Exam 1 Part 2 Flashcards
what is bronchiectasis?
dilation of bronchus; large airway disease
what processes/diseases are associated with bronchiectasis?
- congenital (cystic fibrosis)
- TB, other bac & viruses
- RA, SLE, IBD
- bronchial obstruction due to tumor, foreign bodies or mucus plugs
how does bronchiectasis present clincally?
- cough
- fever
- expectoration of copious amounts of foul smelling, purulent sputum
what is cystic fibrosis?
- disorder of ion transport epithelial cells (CTFR gene disorder) which leads to excessive mucus in the lung
- on skin Na stays outside epi cells, in airways it stays in epi cells
what 6 groups are mutations of CF in?
- defective syn
- abnormal folding
- defective regulation
- defective conductance
- reduced abundance
- altered regulation of separate ion channels
what is the main pathology associated with CF?
85-90% pancreatic insufficiency
what can the thick mucus and repeated infxns in CF lead to?
chronic bronchitis and bronchiectasis
what are the two diseases which lead to morbidity and mortality in CF pts?
cor pulmonale and COPD
what is fibrosing?
- fibrosing of lungs following inflammation but absence of infectious organisms
- perhaps autoimmune
how does a restrictive lung ‘feel’?
-if squeezed it it wouldn’t feel as spongy as a normal lung
what will an obstructive lung disease look like on an XR?
diffuse increased density and increased opacity
what is idiopathic pulmonary fibrosis?
chronic idiopathic inflammation of alveolar walls w/progressive fibrosis
pathophys of IPF?
- alveolar MOs malfxn
- lung epi cells produce abnormal amount of PDGF
- alveolar glutathione reduced
how does IPF present clinically? on XR what is distinctive? tx?
- progressive DOE
- non-productive cough
- crackles
- cor pulmonale, clubbing of nails
- XR: ‘honey-comb’ like appearance
- tx: high dose steroids, cytotoxic drugs, supplemental O2, most die 4-6 yrs after dx
what is COP?
cryptogenic organizing pneumonia
- idiopathic
- bronhiolitis obliterans
- often occurs in transplanted lung
what are the 3 collagen vascular dz?
- scleroderma
- RA
- SLE
what are the categories of pneumoconioses? obstructive or restrictive?
- occupational
- coal miners lung
- dust or chemicals or organic material: coal, silica, asbestos, Be, FeO, hay, flax, bagasse, insecticides, etc
- restrictive lung disease leading to fibrosis
the development of pneumoconiosis depends on what 4 factors?
- amount of dust retained in airways and lung
- size and shape of particulate matter
- solubility of particulate matter
- additional effects of other irritants (tobacco)
what range of size of particles are the most dangerous?
5 micrometers b/c 5 can be coughed up
what is silicosis due to?
- inhalation of silicon dioxide crystals from sand blasters, foundry workers, stone cutters etc
- IT IS THE MOST COMMON CHRONIC OCCUPATIONAL DX IN THE WORLD
where are fibrotic nodules from silicosis located in the lungs?
-initially confined to upper lobes
what is the pathognomonic sign?
eggshell calcifications of hilar lymph nodes
how fast does silicosis progress?
- generally very slowly, yrs to decades
- accelerated silicosis: 5-15 yrs
- acute silicosis: weeks
how do pts with silicosis present?
- sometimes asx or only complain of cough
- SOB in late stages
- may continue to worsen even w/o exposure
what is Caplan’s syndrome?
pneumoniosis coexisting with RA usually due to asbestosis, silicosis or coal worker’s pneumoconioses
-lg distinct pulmonary nodules often develop w/center that cracks open
where would one be exposed to asbestos?
- insulation fabrication
- mining
- break lining fabrication
- asbestos removal workers
what do asbestos crystals do to the lungs?
transverse the lung, enter the interstitium and impale the pleural lining
what are ferruginous bodies?
asbestos fibers coated by proteins w/in lungs
what kind of parenchymal disease is asbestosis?
-diffuse
signs usually appear where first?
-pleural and sub-pleural fibrosis usually precede any signs of pulmonary fibrosis
what are pleural plaques?
- large dense plaques that form on both sides of pleural surface
- appear first at base then surround lung
- seen on CXR
what greater risks in relation to cancer do asbestos workers have? smokers?
- 5x greater risk of bronchogenic carcinoma in asbestos workers
- mesothelioma, rare tumor of pleura almost always associated with asbestos exposure
- smoking + asbestos exposure raises risk of developing bronchogenic cancer from 5-55x
- smoking + asbestos doesn’t seem to raise risk for developing mesothelioma
where does one get exposed to berylliosis?
-aerospace, nuclear & defense industries
is coal worker’s pneumoconiosis a serious respiratory problem? what is the more mild form called and where is it found?
- NO not unless additional pathology is present (TB, emphysema)
- anthracosis: milder form of deposition often found in urban pops, tobacco users
what is black lung disease? becoming more or less common?
- CWP progressing to more severe diffuse pulmonary fibrosis
- becoming more common b/c of ppls lack of adherence to safety regulations
what is siderosis?
-consequence of inhalation or iron oxide dust
what are the 3 types of granulomatous dz?
- sarcoidosis
- non-caseating granulomas (idiopathic)
- hypersensitivity
what is sarcoidosis?
- multi-system granulomatous disorder of unknown etiology
- lungs common to be affected, but can occur elsewhere
- non-caseating
who gets sarcoidosis?
