Restrictive Lung Disease Flashcards
What characterizes restrictive lung disease?
Difficulty filling the lungs, decreased compliance, FVC and FEV1, normal FEV1/FVC ratio, decreased diffusion capacity
List common causes of restrictive lung disease.
Interstitial lung disease, pneumoconiosis, sarcoidosis
What are typical histologic patterns in restrictive lung disease?
Usual Interstitial Pneumonia (UIP), Non-Specific Interstitial Pneumonia (NSIP), Organizing Pneumonia, Granulomas, Honeycomb lung
What are common pathologic features of interstitial lung disease (ILD)?
Increased inflammatory cells, fibroblasts, collagen, alveolar epithelial changes, honeycombing in end stage
What age group is most affected by usual interstitial pneumonia (UIP)?
Most commonly presents in 40s-60s
What is the typical clinical course of UIP?
Insidious onset with slow progression, poor response to therapy, 50% 5-year survival rate
Describe the histologic pattern of UIP.
Patchy distribution with interstitial fibrosis and fibroblastic foci
What is honeycombing in the context of lung disease?
End-stage fibrosis with cystic spaces, often seen in lower lobes and subpleural areas
Where is the fibroblastic focus located in UIP?
In the interstitium, not the airspace
Describe the pattern in non-specific interstitial pneumonia (NSIP).
Diffuse interstitial fibrosis, lower lobe predominance, 5-year survival 75-85%
What are the common causes of NSIP?
Often related to collagen vascular disease, drug injury, resolving infection, idiopathic
What are granulomas?
Aggregations of histiocytes, sometimes with multinucleated giant cells
What is hypersensitivity pneumonitis?
Immune response to inhaled organic dusts, leading to interstitial inflammation and fibrosis
Name some triggers for hypersensitivity pneumonitis.
Farmer’s lung, Pigeon breeder’s lung, hot tub use
Describe the histology of hypersensitivity pneumonitis.
Poorly formed non-necrotizing granulomas with lymphocytic inflammation and fibrosis in the interstitium
