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