ILD

1
ILD

• Rachel Cheong and Grace Pink
• FY1

2
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

3
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!

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

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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

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O/E

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

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How will you investigate?

• According to BTS guidelines
• Urine dip
• FBC, UE, LFTs
• Spirometry and gas transfer
• CXR and HRCT (especially if normal CXR)
• 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

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CXR/ Is that a whistle??

• CXR changes
• Reticulonodular shadowing
• Loss of volume
• Widespread/ bilateral

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HRCT- Whats HR?
CT changes honeycombing, GGO, loss of lung
architecture, traction bronchiectasis
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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

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Spirometry
Restrictive defect FVC is reduced FEV1 is reduced
in proportion or slightly less FEV1FVC ratio
normal or raised
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General Management

• LTOT
• paO2 lt7.3 on 2 occasions separated by 2-3 weeks
  when clinically stable
• paO2 7.3-8 if there is evidence of
• Nocturnal hypoxia
• Seconday polycythaemia
• Pulmonary HTN

• 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?

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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)
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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)

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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

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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 washers lung,
  thatched roof disease, Japanese summer house HP

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Hypersensitivity Pneumonitis
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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
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Hypersensitivity Pneumonitis

• Causes (gt300 known!) Pigeon/Bird fanciers
  farmers/mushroom/malt workers 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

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Industrial Dusts

• CABS
• Coal Workers 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

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Asbestos Lung Disease

• Inhalation of asbestos fibres used in
  fireproofing/wire insulation, latency period
• Fibrogenicity White gt Brown gt 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

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Asbestos CTs
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Malignant Mesothelioma

• Tumour of mesothelial cells in pleura,
  occasionally found in peritoneum/other organs
• 96 had exposure gt20 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, lt2yrs

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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

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Spot diagnosis?
Lupus pernio
Erythema nodosum
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Sarcoidosis

• Prognosis 60 thoracic involvement have
  spontaneous resolution, 20 respond to steroids

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
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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, dont forget the
  basics ABC/ stop smoking/ pulmonary rehab/ oxygen

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More reading

• http//www.brit-thoracic.org.uk/guidelines-and-qua
  lity-standards/interstitial-lung-disease-guideline
  s/
• Kumar and Clark page 935-947
• http//www.pneumotox.com/pattern/view/8/I.g/pulmon
  ary-fibrosis/
• OHCM
• Patient.co.uk professional reference

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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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My Finals Cases

• 1. Schizophrenia
• Predisposing factors, management including
  psychotherapy, what is risperidone, explain to
  patient why they should stay on their depot
• 2. Unknown gastro complaint
• Differentials, investigations, tell me about IBD,
  what is the difference between Crohns and UC,
  explain to the patient about a colonoscopy
• 3. Aortic stenosis (asymptomatic)
• What causes, draw the cardiac cycle in terms of
  pressures, endocarditis, explain to patient about
  AS
• 4. MALT lymphoma
• Differentials, investigations, different types
  lymphoma, staging, virus involved lymphoma, other
  diseases associated EBV, histological findings of
  HL, side effects of chemotherapy, side effects of
  steroids, explain to the patient about
  chemotherpay

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Contact me

• gracepink88_at_gmail.com