Restrictive Diseases Flashcards
Basic Concept/Cause/Spirometry of Restrictive Lung Disease
Restricted filling of the lung, usually due to interstitial diseases but maybe chest wall abnormalities like morbid obesity (like Ronak)
Decreased TLC, decreased FVC and FEV1, but FVC more so FEV1:FVC ratio INCREASED (>80%)
Idiopathic Pulmonary Fibrosis (pathophys/mediator, radiology, and treatment)
Etiology unknown but likely cyclical lung injury and healing. TGF-Beta induces fibrosis
Fibrosis on CT, initially in subpleural patches but eventually diffuse “honeycombing”
Treatment is lung transplant
Pneumoconioses (what it is, requirements, mediator)
Interstitial fibrosis due to occupational exposure. Requires chronic exposure to particles small enough to reach bottom of the lung, where macs engulf and induce fibrosis
Coal Workers’ Pneumoconiosis (cause, disease association)
Carbon dust leads to diffuse fibrosis (black lung)
Associated with RA (Caplan’s Syndrome)
Anthracosis
Mild exposure to carbon like pollution leads to collections of carbon-laden macrophages; not clinically significant
Silicosis (location, important complication/disease)
In upper lobes of the lung
Inhibits phagolysosome formation in macs so increased risk of TB (only pnemoconiosis that does this)
Berylliosis (population at risk, what it causes, similar disease presentation)
Beryllium miners (duh) and AEROSPACE industry workers Causes noncaseating granulomas in lungs/hilar LNs/systemic organs like sarcoidosis so don't jump to sarcoidosis
Asbestosis (important point about mesothelioma and lung cancer, histological finding)
Increases risk for both, but lung carcinoma more common in exposed individuals
See “asbestos bodies” - long, golden brown fibers with associated iron
Sarcoidosis (what it is, demographic)
Systemic disease of noncaseating granulomas in multiple organs (commonly lungs and hilar LNs) usually in African American females. Likely due to CD4+ helper T cells and unknown antigen
Sarcoidosis Histological Findings and other common sites/disease misassociations
“Asteroid bodies” - stellate inclusions within giant cells
Also uveitis, skin nodules/erythema nodosum, and salivery/lacrimal gland - mimics Sjogren’s syndrome
Sarcoidosis Clinical Presentation (3) and Treatment
Presents most commonly with COUGH (or dyspnea), elevated serum ACE, and Hypercalcemia (1-alpha hydroxylase activity of epithelioid histiocytes converts Vitamin D to active form in all noncase granulomatous diseases)
Treat with steroids but often resolves spontaneously
Hypersensitivity Pneumonitis (what it is, who’s at risk, presentation, continued risk)
Granulomatous reaction to inhaled organic antigens (pigeon breeder’s lung) that presents with fever/cough/dyspnea hours after exposure and resolves with removal, but can cause interstitial fibrosis with chronic exposure
