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24 Cards in this Set
- Front
- Back
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what is the pathogenesis for interstitial lung disease?
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- injury --> activates endothelial cells --> activates T cells --> T cell alveolitis --> cytokines (more T cells, monocytes) --> granulomas (chronic inflammatory reactions, lymphocytes, monocytes, epithelial cells, multinucleated giant cells) --> activate B cells (can get auto-antibodies) --> injury capillaries or epithelium --> diffuse alveolar hemorrhage syndrome (bleeding) --> fibrosis
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what is the pathogenesis in idiopathic pulmonary fibrosis?
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- injury to epithelium --> TGF-beta1 --> fibroblasts & myofibroblasts (inhibit caveolin which would normally inhibit deposition of ECM)
- leads to pulmonary fibrosis (we don't even know if there is an inflammatory component) |
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why can there sometimes be an increased FEV1/FVC ratio in restrictive diseases?
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- there is often improved tethering of the airways during expiration
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what 3 factors does diffusion depend on?
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- thickeness, SA, pressure gradient
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what happens to V/Q during the progression of ILD?
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- hypoxemia at rest begins from low V/Q
- later in stages get high V/Q from increased dead space due to fibrosis or hypoxic vasoconstriction or cystic air spaces that are not well perfused |
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how does a patient with ILD breathe?
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- rapidly b/c need to expel the CO2, but shallowly b/c of restriction (aka decreased TV)
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how can ILD cause pulmonary vascular disease?
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- chronic hypoxemia & direct pulmonary vascular involvement by interstitial process can lead to pulm HTN & cor pulmonale
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how can ILD lead to pneumothorax?
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- honeycombing - cystic structures may rupture into pleura causing pneumothorax
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how can ILD lead to PE?
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- some of the diseases associated with ILD have thrombophilia (ex. lupus), & as pts get sicker & sicker & immobile & develop edema (b/c RHF) increase risk of forming DVT
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when can wheezing occur in ILD?
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- unusual symptom, but can occur w/ chronic eosinophilic pneumonia & vasculitis
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when can hemoptysis occur in ILD?
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- diffuse alveolar hemorrhage syndrome, vasculitis
- unusual symptom may also suggest lung cancer |
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what does chest pain w/ ILD suggest?
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- unusual symptom, but might suggest pneumothorax or underlying process that might affect pleural space (RA, SLE, asbestosis)
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when do you here crackles/rales "velcro" on exam?
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- ILD, end-inspiration squeaks
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what are diffuse reticular infiltrates? What do they suggest?
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- vessels in normal patient don't reach the pleura but in ILD you see these blood vessels
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what is honeycoming?
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- cystic spaces
- it means 100% fibrosis |
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what is the difference b/w reticular opacities vs nodular opacities?
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- reticular often indicate fibrosis vs nodular often mean inflammatory (granulomas)
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what do ground-glass opacities usually indicate?
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- usually indicate inflammation (penumonitis/alveolitis)
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what is usual interstitial pneumonia (UIP)? what is the patholophysio? what do you see on X ray? how do you treat it?
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- characteristic finding of IPF if other diseases ruled out (asbestosis, hypersensitivity pneumonitis, etc.)
- most common lung disease in older people (>50), M>F - injury to alveolar epithelium, abnormal fibroblast response (might not even have inflammation) - reticular infiltrates, honeycombing - this is NOT treatable |
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what is desquamative interstitial pneumonia (DIP)? what is the pathophysio? what do you see in X ray? how do you treat it?
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- occurs in smokers (4-5th decade)
- alveolar macrophages (smokers macrophages) w/ abnormal alveolar septa w/ mild inflammation (plasma cells, eosinophils) - ground glass opacities - very treatable! stop smoking & give anti-inflammatories |
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what is cryptogenic organizing pneumonia? what is the pathophysio? what do you see in X ray? how do you treat it?
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- proliferative bronchiolitis & organizing pneumonia, can be complication of another disease or be idiopathic
- granulation tissue (collagen & fibroblasts) & inflammation (plasma cells & lymphocytes) - alveolar infiltrates on x ray - lung can often self-regulate & clear this |
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what is sarcoidosis? what is the pathophysio? what do you see in X ray? how do you treat it?
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- non-caseating granulomatous disease, idiopathic, multisystem, lung involvement in 90%
- starts w/ CD4 alveolitis --> activate macrophages --> granulomas - hilar/mediastinal adenopathy, reticulonodular infiltrates - increased ACE, treat w/ glucocorticoids |
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which disease is serum ACE increased?
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- sarcoidosis
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what is pulmonary langerhans cell histiocytosis (eosinophilic granuloma)? where is it typically found?
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- young adults, smokers, interstitial infiltrates
- lung volumes preserves, stellate nodules & birbeck granules - upper lobes |
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what is chronic eosinophilic pneumonia? how can you diagnose? how do you treat?
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- accumulation of eosinophils in lung
- associated with asthma (>50%), patients wheeze so think asthma + chronic eosinophilic pneumonia - non-smokers - diagnose via bronchoalveolar lavage (BAL) > 24% eosinophils - treat with steroids |
