ILD Flashcards

1
Q

what is Interstitial Lung Disease (ILD)

what does it include?

A

Umbrella term describing a number of conditions that affect the lung parenchyma in a diffuse manner

I NESS

  1. Usual Interstitial Pneumonia (UIP) (IDIOPATHIC)
  2. Non-specific Interstitial Pneumonia (NSIP)
  3. Extrinsic Allergic Alveolitis
  4. Sarcoidosis
  5. Several other conditions
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2
Q

Principal Features of ILD

A

4 D’s

  • Dyspneo
  • Digital clubbing
  • Dry cough
  • Diffuse inspiratory crackles
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3
Q

Cause of ILD by Location

A
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4
Q

Assoc. c ̄ systemic disease in ILD?

A
  •  Sarcoidosis
  •  RA
  •  SLE, systemic sclerosis, Sjogren’s, MCTD
  •  UC, ankylosing spondylitis
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5
Q

Causes of ILD

A

 Environmental: asbestosis, silicosis

 Drugs: BANS ME

  •  Bleomycin, Busulfan
  •  Amiodarone
  •  Nitrofurantoin
  •  Sulfasalazine
  •  MEthotrexate, MEthysergide
  •  Hypersensitivit*y: EAA
  •  Infection*: TB, viral, fungi
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6
Q

array of investigations include?…

A
  • ANA
  • ENA
  • Rh F
  • ANCA
  • Anti-GBM
  • ACE
  • Ig G to serum precipitins
  • (HIV)
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7
Q

Commonest cause of interstitial lung disease ?

A

Usual Interstitial Pneumonia (UIP)

also called

Idiopathic Pulmonary Fibrosis (CFA)

mainly males

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8
Q

Classical Findings of UIP

A
  • clubbing, reduced chest expansion
  • Auscultation – fine inspiratory crepitations (like pulling Velcro slowly) – usually best heard basal / axillary areas
  • CVS – may be features of pulmonary hypertension
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9
Q

Dx of IPD

A

PFT>> restrictive lung disease

CXR>> interstitial lung edema

biopsy>> UIP

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10
Q

Mx of UIP

A

Nintedanib >>slows disease progression

Pirfenidone>> down regulated coll 1 & 2

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11
Q

what is Extrinsic Allergic Alveolitis?

A

In sensitized individuals >> repetitive inhalation of allergens >> hypersensitivity 3 reaction.

ACUTE EXPOSURE 4-8 hrs

Fever, rigors, myalgia,

alveoli infiltrated w/ acute inflammatory cells.

reversible, spontaneoudly settle 1-3 days, can reocur

CHRONIC EXPOSURE mnth-yrs

Granuloma formation and obliterative bronchiolitis occur.

Less reversible

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12
Q

Causes of EAA

A
  • Bird-fancier’s and pigeon-fancier’s lung (proteins in bird droppings).
  • Farmer’s and mushroom worker’s lung (Micropolyspora faeni, Thermoactino-myces vulgaris).
  • Malt worker’s lung (Aspergillus clavatus).
  • Bagassosis or sugar worker’s lung (Thermoactinomyces sacchari).
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13
Q

Dx of EAA

A

Blood: FBC (neutrophilia); ABGS; serum antibodies (may indicate exposure/previous sensitization rather than disease).

CXR: upper-zone mottling/consolidation; hilar lymphadenopathy (rare).

PFT: Reversible restrictive defect; reduced gas transfer during acute attacks.

CT chest: nodules, ground glass appearance, extensive fibrosis. Bronchoalveolar lavage (BAL) fluid shows lymphocytes and MAST cells.

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14
Q

Management of EAA

A

Acute:

  1. Remove allergen and give O2 (35–60%),
  2. PO prednisolone (40mg/24h PO), reducing course.

Chronic:

Allergen avoidance, or wear a facemask or +ve pressure helmet. Long-term steroids often achieve CXR and physiological im- provement.

Compensation (UK Industrial Injuries Act) may be payable.

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