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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