Interstitial lung disease: treatment options and considerations - 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: treatment options and considerations 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 This non-promotional material is for medical-to-medical use only Date of preparation: October 2019 | SC-UK-00618

By the end of this module you should be able to: ▪ Identify treatment options for inflammatory-type ILD ▪ Understand the different treatment considerations for inflammatory and fibrotic forms of ILD ▪ Identify non-drug treatments and other therapies for ILD ILD, interstitial lung disease; IPF, idiopathic pulmonary fibrosis

Types of interstitial lung disease (ILD) Interstitial lung disease Inflammatory Granulomatous: Other forms, e.g. Known cause or association: Fibrotic • Connective tissue diseases • Sarcoidosis • Lymphangioleio- Idiopathic interstitial • Hypersensitivity pneumonitis • Occupational causes pneumonias myomatosis • Drug side-effects • Infections • Histiocytosis X IPF Unclassifiable Rare Major Idiopathic Idiopathic lymphoid Chronic fibrosing Smoking related Acute and subacute pleuroparenchymal interstitial pneumonia fibroelastosis Cryptogenic Desquamative organising interstitial Idiopathic Non-specific pneumonia pneumonia* pulmonary interstitial fibrosis pneumonia Acute interstitial Respiratory pneumonia bronchiolitis-ILD *Desquamative interstitial pneumonia can occasionally occur in non-smokers. Figure adapted from American Thoracic Society and European Respiratory Society (2002) 1 and Travis, WD. et al. (2013). 2 ILD, interstitial lung disease; IPF, idiopathic pulmonary fibrosis; NSIP, non-specific interstitial pneumonia 1. American Thoracic Society and European Respiratory Society (2002); 2. Travis, WD. et al. (2013).

Summary: treatment options and considerations ▪ Accurate diagnosis can get patients on to the appropriate treatment that will make a difference 3 ▪ Treatment options depend on whether ILD is diagnosed as inflammatory or fibrotic 3 ▪ Non-drug treatments should also be considered, such as: exercise, weight management and quitting smoking, as well as pulmonary rehabilitation 3,12 ▪ Consider oxygen therapy where appropriate 3,12 ▪ Manage co-morbidities such as gastro-oesophageal reflux, which can exacerbate ILD 3,12 ILD, interstitial lung disease; IPF, idiopathic pulmonary fibrosis 3. Meyer, K. (2014); 12. NICE clinical guideline 163. June 2013 (last updated May 2017).

References 1. 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. 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. Meyer, K. (2014). Diagnosis and management of interstitial lung disease. Transl Respir Med, 2, pp. 4. 4. NHS. Treatment: Idiopathic pulmonary fibrosis. May 2019. Available at: www.nhs.uk/conditions/idiopathic-pulmonary-fibrosis/treatment/ (accessed October 2019). 5. Wells, A.U. and Hirani, N. (2008). Interstitial lung disease guideline: the British Thoracic Society in collaboration with the Thoracic Society of Australia and New Zealand and the Irish Thoracic Society. Thorax, 63(suppl V), pp. 1 – 58. 6. British Lung Foundation. Hypersensitivity pneumonitis formerly called extrinsic allergic alveolitis. August 2016. Available at: www.blf.org.uk/sites/default/files/5.%20Hypersensitivity%20pneumonitis.pdf (accessed October 2019). 7. van den Hombergh, W.M.T., Simons, S.O., Teeselink, E., Knaapen-Hans, H.K.A., van den Hoogen, F.H.J., Fransen, J. and Vonk, M.C. (2018). Intravenous cyclophosphamide pulse therapy in interstitial lung disease associated with systemic sclerosis in a retrospective open-label study: influence of the extent of inflammation on pulmonary function. Clin Rheumatol, 37(10), pp. 2715 – 2722.

References 8. Robles-Perez, A. and Molina-Molina, M. (2015). Treatment considerations of lung involvement in rheumatologic disease. Respiration, 90, pp. 265 – 274. 9. Berezne, A., Ranque, B., Valeyre, D., Brauner, M., Allanore, Y., Launay, D., Le Guern, V., Kahn, JE., Couderc, L.J., Constans, J., Cohen, P., Mahr, A., Pagnoux, C., Hachulla, E., Kahan, A., Cabane, J., Guillevin, L. and Mouthon, L. (2008). Therapeutic strategy combining intravenous cyclophosphamide followed by oral azathioprine to treat worsening interstitial lung disease associated with systemic sclerosis: a retrospective multicenter open-label study. J Rheumatol, 35(6), pp. 1064 – 1072. 10. Coker, R.K. (2009). Management strategies for pulmonary sarcoidosis. Ther Clin Risk Manag, 5, pp. 575 – 584. 11. Margaritopoulos, G.A., Antoniou, K.M. and Wells, A.U. (2017). Comorbidities in interstitial lung diseases. Eur Respir Rev, 26(143), pp. 160027. 12. 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). 13. 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 14. Raghu, G., Rochwerg, B., Zhang, Y., Garcia, C.A., Azuma, A., Behr, J., Brozek, J.L., Collard, H.R., Cunningham, W., Homma, S., Johkoh, T., Martinez, F.J., Myers, J., Protzko, S.L., Richeldi, L., Rind, D., Selman, M., Theodore, A., Wells, A.U., Hoogsteden, H. and Schünemann, H.J.; American Thoracic Society; European Respiratory society; Japanese Respiratory Society; Latin American Thoracic Association. (2015). An official ATS/ERS/JRS/ALAT clinical practice guideline: treatment of idiopathic pulmonary fibrosis. An update of the 2011 clinical practice guideline. Am J Respir Crit Care Med , 192(2), pp. e3 – 19.