ILD Flashcards
what is Interstitial Lung Disease (ILD)
what does it include?
Umbrella term describing a number of conditions that affect the lung parenchyma in a diffuse manner
I NESS
- Usual Interstitial Pneumonia (UIP) (IDIOPATHIC)
- Non-specific Interstitial Pneumonia (NSIP)
- Extrinsic Allergic Alveolitis
- Sarcoidosis
- Several other conditions
Principal Features of ILD
4 D’s
- Dyspneo
- Digital clubbing
- Dry cough
- Diffuse inspiratory crackles
Cause of ILD by Location
Assoc. c ̄ systemic disease in ILD?
- Sarcoidosis
- RA
- SLE, systemic sclerosis, Sjogren’s, MCTD
- UC, ankylosing spondylitis
Causes of ILD
Environmental: asbestosis, silicosis
Drugs: BANS ME
- Bleomycin, Busulfan
- Amiodarone
- Nitrofurantoin
- Sulfasalazine
- MEthotrexate, MEthysergide
- Hypersensitivit*y: EAA
- Infection*: TB, viral, fungi
array of investigations include?…
- ANA
- ENA
- Rh F
- ANCA
- Anti-GBM
- ACE
- Ig G to serum precipitins
- (HIV)
Commonest cause of interstitial lung disease ?
Usual Interstitial Pneumonia (UIP)
also called
Idiopathic Pulmonary Fibrosis (CFA)
mainly males
Classical Findings of UIP
- 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
Dx of IPD
PFT>> restrictive lung disease
CXR>> interstitial lung edema
biopsy>> UIP
Mx of UIP
Nintedanib >>slows disease progression
Pirfenidone>> down regulated coll 1 & 2
what is Extrinsic Allergic Alveolitis?
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
Causes of EAA
- 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).
Dx of EAA
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.
Management of EAA
Acute:
- Remove allergen and give O2 (35–60%),
- 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.