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28 Cards in this Set
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Idiopathic FibrosingInterstitial Pneumonia (Idiopathic Pulmonary Fibrosis) etiology? age of onset? |
•Specificform of chronic fibrosinginterstitial pneumonia. etiology = unknown. onset = 50 to 70 years old |
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risk factors with IPF? |
smoking. pollution, chronic microaspiration. genetic predisposition. |
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pathphysiology of IPF? |
Leads to interstitial thickening anddistortion. –Dependingon extent of fibrotic process, lung architecture can become almost completelydestroyed.– |
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other names for IPF? |
Other names •Interstitial pneumonia •Chronic interstitial pneumonia •Cryptogenic fibrosing alveolitis |
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clinical findings with IPF? |
progressive disabling dyspnea. NON productive cough. VELCRO crackles in 80% of patients. characteristically end-inspiratory. most prevalent at the lung bases. clubbing |
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imaging with IPF is? CXR? CT? |
CXR irregular with peripheral basilar reticular criss cross opacities that are usually bilateral and often asymetric. diffuse patchy fibrosis with pleural based honey combing on CT. Ground glass on CXR. Honey combing on CXR. and ground glass on CT/ |
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PFT's with IPF |
REduced TLC and FVC. Normal or increased FEV1/FVC ratio decreased Pa O2 and DL CO. |
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how to dx IPF? |
exclude through serologic testing and definitive dx through surgical lung bx. |
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Tx for IPF |
supportive. tx GERD and SABA for cough. long term abx. vaccinate. tx PHTN. Tx cardiac dysfunction. lung transplant. |
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sarcoidosis is what? |
Multisystem granulomatous disorder with an unknown etiology. noncaseating granulomas in involved organs. 90% have lung granulomas. |
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who gets sarcoidosis? |
north american blacks or AA. females > males and northern european whites. 10 to 40 years old. |
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pathophysiology of sarcoidosis |
intial lesions are noncaseating within the pulmonary system from T cells. other tissues involved are skin, eyes and lymph nodes. |
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what are these S&S for? insidious onset, fever, dyspnea, malaise. dry cough and or chest pain. 50% are asymptomatic and seek medical attention after abnormal CXR findings. typically bilateral hilar and right paratracheal lymphadenopathy. erythema nodosum. parotid gland enlargement. |
sarcoidosis signs and symptoms. |
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labs with sarcoidosis |
elevated ESR/ ACE enzyme are elevated in 75% high alk phos if granulomas in liver. hypercalcemia (5%) |
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radiographic staging of sarcoidosis. |
normal = stage 0 bilateral adenopathy +/- paratracheal adenopathy = stage 1 reticular opacities and shrinking hilar nodes = stage 2. reticular opacities and volume loss = stage 4. poor prognosis. |
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CT scan of sarcoidosis |
beaded or irregular thickening of bronchovascular bundles. ;lots of others that I will forget for sure. |
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3 elements of dx for sarcoidosis? |
clinical and radiographic manifestations. exclusion of other ds. histopathological detection of noncaseating granulomas. |
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tx of sarcoidosis? |
oral corticosteroids 0.3 to 0.6 mg/kg/day. TNF therapy. (anti-tumor necrosis factor. |
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how will you be able to tell if the patient is improving with sarcoidosis tx? |
serum ACE levles fall. |
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prognosis of sarcoidosis numbers |
20% suffer irreversible lung impairment. myocardial sarcoidosis in 5%. death from pulmonary insufficiency in 5%. |
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Pneumoconiosis is what? who gets it? tx? |
•Fibrotic lung diseases caused by theinhalation of coal dust and various other inert, inorganic, or silicate dusts •Clinically important pneumoconiosesinclude–Coalworkers’ pneumoconiosis–Silicosis–Asbestosis •Treatmentfor each is supportive (don’t get fooled by that) |
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complicatinos with Pneumoconiosis in coal workers? |
progressive massive fibrosis. |
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complications with Pneumoconiosis in silicosis? |
the upper lung-coalescing of nodules–Dyspnea–Obstructiveand restrictive pulmonary dysfunction |
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symptoms with silicosis? |
usually none. complication |
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CXR for silicosis? |
–Formation of small rounded opacities (silicotic nodules) throughout the lung –Calcification of the periphery of hilar lymph nodes (“eggshell” calcification) |
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Asbestosis exposure time? symptoms and signs. |
10 to 20 years. progressive dyspnea. inspiratory crackles. +/- clubbing and cyanosis. |
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CXR and CT of Asbestosis ? |
CXR PHOL –Pleural calcifications (50%) Honeycomb changes in advanced cases –Opacities of various shapes and sizes – –Linearstreaking at the lung bases CT presence of coexisting pleural plaques and parenchymal fibrosis. |
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PFT's with fibrosis ling ds |
•Normalor high FFEV1/FVC ratio and decreased difussingcapacity is associated with fibrosing lungds |