Interstitial lung disease: diagnosis and testing Professor Joanna - 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: diagnosis and testing 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-00617 This non-promotional material is for medical-to-medical use only

By the end of this module you should be able to: ▪ Outline how ILD is reviewed and investigated in secondary care, including: – Lung function tests – Blood tests for autoimmune disease – Bronchoalveolar lavage – Biopsy ▪ Explain the importance of monitoring disease progression ▪ Explain how ILD is diagnosed in tertiary care and the role of the MDT ▪ Outline the provision of services available in secondary and tertiary ILD care ILD, interstitial lung disease; MDT, multidisciplinary team

ILD diagnostic tools Comprehensive medical history 1,2 - Social and family history - Occupational, environmental and drug exposures ILD, interstitial lung disease 1. Raghu, G. et al. (2011); 2. NICE clinical guideline 163. June 2013 (last updated May 2017).

ILD diagnostic tools Pulmonary function tests 1,2 Comprehensive medical - FVC history 1,2 - DL CO - Social and family history - 6MWT - Occupational, Serological tests 1,2 environmental and drug exposures 6MWT, 6-minute walk test; DL CO , diffusing capacity for carbon monoxide; FVC, forced vital capacity; ILD, interstitial lung disease 1. Raghu, G. et al. (2011); 2. NICE clinical guideline 163. June 2013 (last updated May 2017).

ILD diagnostic tools Pulmonary function tests 1,2 Comprehensive medical - FVC history 1,2 - DL CO - Social and family history - 6MWT - Occupational, Serological tests 1,2 environmental and drug exposures Radiological assessment Chest x-ray 2 - HRCT 1,2 - 6MWT, 6-minute walk test; DL CO , diffusing capacity for carbon monoxide; FVC, forced vital capacity; HRCT, high-resolution computed tomography; ILD, interstitial lung disease 1. Raghu, G. et al. (2011); 2. NICE clinical guideline 163. June 2013 (last updated May 2017).

Guidelines recommend multidisciplinary diagnosis to improve accuracy Consultant Consultant ILD specialist respiratory radiologist nurse physician MDT Consultant Thoracic surgeon* coordinator histopathologist* Please note local practice may differ. NICE recommend at a minimum the MDT is comprised of a consultant respiratory physician, consultant radiologist, interstitial lung disease specialist nurse and a multidisciplinary team coordinator. *In cases where biopsies or bronchoalveolar lavage are being considered, the MDT should also include a consultant histopathologist and a thoracic surgeon. ILD, interstitial lung disease; MDT, multidisciplinary team. 2. NICE clinical guideline 163. June 2013 (last updated May 2017).

ILD diagnostic tools Pulmonary function tests 1,2 Comprehensive medical - FVC history 1,2 - DL CO - Social and family history - 6MWT - Occupational, Serological tests 1,2 environmental and drug exposures Radiological assessment Surgical assessment Chest x-ray 2 - Bronchoalveolar lavage 2 - HRCT 1,2 - Transbronchial biopsy 2 - Surgical lung biopsy 1,2 - Cryosurgical assessment 4 - 6MWT, 6-minute walk test; DL CO , diffusing capacity for carbon monoxide; FVC, forced vital capacity; HRCT, high-resolution computed tomography; ILD, interstitial lung disease 1. Raghu, G. et al. (2011); 2. NICE clinical guideline 163. June 2013 (last updated May 2017); 4. Mikolasch, T.A. et al. (2017).

Interstitial lung disease specialist centres in England* Manchester University NHS Foundation Trust Aintree University Hospital NHS Foundation Trust Newcastle upon Tyne Hospitals NHS Foundation Trust Leeds Teaching Hospitals NHS Trust University Hospital of North Midlands NHS Trust Sheffield Teaching Hospitals NHS Foundation Trust Hull and East Yorkshire Hospitals NHS Trust Nottingham University Hospitals NHS Trust University Hospitals Birmingham NHS Foundation Trust University Hospitals of Leicester NHS Trust North Bristol NHS Foundation Trust University Hospitals Coventry and Warwickshire NHS Trust Norfolk and Norwich University Hospital NHS Foundation Trust Royal Papworth Hospital NHS Foundation Trust University College London Hospitals NHS Foundation Trust Guy’s and St Thomas' NHS Foundation Trust Oxford University Hospitals NHS Foundation Trust Imperial College Healthcare NHS Trust Royal Devon and Exeter NHS Foundation Trust Royal Brompton and Harefield NHS Foundation Trust University Hospital Southampton NHS Foundation Trust Portsmouth Hospitals NHS Trust *There are different prescribing rules outside of England. 8. British Lung Foundation. Pulmonary fibrosis services in the UK.

Summary: diagnosis and testing ▪ A good medical history is key to diagnosis 1,2,4 ▪ As well as lung function testing, blood tests will be used to investigate any possible autoimmune disease 1,2,4 ▪ A CT scan will also be carried out; if the diagnosis is unclear or more information is needed bronchoalveolar lavage may be performed and, if necessary, a lung biopsy 1,2,4 ▪ The differentiation between fibrotic and inflammatory ILD, and identifying a cause where possible, is important because of the complexity of the condition and the treatment options available 1,4 COPD, chronic obstructive pulmonary disease; CT, computed tomography; ILD, interstitial lung disease 1. Raghu, G. et al. (2011); 2. NICE clinical guideline 163. June 2013 (last updated May 2017); 4. Mikolasch, T.A. et al. (2017).

Summary: diagnosis and testing ▪ Many people with ILD are managed in secondary care, while complex cases may be referred to a specialist tertiary centre; these centres are the only ones able to prescribe some treatments ▪ Patients may be concerned about travelling to a specialist centre and shared care arrangements between secondary and tertiary care are sometimes made, although contact with the tertiary centre needs to be maintained ILD, interstitial lung disease

References 1. 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, BS., 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. 2. 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). 3. British Lung Foundation. January 2017. Tests to measure your breathing. Available at: cdn.shopify.com/s/files/1/0221/4446/files/1._Tests_to_measure_your_breathing.pdf?6157169238646367902&_ga=2.145315060.197531 3960.1568016302-1905844842.1559040729 (accessed October 2019). 4. Mikolasch, T.A., Garthwaite, H.S. and Porter, J.C. (2017). Update in diagnosis and management of interstitial lung disease. Clin Med, 17(2), pp. 146 – 153. 5. 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. 6. Hutchinson, J.P., Fogarty, A.W., McKeever, T.M. and Hubbard, R.B. (2016). In-hospital mortality after surgical lung biopsy for interstitial lung disease in the United States 2000 to 2011. Am J Respir Crit Care Med, 193(10), pp. 1161 – 1167.

References 7. Meyer, K.C. and Raghu, G. (2011). Bronchoalveolar lavage for the evaluation of interstitial lung disease: is it clinically useful? Eur Respir J, 38(4), pp. 761 – 769. 8. British Lung Foundation. Pulmonary fibrosis services in the UK. September 2016. Available at: www.blf.org.uk/sites/default/files/Table%20of%20UK%20PF%20services%20in%20the%20UK%20v2.1.pdf (accessed October 2019).