Monday 10 September Interstitial lung disease 15:10 15:35. The uncommon interstitial lung diseases (ILD)

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1 Interstitial lung disease 15:10 15:35 The uncommon interstitial lung diseases (ILD) Dr Grant Griffiths, Cwm Taf University Health Board, Cardiff Be familiar with the Diagnostic criteria for idiopathic pulmonary fibrosis: a Fleischner Sociey White Paper (Lancet 2018), to facilitate assessment of ILD on high-resolution computed tomography (HRCT). Pleuroparenchymal fibroelastosis (PPFE) is a rare ILD which is under recognised. It is characterised by striking and progressive upper lobe predominant fibrosis that involves both the visceral pleura and subpleural lung parenchyma. Lymphocytic interstitial pneumonitis (LIP) is a rare sub-type of ILD. The condition has a strong association with autoimmune disease, such as Sjogren syndrome and is far more common in women. Always consider this diagnosis in the corect clinical context, especially if there is a cystic infiltrate with peribronchovascular thickening/nodules.the differential should include lymphangioleiomyomatosis (LAM) and Langerhans cell histiocytosis (LCH). Combined pulmonary fibrosis (CPFE) is recognised as a coexisting pattern on HRCT. CPFE comprises a heterogeneous population of patients, not believed to represent a distinctive IIP. These patients have increased risk of developing pulmonary hypertension (and therefore poorer prognosis). Travis WD, Costabel U, Hansell DM et al. 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 2013; 188 (6): Piciucchi S, Tomassetti S, Casoni G et al. High resolution CT and histological findings in idiopathic pleuroparenchymal fibroelastosis: features and differential diagnosis. Respir Res 2011; 12(1): 111. Cottin V, Cordier JF. The syndrome of combined pulmonary fibrosis and emphysema. Chest 2009; 136 (1): 1 2.

2 Interstitial lung disease 15:35 16:00 Update on clinical management of idiopathic pulmonary fibrosis (IPF) Dr Huzaifa Adamali, North Bristol NHS Trust Idiopathic pulmonary fibrosis is a chronic, progressive interstitial lung disease in which progressive lung scarring occurs in the intersitium of the lungs; the biological process underlying IPF are though to reflect aberrant reparative responses to repetitive alveolar epithelial injury in genetically susceptible aging individuals. IPF is defined on the presence of a radiographic and/or histological pattern of usual interstitial pneumonia (UIP) in the absence of an alternative aetiology for this pattern; the multidisciplinary team meeting is essential for its diagnosis. The disease is characterised by increasing cough and shortness of breath with devastating effect of quality of life. Progress has been made in the understanding the clinical management of IPF with the availability of two phramacotherapeutic agents pifenidone and nintedanib. Acute exacerbation preceded approximately 40% of IPF deaths and is often associated with a poor prognosis; the median survival following an acute exacerbation is three to four months. Lederer MD, Martinez MD. Idiopathic pulmonary fibrosis. N Engl J Med 2018; 378: 1,811 1,823. Soo E, Adamali H, Edey AJ. Idiopathic pulmonary fibrosis: current and future directions. Clin Radiol 2017; 72: Lynch DA, Sverzellati N, Travis WD et al. Diagnostic criteria for idiopathic pulmonary fibrosis: a Fleischner Society White Paper. Lancet Respir Med 2018; 6:

3 Interstitial lung disease 16:40 17:05 Computerised evaluation of idiopathic pulmonary fibrosis (IPF) Dr Joseph Jacob, King s College Hospital NHS Foundation Trust, London Computer analysis is allowing us to identify novel computed tomography (CT) features predictive of mortality in IPF. New automated CT features such as the pulmonary vessels described by CALIPER have no simple visual correlate. The volume of vessels-related structures has been shown to have prognostic strength across a range of fibrosing lung diseases. In the future we may evaluate automated CT features that have absolutely no visual correlate providing new challenges for radiologists, clinicians and patients. Computer analysis is currently hampered by limitations in longitudinal CT datasets in IPF patients. Jacob J, Bartholmai BJ, Rajagopalan S et al. Predicting outcome in idiopathic pulmonary fibrosis using automated CT analysis. AJRCCM 2018: doi: /rccm OC. Wu X, Kim GH, Salisbury ML et al. Computed tomographic biomarkers in idiopathic pulmonary fibrosis: the future of quantitative analysis. Am J Respir Crit Care Med; ln press. Robbie H, Daccord C, Chua F, Devaraj A. Evaluating disease severity in idiopathic pulmonary fibrosis. European Respiratory Review 2017; 26(145): doi: /

