Interstitial Lung Disease/Diffuse Parenchymal Lung Disease

-Various etiologies  extensive fibrosis of lung  restrictive lung that does not oxygenate well

Presentation:
-usually chronic and insidious development of hypoxemia +/- dry cough +/- dry crackles,
restrictive pattern
Clinical presentation: sx = exertional dyspnea  dyspnea at rest, persistent non-productive
cough, fatigue, digital clubbing due to chronic hypoxia

Xray: reticular most common, nodular (normal in 10% esp hypersensitivity)  high res CT best
for DPLD: ground glass opacities, honeycombing, irregular thickening of interlobular septa
Labs: ABG shows high A-a gradient, decreased PO2, screen for autoimmune disease

PFTs: r/o obstructive lung disease: FEV1/FVC normal, but numbers normal, low DLCO
Best dx: biopsy: via bronchoscopy vs. VATS
TX: steroids—prognosis generally poor, DMARDs (azathioprine, colchicine, cyclophosphamide,
methotrexate, etc.), biologics rarely used
Effective: supplemental O2, pulmonary rehab, vaccination for influenza, pneumococcal vaccine,
eventually palliateive care/hospice
Two agents slow

Idiopathic: acute interstitial pneumonitis

Chronic: idiopathic pulmonary fibrosis

Drug-induced: bleomycin, busulfan, amiodarone, radiation

Rheumatologic disease: SLE, RA, SS
Sarcoidosis: autoimmune (asx bilateral hilar lymphadenopathy or insidious hypoxemia  ILD
+/- heart block, Bell’s palsy, erythema nodosum, non-caseating granulomas) Tx = steroids

Exposure related:
Asbestosis: shipyard/construction: asbestos bodies for 30 years—pleural plaques: deposits of
hyalinized collagen fibers in parietal pleura; biopsy w/ Barbell bodies w/ asbestosis but no tx
besides smoking cessation
Hypersensitivity Pneumonitis: antigen mediated—takes 24-48hr to set in after exposure for
fevers, chills, SOB, abn cxr, but relief within 24-48hr when exposure removed (birds, feathers)—
REMOVE exposure
Pneumoconiosis: silicosis (sandblasting, rock quarries r/o TB b/c in upper lung lobes w/ nodules)
berylliosis (aeronautics/electronics); coal miner’s lung (Kaplan’s syndrome: arthralgias +
pulmonary fibrosis: get RF + anti-CCP , RA)

Eosinophilic pneumonitis: hx of allergic rhinitis and asthma,

Eosinophilic granulomatosis w/ polyangiitis (Churg-Strauss) vs. Wegener’s
Immunocompromised state: may suggest infectious etiology
Diffuse alveolar hemorrhage:
Granulomatosis w/ polyangiitis (Wegener’s) c- ANCA; MP or eosinophilic gwp (Churg-Strauss),
Goodpasture’s (anti-GBM ab)
Diffuse alveolar hemorrhage: grossly bloody or blood-streaked sputum
Macrolides as prophylaxis in ILD: studied in ILD in limited retrospective reviews and animal
models –review from European respiratory review
-antifibrotic agents, promote autophagy and clearance of IC protein aggregates
-immunomodulatory effect (similar argument as in COPD)
-modulators of lung microbiota (changes in microbiota associated with progression of disease in
IPF)

IPF specifics:
-acute exacerbations: worsened sx, CT chest w/ new bilateral GGO and/or consolidation (5-10%
of pts /year), high mortality rate
-Gender-Age-Physiology model = prognostication: (FVC, DLCO into scoring system predictive
of 1-3 year mortality)
TX: steroids—prognosis generally poor, DMARDs (azathioprine, colchicine, cyclophosphamide,
methotrexate, etc.), biologics rarely used (bosetan = endothelin receptor antagonists—antifibrotic
but BUILD 1 trial w/o difference, sildenafil used in pulmonary hypertension which happens with
IPF but no benefit
Effective: supplemental O2, pulmonary rehab, vaccination for influenza, pneumococcal vaccine,
eventually palliative care/hospice
Two agents slow disease: nintedanib (tyrosine kinase inhibitor that mediates FGFs) 150mg bid,
tomorrow and inpulsis trials (decreases progression of FVC and exercise capacity) + pirfenidone
(antifibrotic agent)
Definitive tx = lung transplant DLCO <40, FVC <80, functional limitation, SpO2 <89%
Median survival= two to five years
Usual interstitial pneumonia UIP is characterized by heterogeneous (separated by time and
space) lung abnormalities consisting of subpleural honeycombing (arrowhead), reticular
opacities, and traction bronchiectasis

Nonspecific interstitial pneumonia: NSIP demonstrates homogeneous lung involvement with

predominance of ground-glass opacity combined with sub-pleural linear opacities and
micronodules
**Better response to corticosteroids**

https://pubs.rsna.org/doi/full/10.1148/rg.273065130