20-40 yo african american women
how does a pt w/sarcoidosis present clinically? what is the most common skin sx?
- often asx
- 1st sign may be dyspnea
- often found incidentally on XCR
- erythema nodosum is most common skin lesion
what happens if sarcoidosis affects the liver or heart?
- hepatic granulomas present in 70% of pts but usually no sxs
- heart rarely affected but if affected about 50% will die of cardiac complications
what labs might you find with a pt with sarcoidosis? what is a diagnostic thing you might find in a biopsy?
- elevated vit D; alveolar MOs can synthesize calcitriol
- hypercalcemia & elevated angiotensin converting enzyme
- asteroid bodies!! truly diagnostic but hard to find
what does organic pneumoconiosis develop in response to?
- allergic rxn to inhaled organic dusts
- type I (IgE), III (IgG) or IV (delayed)
- abnormal sensitivity or heightened rxn to an antigen
- usually more acute response than other pneumoconioses
what is farm worker’s lung in response to? what is bagassosis?
what is byssinosis? what are the sxs?
-farm worker’s lung: actinomycetes spores (damp harvested hay)
-bagassosis: hypersensitivity rxn to mold found in sugar cane
-byssinosis: allergic response to airborne fibers of cotton, linen, flax & hemp
SXS: similar to bronchial asthma with coughing and wheezing, prolonged exposure= chronic bronchitis and emphysema
what is DIP?
- desquamative interstitial pneumonia
- cigarette related
- 100% survival
- M»F
what is PAP?
- pulmonary alveolar proteinosis
- very rare, usu acquired
- exudate in alveoli
- like PE but exudate
what are the three types of ‘vascular’ lung diseases?
- pulmonary embolism (usu w/o infarct)
- pulmonary HTN, leading to cor pulmonale
- hemorrhagic syndrome (good pasture, hemosiderosis, wegener granulomatosis)
when do PEs develop? where do they come from? what is the triad it correlates to? do they infarct?
- develop secondarily to debilitated states
- usually from deep leg veins
- virchow’s triad: 1. flow problems 2. endothelial disruption 3. hypercoagulability
- usually don’t infarct, when do it is hemorrhagic
is a PE a problem with diffusion or perfusion?
PERFUSION, ventilated but not perfused
what are some causes of a thromboembolism? what lungs are move involved?
- DVT from legs or pelvis
- long standing IV line catheters
- pro-thrombogenic blood diseases
- lung cancer
- lower lungs 4x more involved
what are the two outcomes of a PE?
-in non-fatal PE’s embolus lyses within several days-weeks
OR
-cardiogenic shock: emboli occludes >50% of arterial supply, death in 1-2 hrs possibly; saddle embolus= death in minutes
what are the factors that will lead to PE mortality? with pre-existing condition? without tx? with anticoagulant tx?
- emboli size and location and pt’s pre-existing cardiopulmonary status
- pre-existing cardiopulmonary status= 25% mortality
- w/o tx= reoccurs 50% of the time, with 50% fatality
what are two other types of emboli and what type of circulation do they block? Sxs?
- fat emboli
- amniotic emboli
- block microciruclation–> may initiate Adult Respiratory Distress Syndrome (ARDS)
- sxs: sudden onset of tachypnea, dyspnea, tachycardia
what is a pulmonary infarction? what % of PE’s progress to a PI?
- complete occlusion of blood supply
- PI
what is the gross morphology of a PI?
- hemorrhagic consolidation
- necrosis of lung tissue
- classic wedge or pie shape
- tissue fibrosis
- cough
- hemoptysis
- pleuritic chest pain
- fever
- friction rub
- severe hypotension will occur in the case of cardiogenic shock
are CXRs helpful in diagnosing a PE?
NO, they are the least helpful thing to do if assuming a PE
what can cause pulmonary HTN?
- COPD or CIPD
- CHD
- recurrent PE’s
- autoimmune
in primary PH it is arterial thickening and obliteration of what sized arteries? more or less common?
- medium & small pulmonary arteries
- less common
in secondary PH what walls are thickening? usually due to what?
- wall thickening may be through entire arterial system
- usu due to other cardiac or pulmonary conditions
- recurrent thromboemboli can cause PH
in primary and secondary PH what is damaged, what thickens, what is there dysfunction of and what is diminished?
- damaged endothelial cells
- thickening of smooth muscle cells
- dysfunction of endothelial cells= less NO
- diminished oxygenation
PH leads to what in the heart?
persistent elevated P leads to right ventricular enlargement
what is cor pulmonale?
right ventricular failure secondary to lung pathology
what are risks of PH?
- alveolar hypoxemia
- ARDS
- sleep apnea
- pulmonary edema at high altitudes
- emphysema
is tx for PH usu very successful?
NO lung transplant usu necessary, death in 1-5 yrs usu
what are the hemorrhagic syndromes?
- goodpasture syndrome
- idiopathic pulmonary hemosiderosis
- wegener granulomatosis
what is goodpasture syndrome?
tx?
- multi-system disease involving lungs & kidneys
- Abs to alveolar & glomerular basement membrane–> hemoptysis & hematuria
- tx= steroids, plasma phoresis but may be resistant & lead to death in days to weeks
what is wegener granulomatosis?
- vasculitis that affects lungs, kidneys & other organs
- end-organ damage= serious disease that can require immunosuppression
what does IPH have that CHF does not have?
- IPH: hemosiderin in alveoli
- CHF: no hemosiderin
