Interstitial and Occupational Lung Disease

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Interstitial and Occupational Lung Disease

• Dr Robin Smith
• Dept of Respiratory Medicine

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Interstitial Disease

• Any disease process affecting lung interstitium
  (ie alveoli, terminal bronchi).
• Interferes with gas transfer
• Restrictive lung pattern (may also have some
  airway obstruction if small airways involved)
• Symptoms breathlessness, dry cough

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Interstitial Lung Disease

• Classification
• Acute
• Episodic
• Chronic - part of systemic disease
• - exposure to agent (e.g. drug, dust etc)
• - idiopathic

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Acute ILD

• Infection - usually viral
• Allergy - eg drug reaction
• Toxins - cytotoxic drugs, toxic fumes e.g.
  chlorine
• Vasculitis - eg Wegeners granulomatosis,
  Churg-strauss, SLE, Goodpastures syndrome
• ARDS - trauma, sepsis

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Episodic ILD

• Pulmonary eosinophilia
• Vasculitis (Churg-Strauss, Wegeners, SLE)
• Extrinsic Allergic Alveolitis
• Cryptogenic Organising Pneumonia

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Chronic ILD as part of systemic disease

• Connective Tissue Disease (eg Rheumatoid
  arthritis, SLE, Systemic Sclerosis, Ankylosing
  Spondylosis)
• Vasculitis (Churg-Strauss, Wegeners, SLE)
• Sarcoidosis
• Cancer (lymphoma, lymphangitis carcinomatosis)
• Miscellaneous - tuberose sclerosis, lipid storage
  disorders, neurofibromatosis, amyloidosis,
  miliary TB, bone marrow transplant)

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Chronic ILD - exposure to foreign agent

• Fibrogenic inorganic dusts - coal, silica,
  asbestos, aluminium,
• Non-fibrogenic dust - siderosis (iron), stannosis
  (tin), baritosis (barium)
• Granulomatous/fibrogenic - berylliosis
• Organic dusts - Farmers lung (Microsporylium),
  bagassosis (mouldy sugar cane), Bird fanciers
  lung - (feather and dropping antigen)

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Chronic ILD - idiopathic

• Idiopathic Pulmonary Fibrosis (IPF) also known
  as Cryptogenic fibrosing alveolitis (CFA)
• Cryptogenic organising pneumonia (COP)
• Sarcoidosis
• Alveolar proteinosis
• Lymphangioleiomyomatosis (LAM)
• many other rarer causes

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• SARCOIDOSIS
• Multiple-system disease
• common - lungs, lymph nodes, joints, liver,
  skin, eyes
• less common - kidneys, brain, nerves, heart
• non-caseating granuloma of unknown aetiology
  probable infective agent in susceptible
  individual. Imbalance of immune system with type
  4 (cell mediated) hypersensitivity
• Types
• Acute sarcoidosis
• erythema nodosum, bilateral hilar
  lymphadenopathy, arthritis, uveitis, parotitis,
  fever.
• Chronic sarcoidosis lung infiltrates
  (alveolitis), skin infiltrations, peripheral
  lymphadenopathy, myocardial, neurological,
  hepatitis, splenomegaly, hypercalcaemia.

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• SARCOIDOSIS
• Differential diagnosis TB (tuberculin test
  -ve), Lymphoma, Carcinoma, fungal infection.
• Chest X-ray (BHL), CT scan of lungs (peripheral
  nodular infiltrate)
• Tissue biopsy (eg transbronchial, skin, lymph
  node) ? non-caseating granuloma.
• Pulmonary function Restrictive defect due to
  lung infiltrates.
• Blood test
• - Angiotensin Converting Enzyme (ACE) levels as
  activity marker (NOT diagnostic test).
• - raised calcium
• - increased inflammitory markers
• Acute self-limiting condition.
• Chronic oral steroids if vital organ affected
  (eg lung, eyes, heart, brain).

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• SARCOIDOSIS
• Treatment
• Acute self-limiting condition - usually no
  treatment
• Steroids if vital organ affected (eg impaired
  lung function, heart, eyes, brain, kidneys)
• Chronic oral steroids usually needed
• Immunosuppression (eg azathioprine,
  methotrexate)
• monitor chest X-ray and pulmonary function for
  several years
• often relapses

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Erythema Nodosum-Sarcoidosis
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Iritis due to sarcoidosis
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Bilateral hilar lymphadenopathy and lung
infiltrares -Sarcoidosis
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• EXTRINSIC ALLERGIC ALVEOLITIS I
• Type III hypersensitivity (Immune complex
  deposition) reaction to antigen ? lymphocytic
  alveolitis (hypersensitivity pneumonitis).
• Aetiology Microsporylium (farmers lung, malt
  workers, mushroom workers), avian antigens (bird
  fanciers lung), drugs (gold, bleomycin,
  sulphasalazine)
• Can be ACUTE or CHRONIC
• ACUTE cough, breathless, fever, myalgia -
  several hours after acute exposure (flu-like
  illness)
• Signs /- pyrexia, crackles (no wheeze!),
  hypoxia
• CxR widespread pulmonary infiltrates
• Treatment oxygen, steroid and antigen avoidance

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• EXTRINSIC ALLERGIC ALVEOLITIS II
• CHRONIC ? repeated low dose antigen exposure
  over time ? progressive breathlessness and cough
• Signs may be crackles, clubbing is unusual
• CxR pulmonary fibrosis - most commonly in the
  upper zones
• PFTs restrictive defect (low FEV1 FVC, high or
  normal ratio, low gas transfer - TLCO)
• Diagnosis history of exposure, precipitins (IgG
  antibodies to guilty antigen), lung biopsy if in
  doubt.
• Treatment remove antigen exposure, oral steroids
  if breathless or low gas transfer.

