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IRM lectures > L7- interstitial lung disease > Flashcards

L7- interstitial lung disease Flashcards

1
Q

what is the pulmonary interstitum?

A

Interstitial compartment is the portion of the lung sandwiched between the alveolar epithelium and capillary endothelial basement membrane

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2
Q

What is pulmonary fibrosis?

A
  • Scarring of lung parenchyma
  • usually after inflammation
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3
Q

What does gas exchange require?

A
  • alveolar ventilation- air to get into the alveolus, impairment can be central or mechanical
  • pulmonary circulation- blood circulating the alveolus
  • functioning alveoli- gas exhange can occur
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4
Q

What would the spirometry of a patient with pulmonary fibrosis show?

A

restrictive spirometry

  • low FVC
  • Normal/high FEV1/FVC
  • low RV(residual volume)
  • low TLC
  • low DLCO- measure gas exchange in the alveolar level
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5
Q

What is the aetiology of pulmonary fibrosis

A

Widespread- diffuse throughout the lungs

  1. drugs
  2. carcinomatosis (cancer)

Upper zone

  1. TB
  2. Ankylosing spondylitis
  3. Sacoidosis
  4. Extrinsic Allergic Alveolitis-hypersensitivity pneumonitis-inhalation of irritant substances- birds or moulds

Lower zone

  1. Cryptogenic/idiopathic fibrosis
  2. Connective tissue disease- e.g. RA, systemic lupus, autoantibody screen to find evidence of connective tissue disease
  3. Occupational- asbestosis
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6
Q

What are the clinical features of pulmonary fibrosis?

A
  • Dry cough and dyspnoea
  • fine end inspiratory creps at base of lungs
  • of the cause
  • cryptogenic/idiopathic
    • usually middle age
    • men>women
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7
Q

What would a chest x ray and CT of a patient with pulmonary fibrosis show?

A
  • reticular shadowing
  • Honeycombing
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8
Q

What is the management for pulmonary fibrosis?

A

Depends on cause, histology, severity and rapidity of progression

  • Usual Interstital Pneumonia UIP- pirfenidone or nintendanib
  • Autoimmune- immunouppression
  • Transplantation
  • Monitor using pulmonary functions tests
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9
Q

When is biopsy needed for pulmonary fibrosis?

A
  • Not clearly UIP (Usual Interstitital Pneumonia)/ idiopathic pulmonary fibrosis on High Resolution CT
  • no clear cause on antibody tests/history
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10
Q

What are the methods of taking a lung biopsy?

A
  • Bronchoscopic washings (BAL)+ transbronchial biopsy
  • Surgery (VATS- video-assissted thoracoscopic surgery)
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11
Q

What are th common radiological and pathological findings in UIP/ idiopathic pulmonary fibrosis?

A
  • Predominently Basal and sub-plueral fibrosis
  • dense scarring
  • Honeycombing
  • Not much ground glass in CT
  • also Nil on antibodies blood test
  • no asbestos exposure in history
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12
Q

What are the common radiological and pathologicl features of NSIP (Non Specific Interstital Pneumonitis?

What is the diagnosis and prognosis?

A
  • No honeycombing
  • only mild inflammatory changes
  • Bronchoscopic washings for diagnosis- BALS
  • Better prognosis than UIP
  • Anything non specific can cause NSIP, i.e. asbestos, connective tissue disorders
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13
Q

What are the other causes of pulmonary fibrosis

A
  • RB-ILD ( respiratory bronchitis related Interstitial lung disease), DIP- Desquamative interstitial Pneumonia
    • smoking related
    • look for emphysema
    • treat by smoking cessation
  • COP- Cryptogenic Organizing Pneumonitis
    • acute onset
    • Look for Rheumatoid Arthritis
    • Respond to steroids
  • LIP- Lymphocytic Interstitial Pneumonia
    • cellular NSIP
    • Autoimmune causes
    • Very rare
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14
Q

What is Extrinsic Allergic Alveolitis?

