lungs 7
Terms
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- causes of restrive lung disease
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- Interstitial lung disease (most common)
- Abnormalities of the chest wall
- Weakness of the respiratory muscles - Abnormalities of the chest wall and PFTs
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- Fibrothorax and Morbid obesity
– it will decrease FVC - Interstitial lung disease and PFTs
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- Sarcoidosis and Idiopathic pulmonary fibrosis (IPF)
– it will decrease TLC, FVC and RV - Weakness of the respiratory muscles and PFTs
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- Myasthenia Gravis and ALS
– it will decrease TLC and increase RV - interstitial lung disease mimicks
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CHF
Pulmonary infection (esp PCP)
Lymphangitic carcinomatosis - interstitial lung disease path
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- ↓ lung compliance (so ↓ FVC and FEV1, normal ratio)
- reduced lung volumes (TLC, FRC, RV)
- Diffusion impairment
- Destruction of small pulmonary vessels and alveolar-capillary interface by inflamm and fibrosis, so ↓ SA
– pulmonary Hypertension - interstitial lung disease Sx
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- Dyspnea and cough
- Crackles (rales), Clubbing, Cor pulmonale (so JVD, Loud P2, TR, Lower extremity edema) - interstitial lung disease radiology
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- CXR shows ↓ lung volumes
– also interstitial pattern, which is reticular (increased linear markings), and reticulonodular (linear and small nodules) - interstitial lung disease HRCT (high resolution CT)
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– Detects sub-radiographic disease
- distinguishes inflam from fibrosis and suggests a dx
– ground glass (Means you can see BV’s and airways running through the infiltrates) suggests inflamm
– consolidation (you cant see BVs) - interstitial lung disease bronchoscopy
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- to exclude infections
- BAL cellular differential can support a dx
– a transbronchial biopsy is for granulomatous disease or lymphangitic spread of cancer - interstitial lung disease VATS biopsy
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- gold standard
– larger sample size and is required for differentiation of idiopathic interstitial pneumonias
– biopsy should be strongly considered in pts w/ DPLD when dx remains uncertain after clinical and radiological assessment - Idiopathic pulmonary fibrosis (IPF) definition
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- a specific form of chronic fibrosing interstitial pneumonia limited to the lung and associated with the histologic appearance of UIP
- almost uniformly fatal (median survival 2-3 yrs) - Idiopathic pulmonary fibrosis (IPF) path
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- involves a defect in injury repair
– some epi cells die and release GF’s and other stuff to regenerate
– in some people they stimulated fibroblasts to convert to myofibroblasts which lay down collagen in the lung - Idiopathic pulmonary fibrosis (IPF) clinical
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- restrictive PFTs
- decreased DLCO
- Age >50 w/ a gradual onset of symptoms
– progressive dyspnea on exertion
– nonproductive paroxysmal cough
– Bibasilar crackles on auscultation
– Digital clubbing - Idiopathic pulmonary fibrosis (IPF) rad and hist
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- show typical UIP pattern
– Fibroblastic foci, Temporal heterogeneity, Honeycombing appearance, Minimal inflammation
– there will be bilateral basilar opacities in CRX
– HRCT will show a reticular pattern w/ sub-pleural distribution and septal thickening - Idiopathic pulmonary fibrosis (IPF) diagnosis
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- Transbronchial biopsy (bronchoscope down nose, outpatient) not useful, but can exclude others
– find UIP in surgical lung biopsy - Idiopathic pulmonary fibrosis (IPF) treatment
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- O2 therapy to prevent or delay cor pulmonae
– IFN-g can also help by blocking TGF-B, which is part of the epithelial injury pathway - Sarcoidosis path
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- lots of evidence that it is Ag driven
- Histiocytes, Dendritic cells (both APCs), and T-lymphocytes are big
– histiocytes are differentiated macros, that aggregate to look like an epithelium - Sarcoidosis and TH1
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- TB and sarcoidosis (and virus and fungus) are mediated by TH1 cytokines (TNF-a, IL-12, IL-18, IL-2, IFN-g) -> progression to fibrosis
– asthma and parasites are by TH2
– the greatest linkage in genomic studies is to HLA region (the MHC genes) - Sarcoidosis Putative antigens
- Mycobacteria and Propionibacteria (gram + thing in hair follicles)
- Sarcoidosis and Lofgren’s syndrome
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- fever, BHL, and polyarthritis
– erythema nodosum (raised red bumps on the back of the legs, esp in whites)
- lupus pernio (represents chronic - indurated plaques and discoloration of the nose, esp in blacks)
– Adjacent joints usually swollen and painful - Sarcoidosis Stages
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- 1 presents has BLH and normal lung parenchyma (2/3 spontaneous resolution)
– 2 has parenchymal and BHL
- 3 has parenchymal and no BHL
- 4 has fibrosis with hilar retraction, honeycomb changes, and large bullae and cysts – lungs are usually clear on exam - Sarcoidosis HRCT
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- nodules, ground glass opacification, intense opacification
- reticular patttern or honey combing - Sarcoidosis Serological clues
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- Peripheral lymphopenia
- ↑ serum ACE
- Hypercalcemia - Sarcoidosis Dx
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- need tissue
- must see granulomas and not other cause of them (TB, fungus, cancer) - Sarcoidosis Treatment
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– Hydroxychloroquine (anti-malerial) alters the pH of the MHC containing peptides to reduce the amount of antigens presented
- Corticosteroids most effective and mainstay
- Immuonsuppressive agents
- Anti-TNF stops TNF from forming granulomas (bad in TB - will cause dissemination)