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Interstitial lung disease Dr Felix Woodhead Consultant Respiratory Physician.

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1 Interstitial lung disease Dr Felix Woodhead Consultant Respiratory Physician

2 Restrictive Defect “Small lungs” vs “Wheezy lungs” (obstructive) Intrinsic lung disease –abnormal radiology –↓TLco Extrathoracic restriction –normal radiology –normal TLco –? ↑Kco (↓V A → TLco/V A ↑)

3 Extrathoracic Restriction Soft tissues –Obesity –BMI not weight Muscles –Diaphragm > intercostals –Orthopnoea –Sitting/lying FVC Thoracic cage –Scoliosis > kyphosis Pleural thickening

4 Radiological patterns 1 Pleural effusions/mass –Dense white with no air bronchograms –Meniscus –Beware the supine effusion Tumours –Spiculated & single (primary) vs round & multiple (mets) –May present as collapse Collapse –Volume loss (shift of fissure/hilum) –Obliteration of adjacent silhouette

5 Alveolar space filling (≡ consolidation) Fairly dense, with air bronchograms (patent airways) –Neutrophils ± microrganisms (pneumonia/organising pneumonia) –Eosinophils (eosinophilic pneumonia) –Blood (pulm haemmorhage) –Fluid (severe pulm oedema) –Surfactant (alveolar proteinosis) –Tumour (bronchoalveolar carcinoma – BAC)

6 Interstitial shadowing Less dense than consolidation, dots and lines on CXR (reticulonodular) –Interstitial fluid (pulm odema) –Trapped lymph (lymphangitis carcinomatosis) –Inflammation/fibrosis Interstitial lung disease Diffuse parenchymal lung disease Fibrosing alveolitis “pulmonary fibrosis”

7 An approach to chest radiographs Normal or abnormal? If abnormal, how long? If consolidation ?pneumonia –treat with antibiotics and repeat film 6-8/52 If interstitial ?pulm oedema –Treat with diuretics and repeat film in a few days Remember –Not all LRTIs are pneumonia (bronchitis/bronchiectasis) –Pulm oedema also seen with fluid resus and renal pts Long-standing shadowing ?diffuse parenchymal lung disease

8 CT radiology terminology Fleischner Society: Glossary of Terms for Thoracic Imaging Radiology ConsolidationDense, white opacity, obliterating vessels Ground glassLess dense, grey. Alveolar filling or fibrosis ReticulationThickened septal lines, usually indicates fibrosis HoneycombingCysts, usually basal and peripheral. Typical in IPF Traction bronchiectasis Airways in abnormal lung pulled apart. Indicates fibrosis Secondary pulm lobule Smallest part of lung surrounded by connective tissue. Central arteriole/bronchiole, periph venule/lymphatics/septal thickening MosaicismPatchy ground glass often of sec pulm lobules. Airways, vessels or interstitium

9 Myths about ILD ILD is always pulmonary fibrosis All ILD is the same IPF and CFA is the same There is no treatment for ILD

10 Respiratory Physiology Restrictive spirometry –FEV1/FVC ratio ≥ 70% –FVC < 80% –But may have normal levels Reduced TLC & RV Reduced TLco & Kco Serial change crucial

11 Interstitial Lung Disease caused by exposure to Inorganic dusts –Pneumoconiosis –Asbestos, silica, coal dust Organic dusts –Hypersensitivity pneumonitis (HP) –Prev called Extrinsic Allergic Alveolitis (EAA) –Birds, moulds etc etc Drugs –Chemotherapeutic, methotrexate, amiodarone, others –

12 ILD associated with Rheumatological Disease Rheumatoid (RF, anti-CCP) Systemic Sclerosis –Skin thickening ± internal organ fibrosis –Anticentromere (ACA), anti-topoisomerase 1 (ATA, prev Scl-70) Idiopathic Inflammatory Myositis –Polymyositis (muscle) (Jo-1) –Dermatomyositis (skin and muscle) SLE less commonly chronic ILD –acute pneumonitis & pleural disease more common Sjögren’s Syndrome – Ro, La (SSA,SSB) –Less commonly present with ILD