4 Interstitial lung disease (continued) 17:05 17:30 Complications of interstitial lung disease (ILD) Dr Jonathan Rodrigues, Royal United Hospitals Bath NHS Foundation Trust Acute worsening of dyspnea in fibrotic interstitial lung disease (ILD) may be due to: 1) acute exacerbation, 2) an acute extra-parenchymal cause and/or 3) rapid progression of underlying fibrosis. Acute exacerbations in idiopathic pulmonary fibrosis (IPF) increase in frequency with disease severity and portend poor prognosis. The high-resolution (HRCT) pattern of groundglass opacity superimposed on reticulation and the extent are independent predictors of survival, but are not aetiology-specific. Acute extra-parenchymal causes include spontaneous extra-alveolar air collections, but these may also be asymptomatic. In IFP, extensive reticulation is a predictor of pneumothorax that, in turn, is an independent predictor of poor outcome. Pneumomediastium, although less common, is associated with mortality in IPF. Fibrotic ILD is a risk factor for primary lung malignancy. It also complicates lung cancer treatment; the presence of IPF is associated with higher morbidity and mortality after lobectomy and radical radiotherapy. More chronically, fibrotic ILD can result in pulmonary artery hypertension (PAH). The underlying ILD aetiology may also directly contribute to PAH, for example, scleroderma. IPF in the presence of emphysema increases the risk of severe PAH. Treatment of ILD-related PAH is challenging. Ryerson CJ, Collard HR. Acute exacerbations complicating interstitial lung disease. Curr Opin Pulm Med 2014; 20(5): Soo E, Adamali H, Edey AJ. Idiopathic pulmonary fibrosis: current and future directions. Clin Radiol 2017; 72(5): Artinian V, Kvale PA. Cancer and interstitial lung disease. Curr Opin Pulm Med 2004; 10(5): Akira M, Kozuka T, Yamamoto S, Sakatani M. Computed tomography findings in acute exacerbation of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2008; 178(4):

5 Colombi D, Ehlers-Tenenbaum S, Palmowski K et al. Spontaneous pneumomediastinum as a potential predictor of mortality in patients with idiopathic pulmonary fibrosis. Respiration 2016; 92(1): Nishimoto K, Fujisawa T, Yoshimura K et al. The prognostic significance of pneumothorax in patients with idiopathic pulmonaryfibrosis. Respirology 2018; 23(5):

6 Interstitial lung disease (continued) 16:40 18:30 Cystic lung disease Dr Judith Babar, Cambridge University Hospitals NHS Foundation Trust Pulmonary cysts can be seen in isolated chest disorders, a manifestation of a multisystemic disease or may be part of the aging changes of the lungs. Distribution and shape of pulmonary cysts, as well as other ancillary cpmputed tomography (CT) signs can be characteristic in suggesting a diagnosis. Ultimately, a confident diagnosis can only be made with integration of CT features, clinical information and histopathology. A practical approach can be to divide those pulmonary cysts seen in isolation, diffuse or with ancillary CT features. Mimics of cystic lung disease can include cavities, honeycombing or cystic bronchiectasis. Beddy P, Babar J, Devaraj. A practical approach to cystic lung disease on HRCT. Insights Imaging 2011; 2(1): 1 7. Seaman DM, Meyer C, Gilman M, McCormack F. Diffuse cystic lung disease at highresolution CT. Am J Roentgenol 2011; 196(6): 1,305 1,311. Raoof S et al. Cystic lung diseases. Chest 2016; 150(4):

7 Interstitial lung disease (continues) 17:55 18:20 Hypersensitivity pneumonitis Dr Carole Ridge, Royal Brompton and Harefield NHS Foundation Trust, London The diagnosis of hypersensitivity pneumonitis ideally requires the identification of a causative antigen, time relation between exposure and disease, mosaic attenuation on chest computed tomography (CT) and poorly formed non-necrotising granulomas on pathology. Acute forms generally resolve without sequelae, while chronic forms, which are caused by persistent low-grade exposures, are associated with poor prognosis. Mosaic attenuation is a prominent feature of this disease. Morisset J, Johannson KA, Jones KD et al. Identification of diagnostic criteria for chronic hypersensitivity pneumonitis: an international modified delphi survey. Am J Respir Crit Care Med 2017; doi: /rccm OC. [Epub ahead of print].