial cryobiopsy.7
Weighing diagnostic yield against potential risks associated with each procedure is crucial for discussion among the members of the multidisciplinary team and with the patient.7
Algorithm for the diagnosis of pulmonary fibrosis7
The participants consider all data available and propose a provisional first-choice diagnosis, assess the need for biopsy and confidence in the diagnosis, and consider possible alternative diagnoses. If a case cannot be addressed by a multidisciplinary team, it is important for the treating clinician to realize that many factors need to be considered before diagnostic decisions can be made.
Adapted from: Wijsenbeek M, Cottin V. N Engl J Med. 2020;383:958–968.
Other considerations for diagnosis of ILDs:
- On chest auscultation, fine Velcro crackles are indicative of fibrosis7
- A thorough patient history, including environmental exposures, medication use and extrapulmonary signs, should be evaluated7
Other considerations for ILD diagnosis in CTDs:
- In CTDs, ILD can develop either after the CTD has been diagnosed or before the rheumatological symptoms of the CTD appear
- This makes it important to conduct physical and serologic examinations if there is any suspicion of CTD-ILD7
What if the cause of ILD is unclassifiable after assessment?
- Some ILD cases remain unclassifiable even after thorough assessment.7 In the instance of new forthcoming information, a working diagnosis can later become a more definitive differential diagnosis
DIAGNOSIS OF IPF
Guidelines recommend a multidisciplinary approach, involving a clinical, radiological and pathological review to achieve an early and accurate IPF diagnosis:3,9–11
In an Australian study (n=90), multidisciplinary discussion resulted in diagnosis change: 10 (37%) of 27 patients referred with a diagnosis of IPF changed to non-IPF diagnosis and 7 patients had diagnosis changed to IPF because of MDT discussion13
Diagnostic algorithm for an accurate, timely diagnosis of IPF from ATS/ERS/JRS/ALAT guidelines3
* Surgical lung biopsy is not indicated in patients at high risk for intra-, peri-, or postoperative complications (e.g. severe hypoxemia at rest and/or severe pulmonary hypertension with a diffusion capacity less than 25% after correction for hematocrit). Surgical lung biopsy may be unnecessary in some familial cases.3
Reprinted from Annals of the American Thoracic Society, 183(6), Raghu et al, An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management, p 788–824, ©2022.
For patients at risk of IPF, HRCT should be evaluated at the first suspicion of ILD involvement.9
Features consistent with a UIP pattern on HRCT include:9
ATS/ERS/JRS/ALAT guidelines recommend that IPF can be confidently diagnosed if the clinical evaluation of the patient points to IPF and the HRCT shows a definite or probable pattern of UIP (conditional biopsy recommended).3
IPF diagnosis based upon HRCT and biopsy patterns according to the ATS/ERS/JRS/ALAT Clinical Practice Guideline 20183
Reprinted from Annals of the American Thoracic Society, 183(6), Raghu et al, An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management, p788–824, ©2022.
