Interstitial lung disease (ILD) or Diffuse parenchymal lung disease (DPLD)

Most common
 Idiopathic pulmonary fibrosis
(50%)
 Hypersensitivity pneumonitis
 Connective tissue disease
associated interstitial lung
disease
 Sarcoidosis

Symptoms (nonspecific) = Dyspnea

or a persistent nonproductive cough

Workup
Careful documentation of PMH - history of smoking, medication, occupational/environmental exposure, FH
Spirometry = restrictive pattern (reduced total lung capacity and forced vital capacity)
Lung examination — nonspecific
o Crackles at lung base
o Late inspiratory high-pitched rhonchi in traction bronchiectasis due to pulmonary fibrosis
Cardiac examination — normal except in advanced pulmonary fibrosis = Findings of pulmonary HTN
Clubbing - late manifestation and suggests advanced fibrosis of the lung
Labs = CBC, UA, LFTs, BUN, Cr, serology (ANA, RF, etc)
Lung biopsy

Imaging = Chest XR or High-Resolution CT – best

 Honeycombing >8% is highly specific
Reflects stage and severity of disease
 Reticular opacities in subpleural lung
Often associated with honeycombing and
traction bronchiectasis
Peripheral and lower lobe predominance
 Ground-glass opacities
 Lung architectural distortion = lung fibrosis
 Lobar volume loss (lower) = advanced fibrosis

DDX with IPF on HRCT

1) Non-specific interstitial pneumonia pattern (NSIP)
2) Chronic hypersensitivity pneumonitis (HP)
Involves the mid and upper zones of the lung
Presence of centrilobular nodules and areas of
air trapping
3) Amiodarone lung fibrosis = Presence of hyperdense
pulmonary nodules or hyperdense liver on a non-
contrast CT
4) Systemic sclerosis = Presence of patulous
esophagus and correlation with hand radiographs
5) Asbestosis = Bilateral pleural plaques with or without calcification or peritoneal calcification
6) Combined pulmonary fibrosis and emphysema (CPFE): especially if there is added upper lobe-predominant
emphysema