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Interstitial lung disease

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Presentation on theme: "Interstitial lung disease"— Presentation transcript:

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 (↓VA → TLco/VA ↑)

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
Consolidation Dense, white opacity, obliterating vessels Ground glass Less dense, grey. Alveolar filling or fibrosis Reticulation Thickened septal lines, usually indicates fibrosis Honeycombing Cysts, 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 Mosaicism Patchy 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
UIP Usual The most common or usual form of IIP, characterises IPF NSIP Non-specific Indeterminate AIP Acute Rapid onset, Diffuse alveolar damage, ARDS “Hamman-Rich Syndrome” DIP Desquamative Alveoli full of ‘desquamed’ alveolar cells Now recognised to be macrophages smoking related LIP Lymphocytic Seen in HIV and conective tissue disease Esp Sjögrens

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

18 IPF CXR

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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

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26 Acute Interstitial Pneumonia

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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

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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 DIP
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

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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

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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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