ILD

1. ILD Rachel Cheong and Grace Pink FY1

2. Introduction • Background/ Initial investigations/ Basic management • Case • Specific types: • IPF • Hypersensitivity Pneumonitis (a.k.a Extrinsic Allergic Alveolitis) – Organic Allergens • Industrial Dusts • Sarcoidosis • Finals questions • Further reading

3. Background • Heterogeneous group of diseases which affect the lung parenchyma • Characterised by chronic inflammation and remodelling +/- progressive interstitial fibrosis, hyperplasia of type II epithelial cells and pneumocytes • Grouped together on the basis of symptoms and radiological changes • But very different in their aetiology and prognosis • Getting the right diagnosis is important!

4. Classification ILD Known Aetiology Associated systemic disease Idiopathic Idiopathic Pulmonary Fibrosis Occupational dusts Drugs- amiodarone, methotrexate, sulfasalazine, nitrofurantoin Hypersensitivity Pneumonitis (organic) Infection- TB Sarcoid RA SLE Sjogrens UC

5. Pointers to ILD • Progressive • Dry persistent cough • Reduced exercise tolerance • Drug history • Occupational history • Pets and hobbies • Signs/symptoms of CT disease • An abnormal CXR

6. Case Mr AF, retired 60 y/o man who has presented to his GP with progressive SOB over 6 months He is now feeling SOB when walking to collect his grandchildren from school, which upsets him He has been bothered by a dry cough and the GP trialled a “blue inhaler” Think of one differential and 1 question that would most help you decide about that diagnosis

7. O/E • Dyspnoeic • Clubbing/ Cyanosis • Reduced expansion • Deviated trachea- • towards pathological side • Dull percussion • Fine end-inspiratory crackles

8. How will you investigate? According to BTS guidelines: • Urine dip • FBC, U&E, LFTs • Spirometry and gas transfer • CXR and HRCT (especially if normal CXR) • BAL and lung biopsy (before treatment) Other tests depend on clinical context e.g. sputum culture, ABG , CRP/ESR, BNP, RF/ anti-CCP, ANA, ANCA, ACE Echo

9. CXR/ Is that a whistle?? • CXR changes: • Reticulonodular shadowing • Loss of volume • Widespread/ bilateral

10. HRCT- What’s HR? CT changes: honeycombing, GGO, loss of lung architecture, traction bronchiectasis

11. Spirometry • All patients with ILD should have resting spirometricand gas transfer measurement at presentation  reasonable measure of disease severity • Carbon monoxide transfer factor (TLCO) levels at presentation are a more reliable guide to outcome than other resting lung function variables. • Radiological changes correspond poorly to disease severity

12. Spirometry • Restrictive defect • FVC is reduced • FEV1 is reduced in proportion or slightly less • FEV1:FVC ratio normal or raised

13. General Management • LTOT: • paO2 <7.3 on 2 occasions separated by 2-3 weeks when clinically stable • paO2 7.3-8 if there is evidence of: • Nocturnal hypoxia • Seconday polycythaemia • Pulmonary HTN • Acute: • ABC • ? ABx if infective exacerbation • Conservative: • Lifestyle – exercise, weight loss, pulmonary rehab • Vaccinations Smoking: All patients with ILD should be advised to stop smoking. Patients with IPF have an up to 10-fold increased risk of developing lung cancer whether they smoke or not LTOT? No evidence that it influences long term survival What are the indications for LTOT?

14. IPF- pathology • Unknown aetiology • Radiologically: bi-basal, peripheral reticulonodular opacities, architectural distortion including traction bronchiectasis and honeycombing. Rarely GGO. • Appearances on the HRCT may be sufficiently characteristic to diagnose • Histology- Usual Interstitial Pneumonia  Patchy fibroblasts with collagen and cystic changes (honeycombing)

15. IPF- prognosis and course • Poor prognosis and relentless course • Mean life expectancy for newly diagnosed cases of between 2.9 and 5 years • Can be complicated by bronchogenic carcinoma • Most people die of respiratory failure (type 1)

16. IPF- management • “Best supportive care” • Symptom management- pulmonary rehab, oxygen therapy, opiates, PPIs, palliative care input • To date no therapy proven to improve survival • Weak recommendation for ‘NAP’ • Transplant list • Clinical trials recruitment

17. “Pneumoconioses” Non-neoplastic lung disease caused by the reaction of the lung to inhalation of mineral or organic dusts. The dust particles reach the terminal airways and settle on the epithelial lining. The inflammatory reaction caused by these leads to inflammation and scarring Jobs to ask about: coal mining, sandblasting, miling, welding, foundry work, farming, working with grain Hobbies to ask about: bird keeping, hot tubs/ sauna Some of the unusual ones: cheese washer’s lung, thatched roof disease, Japanese summer house HP…