If a specific diagnosis is not made or no potential cause for ILD is identified, then clinical findings and appropriate combination of HRCT and histopathological patterns are considered during multidisciplinary discussion to either ascertain or exclude the diagnosis of IPF.3
- * Patients clinically suspected of having IPF are those with unexplained symptomatic or asymptomatic patterns of bilateral pulmonary fibrosis on a chest radiograph or chest computed tomography, bibasilar inspiratory crackles, and age greater than 60 years.3
- † IPF is the likely diagnosis when any of the following features are present:3
- * Moderate-to-severe traction bronchiectasis/bronchiolectasis (defined as mild traction bronchiectasis/bronchiolectasis in four or more lobes including the lingual as a lobe, or moderate-to-severe traction bronchiectasis in two or more lobes) in a man over age 50 years or in a woman over age 60 years
- * Extensive (>30%) reticulation on HRCT and an age >70 years
- * Increased neutrophils and/or absence of lymphocytosis in BAL fluid
- * Multidisciplinary discussion reaches a confident diagnosis of IPF
- ‡ Indeterminate for IPF:3
- * Without an adequate biopsy is unlikely to be IPF
- * With an adequate biopsy may be reclassified to a more specific diagnosis after multidisciplinary discussion and/or additional consultation
DIAGNOSIS OF IDIOPATHIC NON-SPECIFIC INTERSTITIAL PNEUMONIA (iNSIP)
Diagnostic tests for iNSIP involve:
Surgical lung biopsy
(showing uniformity of interstitial involvement) used when HRCT suggests NSIP pattern that overlaps with the OP and DIP patterns15,17
A multidisciplinary discussion involving pulmonologists, radiologists and pathologists is advised in cases where the clinical, radiologic and pathologic features are not definite or probable for NSIP15
DIAGNOSIS OF SARCOIDOSIS-ASSOCIATED INTERSTITIAL LUNG DISEASE
Guidelines recommend that diagnosis of sarcoidosis should involve the following diagnostic tools:18
Histologic and immunologic screening
A comprehensive patient evaluation for sarcoidosis-associated interstitial lung disease should include:18
CT scans of patients with sarcoidosis-associated interstitial lung disease show an extent and type of fibrosis that varies from patient to patient, but is typically manifested as bronchial distortion (marked by traction bronchiectasis and airway angulation), linear scarring, and honeycombing.19
Proportions of patients with sarcoidosis-associated interstitial lung disease with radiographic features on HRCT (n=80)19
If the combination of clinical findings and imaging is not diagnostic, more invasive diagnostic procedures may be needed for sarcoidosis-associated interstitial lung disease, such as BAL.7
DIAGNOSIS OF HYPERSENSITIVITY PNEUMONITIS (HP)
First and foremost, patient exposure history should be thoroughly examined.20
Patients with fibrotic HP typically have reticulation, traction bronchiectasis and volume loss, with or without honeycombing, on HRCT:20
- Subacute HP can usually be seen on HRCT as ground-glass opacities alongside poorly-defined centrilobular nodules20
- Expiratory HRCT imaging may be useful in diagnosing cHP7
On chest auscultation, squeaks indicative of fibrosis may be heard in patients with HP7
A surgical lung biopsy may be needed in cases with no identifiable exposure or HRCT patterns such as UIP which are less suggestive of HP21
If the combination of clinical findings and imaging is not diagnostic, more invasive diagnostic procedures may be needed for HP, such as BAL7
Once diagnosed, how can you break the news to your patients with fibrotic ILDs?
Breaking bad news is a difficult and complex communication task. In our documentary film series, Professor Walter Baile, psychiatrist and co-founder of the SPIKES model, takes us through his practical approach on how to structure difficult conversations
BREAKING BAD NEWS
Chapter 1 – The challenge of breaking bad news
Professor Walter Baile introduces the challenges involved in breaking bad news and what we can learn from experiences in oncology.
Johanna: [00:00:21 – 00:00:41] Breaking bad news about a terminal illness to a patient who’s not expecting it can be emotionally challenging for everyone involved. I know this because it happened to my grandmother who suffered from pulmonary fibrosis. So, I’m in Houston now to speak to Professor Walter Baile, who’s going to tell us more about communication and how to better the conversation. BIG CONVERSATIONS An introduction to Professor Walter Baile and The SPIKES Model Johanna: [00:39:34 – 00:39:37] So, Professor Baile, can you please introduce yourself? Professor Baile: [00:39:37 - 00:39:59] So I’m Walter Baile and I’m a psychiatrist. And I did my psychiatry training at the Johns Hopkins Hospital in Baltimore, Maryland. And for the past 30 years, I’ve worked at M.D. Anderson Cancer Centre and organized a communication skills program called iCare, which is the program for interpersonal communication and relationship enhancement. Johanna: [00:00:34 – 00:00:40] When you first started working in this field, how were bad news being communicated in