Respiratory MCNs - Interstitial lung diseases Dr Nik Hirani Senior Lecturer and Honorary Consultant, Royal Infirmary Edinburgh

Interstitial Lung Diseases Interstitial pneumonias Hypersensitivity Pneumonitis Sarcoidosis Eosinophilic syndromes Known causes Connective tissue diseases Idiopathic Known causes Birds, Farmers lung, drugs etc Idiopathic Known causes Drugs, parasites, vasculitis, ABPA Idiopathic eosinophilic pneumonia DIP RB-ILD AIP OP NSIP LIP UIP = IPF = COP Other rare disease Langerhans cell histiocytosis Lymphangioleiomyomatosis Alveolar proteinosis = Hamman- Rich syndrome

Interstitial Lung Diseases GET ME OUT OF HERE! Interstitial pneumonias Hypersensitivity Pneumonitis Sarcoidosis Eosinophilic syndromes Known causes Connective tissue diseases Idiopathic Known causes Birds, Farmers lung, drugs etc Idiopathic Known causes Drugs, parasites, vasculitis, ABPA Idiopathic eosinophilic pneumonia DIP RB-ILD AIP OP NSIP LIP UIP = IPF = COP Other rare disease Langerhans cell histiocytosis Lymphangioleiomyomatosis Alveolar proteinosis = Hamman- Rich syndrome

1000 patients with ILD in the Edinburgh clinic 2004-2009 IPF Sarcoidosis Rheumatological NSIP HP COP Miscellaneous

Idiopathic pulmonary fibrosis 4-5000 new cases per year in UK Median age at presentation 68 yr, rare under 40 yrs M:F 2:1 60% smokers 5000 deaths per year Gribben et al Thorax 2006

Clinical Presentation of IPF Breathlessness over few weeks/months Bibasal fine ‘velcro’ crackles Diffuse CXR abnormality, often basal, initially subtle Restrictive lung defect (finger clubbing) Diffuse lines and nodules on CXR = FIBROSING ALEOLITIS SYNDROME

HRCT is cornerstone of investigation ….but only 60% of HRCT’s are ‘diagnostic’ in the right clinical context

Lung biopsy enhances diagnostic process ….but only 30% of patients with ‘non-diagnostic’ clinical and radiological features have a biopsy

Variability in the clinical course of IPF Diagnosis = prognosis LUNG FUNCTION / SYMPTOMS TIME Sarcoidosis NSIP, CTD-ILD IPF From Flaherty et al, Thorax, 2003

How to predict poor prognosis in IPF The more ‘classic’ the clinical features, the worse the prognosis Smoking males Age? Hospital admission with ‘exacerbation’ Serial lung function – 10% change in FVC or 20% change in TLCO

Treatment of IPF Best supportive care Transplant for the minority Recruit to clinical trials

Standards of care in ILD

1. Diagnosis of ILD Requires a multidisciplinary approach Consensus between chest physician, radiologist and pathologist (+rheumatology, occupational health)

2. Treatment including supportive care Tailored therapy requires diagnostic precision! Smoking cessation Pulmonary rehabilitation Treat co-existing chest disease Anti-reflux therapy Oxygen therapy Withdrawal of potentially toxic futile therapy Early referral for lung transplant Recruitment to clinical trials

3. Palliation Equality of access to palliative care services Condition-specific palliative care protocols

4. Organisation of services Modified from current care models for pulmonary hypertension, CF and lung cancer ‘Regional centres’ comprising of teams that can address diagnostic and treatment standards. Each centre must: 1)establish a consistent care pathway for ILD patients 2) hold a regular regional MDM 3) enable recruitment to multi-centre clinical trials 4) establish and audit programme of outcome measures of ILD patients 5) deliver approved new therapies to selected patients.

Summary ILDs are an important burden of disease IPF is a lethal disease requiring expert care Diagnosis and management requires a multidisciplinary approach MCNs in ILD are in an early stage of development but will be pivotal in delivering care