13 Idiopathic Interstitial Pneumonitis Cryptogenic Fibrosis Alveolitis (UK) Idiopathic Pulmonary Fibrosis (USA and elsewhere) Idiopathic disease usually does worse than secondary Idiopathic disease phenotypically very variable Idiopathic disease can be classified by surgical lung biopsy into the “IPs”

14 The “IPs” _ Interstitial Pneumonitis UIPUsualThe most common or usual form of IIP, characterises IPF NSIPNon-specificIndeterminate AIPAcuteRapid onset, Diffuse alveolar damage, ARDS “Hamman-Rich Syndrome” DIPDesquamativeAlveoli full of ‘desquamed’ alveolar cells Now recognised to be macrophages smoking related LIPLymphocyticSeen in HIV and conective tissue disease Esp Sjögrens

15 2002 ATS/ERS guidelines for Idiopathic Interstitial Pneumonia Am J Respir Crit Care Med 2002Nicholson et al Am J Respir Crit Care Med 2000

16 Common DPLDs

17 Idiopathic Pulmonary Fibrosis (IPF) Not interchangeable term for IIP (a subtype of it) ?most common IIP Relatively poor prognosis Median survival 2 ½ years Histology –Usual Interstitial Pneumonitis (UIP) when biopsied –Temporal and Spatial heterogeneity, not uniform Radiology –Typical features: basal, peripheral honeycombing, little GGO –May have any appearance –Typical features – don’t need to biopsy, atypical – biopsy helpful





22 (Cryptogenic) Organising Pneumonia - COP Was also called Bronchiolitis-obliterans OP (BOOP) Histology –Buds of organising inflammatory material in terminal bronchioles and airspaces –Characteristic lesion is the Masson body Radiology –Consolidation ± GGO –May fibrose (?overlap with NSIP) Usually responds well to steroids initially




26 Acute Interstitial Pneumonia



29 Hypersensitivity Pneumonitis Prev called EAA Variety of inhaled stimuli, can occur with drugs too Check precipitins (IgG to allergens) Histology –Lymphocytic –Poorly-formed granulomata –Bronchocentric Radiology –Variety of appearances inc similar to IPF –Typical appearance mosaicism due to small airways involvement BAL may be lymphocytic



32 Non-specific Interstitial Pneumonitis A holding diagnosis Histology –Appearances not typical for other diagnoses esp UIP –Temporally and spatially uniform in all samples –UIP in 1 and NSIP in 2 of 3 biopsies = UIP Radiology –Typically more GGO than IPF –Often fibrosis –Varied Clinical –Varied outcomes –May overlap with IPF, HP or COP? –Typical finding in CTD, many pts with idiopathic NSIP have CTD features

33 Smoking-related IIP Respiratory Bronchiolitis –Histological appearance in ‘healthy smokers’ –Pigmented macrophages in terminal bronchioles –Clinical condition RB associated ILD (RBILD) –RBILD radiology similar to HP –BAL not lymphocytic DIP –‘Desquamative’ due to erroneous belief cells shed into alveoli –Diffuse collection of pigmented macrophages throughout alveoli –Radiologically typified by diffuse GGO ?smoking related NSIP, Fibrosis and emphysema




37 Sarcoidosis Often doesn’t need CT Scadding CXR stages of limited use CT appearances –Perihilar ‘Snow splats’ –Beading of fissures –Lymphadenopathy –May have atypical features Histology typical –Noncaseating granulomata –Worth doing transbronchial and endobronchial biopsies BAL often lymphocytic



40 ILD hints CXRs tell you how long abnormality present and progress History esp CTD features vital (trumps biospy etc) CTs may be pathognomic BAL helpful in HP/sarcoid Phenotypically heterogeneous Expert advise needed and MDT Open biopsy may be helpful after expert advise Treatment uncertain, ?trials

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