oncology? Professor Baile: [00:16:03 – 00:16:51] So, the fact is, is that bad news was rarely given to cancer patients and the diagnosis was often disguised behind words such as inflammation or mass. And clinicians at that time had very few treatments for cancer and patients feared it very much because often patients died in pain. And there was very little support for them. So, not giving a diagnosis of cancer or not telling the truth to patients was considered to be something ethical that doctors would do because they would spare the patient the suffering of knowing. So, almost uniformly, patients weren’t told. Johanna: [00:16:52 – 00:17:00] But things have changed, and new treatments have become available. So, what are some of the new challenges that physicians are facing nowadays? Professor Baile: [00:01:53 – 00:02:30] So as a colleague of mine once said, you know, the question is not whether to give bad news, but how to give it. And it’s still it’s a very big challenge for doctors, because many of them now really don’t have sufficient training to give bad news and because of that, they find it very challenging and stressful to deal with the emotions that often arise when patients are given adverse information about their medical condition, even though patients today want to be informed. Johanna: [00:03:31 – 00:03:36] And you came out with a method for it. Can you briefly describe it? Professor Baile: [00:03:39 – 00:03:59] We came up with a method which we call SPIKES, which is an organized stepwise program or a method for teaching communication skills and specifically giving bad news, which consists of six separate steps, which I can talk about in a little while. Professor Baile: [00:04:34 – 00:04:59] It makes a lot of sense to doctors because they often learn to provide treatment and to diagnose disease based upon protocols and to follow guidelines for giving bad news. So that I think it’s become widely accepted because it has an inherent intuitive sense to doctors in other conditions who are giving bad news. Johanna: [00:08:31 – 00:08:33] So, Professor Baile, what does SPIKES stand for? Professor Baile: [00:08:34 – 00:09:22] So the S of SPIKES is for setting or setting up the interview, how you organise yourself before you go into the conversation. P is for perception. Finding out how much the patient already knows about the disease or the tests or any other information you want to know ‘I’ is for invitation and that is making sure the patient’s ready for the information. K is for knowledge and that is how to give the information in a way that the patient understands and allow them to share decisions. E is for emotions how to be empathic when the patient gets upset and ’S’ is for strategy and summary. How you sum up the conversation and help the patient understand what the treatment program is. Johanna: [00:08:55 – 00:09:01] What is the objective of SPIKES with not just clinicians but also with patients? Professor Baile: [00:09:05 – 09:13 and 00:09:27 – 09:52] Patients who receive bad news want to hear it in a compassionate way. So, what SPIKES does is addresses the most common emotional reactions with patients and whether it’s sadness or whether it’s fear or anxiety or crying spells and helps the clinician address that. It also prompts the doctor to prepare for giving bad news by kind of rehearsing in their own mind what they’re going to say and what they’re going to tell the patient.
Once you have reached a diagnosis, how should you manage your patient with fibrotic ILDs?
Interventions for ILDs
- ALAT, Latin American Thoracic Society; ATS, American Thoracic Society; BAL, bronchoalveolar lavage; cHP, chronic hypersensitivity pneumonitis; CT, computed tomography; CTD, connective tissue disease; CTD-ILD, connective tissue disease-associated interstitial lung disease; DIP, desquamative interstitial pneumonia; ERS, European Respiratory Society; HP, hypersensitivity pneumonitis; HRCT, high-resolution computed tomography; ILD, interstitial lung disease; iNSIP, idiopathic non-specific interstitial pneumonia; IPF, idiopathic pulmonary fibrosis; JRS, Japanese Respiratory Society; MDD, multidisciplinary discussion; MDT, multidisciplinary team; NSIP, non-specific interstitial pneumonia; OP, organizing pneumonia; uILD, unclassifiable interstitial lung disease; UIP, usual interstitial pneumonia.
Cottin V, Hirani N, Hotchkin D, et al. Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150):180076.
Wong AW, Ryerson C, Guler S. Progression of fibrosing interstitial lung disease. Respir Res. 2020:29;21(1):32.
- Raghu G, Remy-Jardin M, Myers JL, et al; on behalf of the ATS/ERS/JRS/ALAT. Diagnosis of Idiopathic Pulmonary Fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. 2018;198(5):e44–e68.
- De Sadeleer LJ, Meert C, Yserbyt J, et al. Diagnostic ability of a dynamic multidisciplinary discussion in interstitial lung diseases: A retrospective observational study of 938 cases. Chest. 2018;153(6):1416–1423.
- Antoniou KM, Margaritopoulos GA, Tomassetti S, Bonella F, Costabel U, Poletti V. Interstitial lung disease. Eur Respir Rev. 2014;23(131):40–54.
