Monday, September 25, 2017

Cystic Lung Disease

This post is derived from notes I took during training. Any images are copyright their respective owners.

Cystic Lung Disease

  • Lymphangioleiomyomatosis
    • Demographics: reproductive age women
    • Pathophysiology: mutations of TSC1 & 2. non-neoplastic hamartomatous proliferation of atypical muscle cells that leads to progressive cystic destruction of lung parenchyma
    • Associated with recurrent pneumothoraces, chylous effusion, tuberous sclerosis, renal angiomyolipomas, autosomal dominant polycystic kidney disease (gene location closeby)
    • Findings: 2 mm thick walled cysts, diffuse smooth walled, without other features of interstitial lung disease
    • Treatment: sirolimus

  • Pulmonary Langerhans cell histiocytosis
    • Inhalational disease associated with smoking
    • Lab findings: CD1a, S-100+. Electron microscopy: Birbeck granules
    • Findings: upper lobe predominant. Nodules up to 1 cm which cavitate with varied shape “cysts” (really, distorted airways). Spares costophrenic angles. Associated with recurrent pneumothorax

  • Lymphocytic interstitial pneumonia (follicular bronchiolitis)
    • Demographics: Men
    • Pathophysiology: ball-valve mechanism, peribronchovascular lymphoid aggregates
    • Findings: Mid/lower lobe ground glass opacities, centrilobular/subpleural nodules, thickening of septa, perivascular varying 3 - 20 mm cysts with basilar predominance and < 20 in number



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