9.5 Restrictive Diseases Flashcards
What happens to the TLC in restrictive diseases?
Decreases
What happens to the FEV1/FVC in restrictive diseases?
Increased (greater than 80%)
What happens to the FVC in restrictive diseases?
Decreases drastically
What happens to the FEV1 in restrictive diseases?
Decreases slightly
What is the most common cause of restrictive diseases?
Interstitial Disease
What are some other causes of restrictive diseases?
Chest Wall Disease
Idiopathic Pulmonary Fibrosis
Fibrosis of lung interstitium
What is the pathogenesis of IPF?
It is due to cyclical lung injury with TGF-b from injured pneumocytes will induce the fibrosis
What are some drugs that can cause Interstitial fibrosis
Amiodarone
Bleomycin
Where does the fibrosis from IPF start at?
Sub-pleural space
Pneumoconiosis
Interstitial fibrosis due to exposure to different types of dust that are fibrogenic
What is fibrosis in pneumoconiosis caused by?
Macrophages
Coal Worker’s Pneumoconiosis
Carbon is taken in from coal miners most often that results in a black lung
Caplan Syndrome
Rheumatoid Arthritis associated with coal worker’s pneumoconiosis
Anthracosis
Collections of carbon laden macrophages in the lungs
Silicosis
Silica is taken in often in sand blasters and it impairs the formation of the phagolysosome in macrophages - the only pneumoconiosis that increases risk for TB
Where are the fibrotic nodules from silicosis found?
Upper lobes of the lung
Berylliosis
Beryllium in miners or aerospace workers that cause noncaseating granulomas in the lungs with increased risk for lung cancer - looks like sarcoidosis
Asbestosis
Asbestos in construction, plumbers or ship builders which causes fibrosis of the lung and pleura as well as carcinoma of the lung and mesothelioma
What will be seen on histology of asbestosis?
Ferruginous Bodies
Sarcoidosis
Systemic disease characterized with noncaseating granulomas in multiple organs
Who is the classical patient of sarcoidosis?
African American females
What are the characteristic findings of the granulomas in sarcoidosis?
- Asteroid Body
- Schaumann Body
What are the clinical features of sarcoidosis?
- Dyspnea
- Cough
- Elevated serum ACE
- Hypercalcemia - excessive 1-alpha hydroxylase activity which produces more Ca2+
Hypersensitivity Pneumonitis
Granulomatous reaction to inhaled organic antigens and resolves with removal from the exposure - eosinophils will be seen
What happens with chronic hypersensitivity pneumonitis?
It will lead to interstitial fibrosis