- Holtze C, Flaherty K, Kreuter M, et al. Healthcare utilisation and costs in the diagnosis and treatment of progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150):180078.
- Wijsenbeek M, Cottin V. Spectrum of Fibrotic Lung Diseases. N Engl J Med. 2020;383:958–968.
- Chowaniec M, Skoczyńska M, Sokolik R, Wiland P. Interstitial lung disease in systemic sclerosis: challenges in early diagnosis and management. Reumatologia. 2018;56(4):249–254.
- Raghu G, Collard HR, Egan JJ, et al; on behalf of the ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosis. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788–824.
- Martinez FJ, Chisholm A, Collard HR, et al. The diagnosis of idiopathic pulmonary fibrosis: current and future approaches. Lancet Respir Med. 2017;5(1):61–71.
- Molina-Molina M, Aburto M, Acosta O, et al. Importance of early diagnosis and treatment in idiopathic pulmonary fibrosis. Exp Rev Resp Med. 2018;12(7):537–539.
- Walsh SLF, Wells AU, Desai SR, et al. Multicentre evaluation of multidisciplinary team meeting agreement on diagnosis in diff use parenchymal lung disease: a case-cohort study. Lancet Respir Med. 2016;4:557-565.
- Jo HE, Glaspole IN, Levin KC, et al. Clinical impact of the interstitial lung disease multidisciplinary service. Respirology. 2016;21(8):1438–1444.
- Tomassetti S, Ryu JH, Piciucchi, et al. Nonspecific Interstitial Pneumonia: What Is the Optimal Approach to Management? Semin Respir Crit Care Med. 2016;37:378–394.
- Travis WD, Hunninghake G, King Jr TE, et al. Idiopathic nonspecific interstitial pneumonia: report of an American Thoracic Society project. Am J Respir Care Med. 2008;177:1338–1347.
- Mueller-Mang C, Grosse C, Schmid K, et al. What Every Radiologist Should Know about Idiopathic Interstitial Pneumonias. Radiographics. 2007;27:595–615..
- Lynch DA, Travis WD, Müller NL. Idiopathic interstitial pneumonias: CT features. Radiology. 2005;236:10–21.
- American Thoracic Society. Statement on sarcoidosis. Joint Statement of the American Thoracic Society (ATS), the European Respiratory Society (ERS) and the World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG) adopted by the ATS Board of Directors and by the ERS Executive Committee, February 1999. Am J Respir Med. 1999;160:736–755.
- Abehsera M. Sarcoidosis with pulmonary fibrosis: CT patterns and correlation with pulmonary function. AJR Am J Roentgenol. 2000;174:1751–1757.
- Kouranos V, Jacob J, Nicholson A, et al. Fibrotic Hypersensitivity Pneumonitis: Key Issues in Diagnosis and Management. J Clin Med. 2017;6:62.
- Morisset J, Johannson KA, Jones KD, et al. Identification of Diagnostic Criteria for Chronic Hypersensitivity Pneumonitis. An International Modified Delphi Survey. Am J Resp Crit Care Med. 2018;197:1036–1044.
- Wijsenbeek MS, Kreuter M, Olson A, et al. Progressive fibrosing interstitial lung diseases: current practice in diagnosis and management. Curr Med Res Opin. 2019:1–10.
- Kolb M, Vašáková M. The natural history of progressive fibrosing interstitial lung diseases. Respir Res. 2019;20(1).
- Cottin V, Wollin L, Fischer A, et al. Fibrosing interstitial lung diseases: knowns and unknowns. Eur Respir Rev. 2019b;28(151):pii:180100.
- Maher TM, Molina-Molina M, Russell AM, et al. Unmet needs in the treatment of idiopathic pulmonary fibrosis-insights from patient chart review in five European countries. BMC Pulm Med. 2017;17(1):124.
- Robalo-Cordeiro C, Campos P, Carvalho L, et al. Idiopathic pulmonary fibrosis in the era of antifibrotic therapy: Searching for new opportunities grounded in evidence. Rev Port Pneumol. 2017;23(5):287–293.