18. Hypersensitivity Pneumonitis

19. Hypersensitivity Pneumonitis • Inhaled allergens eg spores/avian protein • Provokes hypersensitivity (allergic) reaction • Histology: lymphocytes and non-caseating granulomas, bronchocentric • Precipitins- IgG to allergens • What is the role? • Radiology- GGO Partial filling of air spaces by exudate as well as interstitial thickening

20. Hypersensitivity Pneumonitis • Causes (>300 known!): Pigeon/Bird fancier’s; farmer’s/mushroom/malt worker’s lungs; bagassosis (sugar cane fibres) • Fevers, rigors, myalgia, weight loss, SOB • Symptoms start 4-6 hours after exposure to the antigen • Rx: it is reversible if diagnosed early Remove allergen/ PPE, O2 therapy, oral prednisolone

21. Industrial Dusts • CABS • Coal Worker’s pneumoconiosis • Asbestosis • Berylliosis • Silicosis • Group of disorders due to inhalation of mineral dusts • Eligible for compensation through Industrial Injuries Act 1965 • Employed not self employed • Within 3 years of diagnosis, not exposure

22. Asbestos Lung Disease • Inhalation of asbestos fibres used in fireproofing/wire insulation, +++ latency period • Fibrogenicity: White > Brown > Blue • Exposure duration and degree also important • Benign pleural plaques/ diffuse pleural thickening/ asbestosis/ malignant mesothelioma/ bronchial adenocarcinoma • Increased risk of adenocarcinoma with smoking + asbestos Asbestosis- fibrosis of lungs +/- parietal or visceral pleura • dyspnoea, clubbing, fine bilat/ bi-basal end-inspcreps • Rx: Symptomatic

23. Asbestos CTs

24. Malignant Mesothelioma • Tumour of mesothelial cells in pleura, occasionally found in peritoneum/other organs • 96% had exposure >20 years prior • 90% had exposure to asbestos but only 20% has asbestosis • NB: weight loss, chest pain, recurrent pleural effusions, SOB • Dx by histology from pleural Bx • Rx: Symptomatic • Prognosis: Poor, <2yrs

25. Sarcoidosis • Multi-system granulomatous disease of unknown aetiology • Age 20-40yo, Afro-Caribbeans • 90% have lung involvement • Erythema nodosum/ uveitis/ keratoconjunctivitissicca/ hepatosplenomegaly/ dysrhythmias/ CCF/ arthralgia/ polyneuropathy/ meningoencephalitis/ fever/ fatigue/ night sweats • Rx: acute NSAIDs, can recover spontaneously • Steroids if 1. symptomatic or static parenchymal disease, 2. uveitis, 3. hypercalcaemia, 4. neurological or cardiac involvement

26. Spot diagnosis? Lupus pernio Erythema nodosum

27. Sarcoidosis Prognosis:60% thoracic involvement have spontaneous resolution, 20% respond to steroids Sarcoidosis of the lungs can be divided into four stages. Stage 0 - No intrathoracic involvement. Stage I - Bilateral hilar adenopathy. Stage II - Pulmonary parenchyma involved. Stage III - Pulmonary infiltrates with fibrosis

28. Take Home Messages • Key symptoms- SOB, dry cough, reduced exercise tolerance • Flags- occupation, pets, hobbies, PMHx • Investigations- think logically and start at the bedside, some specific tests for extra marks • You should be able to mention at least the essential tests the BTS recommends • Management- varies considerably, don’t forget the basics ABC/ stop smoking/ pulmonary rehab/ oxygen

29. More reading • http://www.brit-thoracic.org.uk/guidelines-and-quality-standards/interstitial-lung-disease-guidelines/ • Kumar and Clark page 935-947 • http://www.pneumotox.com/pattern/view/8/I.g/pulmonary-fibrosis/ • OHCM • Patient.co.uk professional reference

30. Some Finals Questions • What are the clinical findings that would suggest ILD? • Explain to the patient what spirometry/ CT/ VATS biopsy/ Echo involves • What is the role of echo in ILD? • What are the changes of ILD on CXR/ CT? • What is a HRCT? • What changes would you expect on spirometry? • Draw the flow volume loop in a normal patient and that of a patient with restrictive lung disease • What are the indications for LTOT? • What is the role of serum precipitins? • What sort of jobs will you ask this patient about? • What are some of the lung diseases asbestos can cause? • What are the systemic manifestations of Sarcoid? • What are the types of inhaler you know and what colour are they?

31. My Finals Cases 1. Schizophrenia Predisposing factors, management including psychotherapy, what is risperidone, explain to patient why they should stay on their depot 2. Unknown gastro complaint Differentials, investigations, tell me about IBD, what is the difference between Crohn’s and UC, explain to the patient about a colonoscopy 3. Aortic stenosis (asymptomatic) What causes, draw the cardiac cycle in terms of pressures, endocarditis, explain to patient about AS 4. MALT lymphoma Differentials, investigations, different types lymphoma, staging, virus involved lymphoma, other diseases associated EBV, histological findings of HL, side effects of chemotherapy, side effects of steroids, explain to the patient about chemotherpay

32. Contact me gracepink88@gmail.com