Interstitial lung disease

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Interstitial lung disease

• Dr Felix Woodhead
• Consultant Respiratory Physician

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Restrictive Defect

• Small lungs vs Wheezy lungs (obstructive)
• Intrinsic lung disease
• abnormal radiology
• ?TLco
• Extrathoracic restriction
• normal radiology
• normal TLco
• ? ?Kco (?VA ? TLco/VA ?)

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Extrathoracic Restriction

• Soft tissues
• Obesity
• BMI not weight
• Muscles
• Diaphragm gt intercostals
• Orthopnoea
• Sitting/lying FVC
• Thoracic cage
• Scoliosis gt kyphosis
• Pleural thickening

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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

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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)

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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

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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/bronchiect
  asis)
• Pulm oedema also seen with fluid resus and renal
  pts
• Long-standing shadowing ?diffuse parenchymal lung
  disease

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CT radiology terminologyFleischner Society
Glossary of Terms for Thoracic Imaging

Radiology 2008 246 697-722
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
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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

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Respiratory Physiology

• Restrictive spirometry
• FEV1/FVC ratio 70
• FVC lt 80
• But may have normal levels
• Reduced TLC RV
• Reduced TLco Kco
• Serial change crucial

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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
• www.pneumotox.com

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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
• Sjögrens Syndrome Ro, La (SSA,SSB)
• Less commonly present with ILD

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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

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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
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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
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Common DPLDs
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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 dont need to biopsy, atypical
  biopsy helpful

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IPF CXR
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(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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Acute Interstitial Pneumonia
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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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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

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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

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Sarcoidosis

• Often doesnt 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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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