ILD Flashcards
Common drugs
Amiodarone MTX Bleomycin Nitrofurantoin Ritux Aza TNF blockers
Associated CTD
RA
Scleroderma
PM/DM
Sarcoid
Occupational exposure
Asbestos
Silicosis
Clinical features ILD
age of onset
chronic dry cough, progressive SOBOE
usually >60
if a/w CTD, 20-40
ILD - upper zones
Hypersensitivity pneumonitis Sarcoidosis AS Silicosis Coal worker's pneumoconiosis
ILD - lower zones
IPF
CTD
Drug related
Asbestosis
Strongest risk factor ILD
Smoking
Genetic factor
Syndrome
Genes
Associated features
Telomeropathy syndrome (short telomeres) High anticipation
Genes - TERT, TERC, RTEL1, PARN
Premature ageing, Aplastic anaemia, opportunistic inf, cancers, pul fibrosis, OP etc
IPF
CF
Imaging
Smokers, Fly hx, 60s
SOB + cough, progressive
clubbing
Subpleural reticular changes, basal predominance, traction bronchiectasis, honeycombing
Classic UIP pattern
IPF Treatment
Fibrinolytics
has mortality benefit but AE ++, improves symptoms
Pirfenidone - TGF-B inhibitor. Photosensitivity rash, nausea
Nintedanib - TKI, increased bleeding and CVS risk. AE Diarrhoea
?NAC inf in TOLLIP mutation
DONT use immunosuppression
NSIP CT
More ground glass opacity than UIP
No basal predominance
Spares subpleural regions
Dx of exclusion
Gold Standard - Surgical biopsy via VATS or thoracotomy is the gold standard
Cryptogenic Organizing Pneumonia
Can be a/w organ transplant
Peribronchiolar opacities with ground glass changes, no honeycombing
Revers halo or Atoll sign = subpleural sparing
BAL - neuts, eosinophils, lymphocytes, nil bacteria
Sarcoidosis
mediastinal/hilar lymphadenopathy
Pulmonary Alveolar Proteinosis
- Accumulation of surfactant in alveoli and terminal airways
- Can be congenital or acquired
Anti GM-CSF
Milky fluid on BAL - Acid Schiff +ve
Mx - while lung lavage, immunomodulation
Lymphangioleiomyomatosis (LAM)
abnormal proliferation of smooth muscle cells –> destruction of lung –> cysts
young, female, can be part of tuberous sclerosis complex
abdo tumours - angiomyolipomas
Mx - Sirolimus