ILD Rachel Cheong and Grace Pink FY1

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

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!

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

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

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

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

How will you investigate? According to BTS guidelines: 1. Urine dip 2. FBC, U&E, LFTs 3. Spirometry and gas transfer 4. CXR and HRCT (especially if normal CXR) 5. 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

CXR/ Is that a whistle?? CXR changes: 1.Reticulonodular shadowing 2.Loss of volume 3.Widespread/ bilateral

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

Spirometry All patients with ILD should have resting spirometric and 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

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

General Management 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? LTOT: paO2 <7.3 on 2 occasions separated by 2-3 weeks when clinically stable paO if there is evidence of: 1.Nocturnal hypoxia 2.Seconday polycythaemia 3.Pulmonary HTN

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)

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)

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

“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…

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

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

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

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-insp creps Rx: Symptomatic

Asbestos CTs

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

Sarcoidosis Multi-system granulomatous disease of unknown aetiology Age 20-40yo, Afro-Caribbeans 90% have lung involvement Erythema nodosum/ uveitis/ keratoconjunctivitis sicca/ 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

Spot diagnosis? Lupus pernio Erythema nodosum

Sarcoidosis 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 Prognosis: 60% thoracic involvement have spontaneous resolution, 20% respond to steroids

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

More reading standards/interstitial-lung-disease-guidelines/ standards/interstitial-lung-disease-guidelines/ Kumar and Clark page fibrosis/ fibrosis/ OHCM Patient.co.uk professional reference

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?

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