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Extrinsic allergic alveolitisAvian
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• IDIOPATHIC PULMONARY FIBROSIS
• (also known as Cryptogenic Fibrosing Alveolitis)
• Most common interstitial lung disease
• Clinical presentation progressive
  breathlessness, dry cough
• OE clubbing, bilateral fine inspiratory
  crackles,
• Ix restrictive defect (reduced FEV1 and FVC
  with normal or raised FEV1/FVC ratio, reduced
  lung volumes, low gas transfer CxR - bilateral
  infiltrates
• CT scan - reticulonodular fibrotic change, worse
  at the lung bases. The presence of ground-glass
  suggests reversible alveolitis fibrosis is
  irreversible.
• Causes Primary (Idiopathic)
• Secondary (eg rheumatoid, SLE, systemic
  sclerosis, drugs - amiodarone, busulphan,
  bleomycin, penicillamine, nitrofurantoin,
  methotrexate).

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• IDIOPATHIC PULMONARY FIBROSIS II
• Differential diagnosis exclude occupational
  disease (asbestosis, silicosis), mitral valve
  disease, left ventricular failure, sarcoidosis,
  extrinsic allergic alveolitis.
• Ask about occupation (in depth), pets and drugs
• Diagnosis combination of history, examination
  and radiology tests
• If the presentation is atypical then lung biopsy
  (either transbronchial or thoracascopic) is
  needed
• Pathology chronic inflammatory infiltrate
  (neutrophils and fibrosis in alveolar walls
  intra-alveolar macrophages.

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• IDIOPATHIC PULMONARY FIBROSIS III
• Treatment not clear if influences course of
  disease
• oral steroids immunosuppressive drugs (eg
  azathioprine combined with N-acetyly cisteine)
  worth trying if patient is lt75 years and evidence
  of acute inflammation on CT scan - some response
  in 30.
• NB treatment is aimed as slowing future
  progression rather than reversing established
  fibrosis.
• Oxygen if hypoxic.
• Lung transplantation in young patients
• Future treatments ?Anti-fibrotic agents
• ?anti-TNFa
• pulmonary artery vasodilators
• Prognosis most patients progress into
  respiratory failure and are dead within 5 years

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DIP-pre steroids
Fibrosing Alveolitis
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Lymphocytic alveolitis and intralumenal
macrophages
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• COAL WORKERS PNEUMOCONIOSIS
• Simple pneumoconiosis - chest X-ray abnormality
  only (no impairment of lung function - often
  associated with chronic obstructive pulmonary
  disease).
• Complicated pneumoconiosis - progressive massive
  fibrosis ? restrictive pattern with
  breathlessness.
• Chronic bronchitis (coal dust smoking).
• Caplans syndrome - rheumatoid pneumoconiosis
  (pulmonary nodules).

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• SILICOSIS
• 15-20 years exposure to quartz (eg mining,
  foundry workers, glass workers, boiler workers).
• Simple pneumoconiosis - chest X-ray abnormality
  only (egg-shell calcification of hilar nodes).
• Chronic silicosis - restrictive pattern,
  pulmonary fibrosis.

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Coal workers progressive massive fibrosis
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Coal workers progressive massive fibrosis
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Baritosis
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• ASBESTOS-related lung disorders
• Mining, construction, shipbuilding, boilers and
  piping, automotive components (eg brake linings).
• Pleural disease -
• 1- Benign pleural plaques - asymptomatic
• 2- Acute asbestos pleuritis - fever, pain,
  bloody pleural effusion
• 3- Pleural Effusion and Diffuse pleural
  thickening - restrictive impairment
• 4- Malignant Mesothelioma - incurable pleural
  cancer. Presents with chest pain and pleural
  effusion. No available treatment - fatal within
  two years.
• Pulmonary Fibrosis - Asbestosis - heavy
  prolonged exposure. Diffuse pulmonary fibrosis
  and restrictive defect. Asbestos bodies in
  sputum. Asbestos fibres in lung biopsy.
• Bronchial carcinoma - asbestos multiplies risk in
  smokers

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Asbestosis
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Asbestos pleural plaques and Bronchial Ca
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Pleural effusion due to mesothelioma
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• OCCUPATIONAL ASTHMA
• Sensitising agents - high molecular weight (eg
  bakers, enzymes, gums, latex) - low molecular
  weight (eg isocyanates, wood dust,
  glutaraldehyde, solder, flux, dye, adhesives,
  drugs).
• Diagnosis RAST test, provocation testing, PEFR
  at home/work.
• Reactive airway dysfunction syndrome - acute
  episode of toxic gas or fume inhalation (eg
  chlorine or sulphur dioxide) ? followed by
  persistent bronchial hyperreactivity.

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hhhttp//www.radiology.co.uk/srs-x/index.htm
guidelines)
Useful clinical X-ray teaching sites
http//www.brit-thoracic.org.uk/clinical-informati
on/interstitial-lung-disease-(dpld)/interstitial-l
ung-disease-(dpld)-guideline.aspx