A

Cause- Inhalation of organic particles

  • Farmer’s Lung- hay fungi
  • Bird Fancier’s Lung- bird proteins
  • Mushroom worker’s Lung- various fungi
  • antibody formation (precipitins) and T cell senstisation
    • leads to type III/type IV hypersensitivity lung damage
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15
Q

Classifiction of EAA

A
  1. Acute
    • presentation- short and intense
    • may be confused with asthma or bronchitis
    • 4-8hr post exposure, dry cough, hypoxia, fevers, myalgia
    • substantial improvement 1-2 day, Complete recovery 7-10 days
  2. Chronic, maybe confused with idiopathic pulmonary fibrosis
    • presentation- Long and low grade
    • fibrosis
    • progressive dyspnoea
    • irreversible lung damage
  3. Sub-acute (acute on chronic)
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16
Q

Daignosis for EAA/ hypersentivity pneumonitis

A
  • Identification of potential antigen
  • characteristic clinicl and radiological findings
    • CXR, HRCT- UL fibrosis (upper lobe fibrosis)
    • Blood- positive avian/aspergillus precipitins, IgE
17
Q

Treatment of EAA

A
  • Antigen avoidance
  • steroids
18
Q

Causes of Granulomatous Lung Disease

A
  • Infectious
    • TB
    • Fungi- Histoplasmosis, Blastomycosis/ Coccidiodomycosis
  • Inflammatory
    • Sarcoidosis
    • EAA
  • Vasculitic
    • Churg-Strauss
    • Wegener’s
    • Polyartetitis nodusa
19
Q

what is sarcoidosis?

A
  • Mulrisystem disease unknown aetiology
  • non- caseating granulomas
  • 3 times more common in afro-caribbean
  • 1/3 get better without treatment
  • 1/3 get better with steroids
  • 1/3 need long term steroids/ immunosuppressants
20
Q

What are the stages of pulmonary sarcoidosis and what is the radiological staging

A
  • Stage 0- normal CXR
  • Stage 1- BHL- Bilateral Hilar Lymphadenopathy
    • equal degree of enlargement of lymph nodes at ‘root’ of both sides of lungs
  • Stage 2- BHL+ infiltrates
    • above + expansion into and throughout lung tissue
  • Stage 3- only infiltrates
    • disease process spread througout lung tissue, no lymph nodes enlargement
  • Stage 4- diffuse fibrosis
    • small lung fields. scaring, retraction of both hila
21
Q

Characteristics of Lofgren’s syndrome

A

Triad

  1. BHL- bilateral hilar lymphadenopathy
  2. Erythema nodosum
  3. Polyarthralgia
22
Q

What other organs may be affected by sarcoidosis?

A
  • Skin
  • eyes
  • heart
  • liver
  • neuro
23
Q

Investigations and treatment fo Sarcoidosis

A

Investigations

  • Full lung function
  • HRCT
  • ACE- marker of inflammation, non-specific
  • ECG

Treatment- steroids

24
Q

What is vasculitis?

A
  • inflammation of blood vessels
  • may obstruct vessels or cause bleeding
  • often multisystemic, commonly renal
  • Example
    • wegner’s granulomatosis
    • Goodpasture’s disease
25
Q

What is pulmonary haemorrage?

  • symptoms
  • CXR
  • PFT
A

Symptoms

  • Dyspnoea
  • Haemoptysis- coughing up blood
  • Opaque on CXR
  • PFTs- high gas transfer
  • visualise at bronchoscopy
26
Q

What are the effects of Wegner’s on upper airway and lungs?

What is the diganostic test?

A

Upper airway

  • Granulomas which then bleed
  • Sinisitis
  • Saddle nose deformity due to septal destruction

Lungs

  • Haemorrhage
  • Cavities

Positive ANCA antibody test

27
Q

What is the lung interstitium?

A
  • not normally visible radiogeaphically, visible in disease
  • continuum of loose connective tissue throughout the lung
  • 3 subdivisions
    • bronchiovascular- surrounding the bronchi, arteries and veins from the lung root to the level of respiratory bronchiole
    • Parenchymal (acinar). between alveolar and capillary basement membranes
    • Subpleural- beneath the pleura and in the interlobular septae
28
Q

What is meant by the following:

  1. interstitial lung disease (ILD)
  2. Cryptogenic fibrosing alveolitis (CFA)
A
  1. umbrella term for all conditions that cause interstital fibrosis
  2. Also known as Idiopathic pulmonary fibrosis, a form of fibrosis with unknown aetiology

IRM lectures (19 decks)

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