Interstitial lung disease: identification and patient referral - PowerPoint PPT Presentation

RCGP accredited until 1 st April 2020 RCN accredited until 2 nd July 2020 Accreditation applies only to the educational content and not to any product RCN cannot confirm competence of any practitioner Interstitial lung disease: identification and patient referral Professor Joanna Porter, Consultant in Respiratory and General Medicine University College London Hospitals and Senior Lecturer in the Department of Medicine at UCL In conversation with Dr Steve Holmes, GP, Park Medical Partnership, Shepton Mallet and Evercreech Education Lead and Executive, Primary Care Respiratory Society UK This video has been developed and funded by Boehringer Ingelheim Date of preparation: October 2019 | SC-UK-00616 This non-promotional material is for medical-to-medical use only

By the end of this module you should be able to: ▪ Identify the causes of different types of ILD ▪ Describe how to take a medical history to identify the possible causes of ILD ▪ Describe how to examine and investigate suspected ILD in primary care ▪ Differentiate ILD from other respiratory conditions (COPD, bronchiectasis, asthma) ▪ Refer a patient with suspected ILD to secondary care ▪ Explain the differences between the sounds of healthy lungs vs COPD and ILD COPD, chronic obstructive pulmonary disease; ILD, interstitial lung disease

Types of interstitial lung disease (ILD) Interstitial lung disease Granulomatous: Other forms, e.g. Known cause or association: • Connective tissue diseases • Sarcoidosis • Lymphangioleio- Idiopathic interstitial • Hypersensitivity pneumonitis • Occupational causes pneumonias myomatosis • Drug side-effects • Infections • Histiocytosis X Unclassifiable Rare Major Idiopathic Idiopathic lymphoid Chronic fibrosing Smoking related Acute and subacute pleuroparenchymal interstitial pneumonia fibroelastosis Idiopathic Desquamative Cryptogenic pulmonary interstitial organising fibrosis pneumonia* pneumonia Non-specific interstitial Acute interstitial Respiratory pneumonia pneumonia bronchiolitis-ILD *Desquamative interstitial pneumonia can occasionally occur in non-smokers. Figure adapted from Travis, WD. et al. (2013) 2 and American Thoracic Society and European Respiratory Society (2002) 3 2. Travis, WD. et al. (2013); 3. American Thoracic Society and European Respiratory Society (2002).

Symptoms of ILD 4,5 ILD UIP/NSIP Audio of ILD, COPD and healthy lungs can be heard at the end of this video COPD, chronic obstructive pulmonary disease; ILD, interstitial lung disease; NSIP, non-specific interstitial pneumonia; UIP, usual interstitial pneumonia 4. NICE clinical guideline 163. June 2013 (last updated May 2017); 5. Raghu, G. et al. (2011).

Finger clubbing may present as a clinical feature of ILD 4,5 ILD, interstitial lung disease Image by Desherinka. 10 4. NICE clinical guideline 163. June 2013 (last updated May 2017); 5. Raghu, G. et al. (2011); 10. Image by Desherinka CC BY-SA 4.0 (https://creativecommons.org/licenses/by-sa/4.0).

Summary: identification and patient referral ▪ There are around 200 different types of ILD with a range of aetiologies. ILD results in damage to the lung, which can be divided into two types: inflammatory and fibrotic, although many patients will develop both responses 1 ▪ ILD can be connected to an underlying autoimmune condition, environmental exposure to animal allergens such as duck feathers or non-living materials (e.g. asbestos), or be related to prior medical intervention 5 ▪ It is important to take a good patient history to help establish the likely cause. If all of the above have been excluded, then the disease will be classified as idiopathic 5 ▪ Shortness of breath and coughing are common signs of ILD, but also of COPD and heart failure, so it is important to be able to distinguish between the conditions. Other symptoms of ILD include finger clubbing and a characteristic crackling when listening to patient breathing with a stethoscope 5,13 COPD, chronic obstructive pulmonary disease; ILD, interstitial lung disease 1. Cottin, V. et al . (2018); 5. Raghu, G. et al. (2011); 13. Hawkins, N.M. et al . (2009).

Summary: identification and patient referral ▪ Spirometry is a useful investigation in primary care; CT scans can help exclude ILD in patients with other reasons for shortness of breath. Echocardiograms can identify pulmonary hypertension, which patients with ILD are at risk of ▪ When making a referral to secondary care for suspected ILD, it’s useful to include information on how debilitated the patient is, how committed they are to a treatment that may involve travelling to hospital, and the results of any investigations already carried out CT, computerised tomography; ILD, interstitial lung disease 4. NICE clinical guideline 163. June 2013 (last updated May 2017); 14. Panagiotou, M. et al . (2017).

Example lung sounds

Sounds of healthy lungs

Sounds of a patient with COPD

Sounds of a patient with IPF ▪ The assessment of basal "velcro crackles" by lung auscultation is currently the only realistic means for the earlier diagnosis of IPF 12 IPF, idiopathic pulmonary fibrosis 12. Cottin, V. et al. (2012).

References 1. Cottin, V., Hirani, N.A., Hotchkin, D.L., Nambiar, A.M., Ogura, T., Otaola, M., Skowasch, D., Park, J.S., Poonyagariyagorn, H.K.., Wuyts, W. and Wells, A.U. (2018). Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases. Eur Respir Rev , 27(150), pii: 180076. 2. Travis, W.D., Costabel, U., Hansell, D.M., King, T.E. Jr, Lynch, D.A., Nicholson, A.G., Ryerson, C.J., Ryu, J.H., Selman, M., Wells, A.U., Behr, J., Bouros, D., Brown, K.K., Colby, T.V., Collard, H.R., Cordeiro, C.R., Cottin, V., Crestani, B., Drent, M., Dudden, R.F., Egan, J., Flaherty, K., Hogaboam, C., Inoue, Y., Johkoh, T., Kim, D.S., Kitaichi, M., Loyd, J., Martinez, F.J., Myers, J., Protzko, S., Raghu, G., Richeldi, L., Sverzellati, N., Swigris, J. and Valeyre, D.; ATS/ERS Committee on Idiopathic Interstitial Pneumonias. (2013). An official American Thoracic Society/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med, 188(6), pp. 733 – 748. 3. American Thoracic Society and European Respiratory Society. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. (2002). This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med, 165(2), pp. 277 – 304. 4. National Institute for Health and Care Excellence. Clinical guideline 163. June 2013 (last updated May 2017). Idiopathic pulmonary fibrosis in adults: diagnosis and management. Available at: www.nice.org.uk/guidance/cg163 (accessed October 2019). 5. Raghu, G., Collard, H.R., Egan, J.J., Martinez, F.J., Behr, J., Brown, K.K., Colby, T.V., Cordier, J.F., Flaherty, K.R., Lasky, J.A., Lynch, D.A., Ryu, J.H., Swigris, J.J., Wells, A.U., Ancochea, J., Bouros, D., Carvalho, C., Costabel, U., Ebina, M., Hansell, D.M., Johkoh, T., Kim, D.S., King, T.E. Jr, Kondoh, Y., Myers, J., Müller, N.L., Nicholson, A.G., Richeldi, L., Selman, M., Dudden, R.F., Griss, B.S., Protzko, S.L. and Schünemann, H.J.; ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosis. (2011). An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med, 183(6), pp. 788 – 824.

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