Sarcoidosis Flashcards
what is the etiology of sarcoidosis ?
unknown
infectious
enivornmnetal and occupational exposure
what is the pathology of sarcoidosis ?
non caseating epithelia granulomas
dominant cell is the epithelia cells , cd4 lymphocytes and mature macrophages
they often under go fibrosis - STELLATE SCAR left behind
symptoms of sarcoidosis ?
resolution within weeks to several months
asymptomatic sarcoidosis - picked by hilar adenopathy
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acute sarcoidosis with or without erythema nodosum
lofgren syndrome : acute onset of erythema nodosum (red nodules the lowe extremities typically the ankles , feet and knees)
, bilateral hilar adenopathy , fever , polyarthrtisi , uveitis
pulmonary sarcoidosis
cough ,
progressive and insidious shortened of breath
hemoptysis with sputum production - fibrocystic sarcoidosis
chest tightness and wheezing = endobronchial disease
what is the diagnosis ?
chest x ray or CT
stage 0 - normal
stage 1- hilar adenopathy , interstitial infiltrations
stage 2 - bilateral hilar adenopathy and pulmonary infiltrates - with fine linear markings and small reticulonodular in the mid and upper zones
stage 3 - interstitial infiltrates seen without evidence of hilar adenopathy
stage 5 - extensive fibrocystic disease
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pulmonary hypertension
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spirometry - restrictive
forced expiratory volume in one second (FEV1) and forced vital capacity (FVC) are reduced, however, the decline in FVC is more than that of FEV1, resulting in a higher than 80% FEV1/FVC
or obstructive impairment
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histology - by biopsy of skin
or enlarged superficial nodule
fiberoptic bronchoscopy - and tranbronchial biopsy
transbornchoscopic needle aspiration of intrathoraci lymph node
mediastinoscopy
BAL - lymphocytosis
lab - elevated ace and hypercalcemia
what ar the extra pulmonary sarcoidosis findings ?
ocular sarcoidosis
cutaneous sarcoidosis
hepatic sarcoidosis
management of sarcoidosis ?
prognosis influenced by the initial manifestations - leofgren syndrome
remission rates of 70-80 percent
stage 1 = 60-90 remission
extensive pulmonary fibrosis - rarely undergo remission
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stage 1 - excellent prognosis = no systemic therapy is required
leofgren syndrome - ranged with bed , rest and nsaids
indication for treatmnet fo sarcoidosis - threatened organ failure - such as severe occular, cardiac, hepatic or neurological disease
progressive pulmonary disease
prednisone drug therapy which becomes tapered
antimalarial drugs - chloroquine
anti inflammatory - pentoxyfyline
immunosuppressive therapies - azathioprine , methotrexate , infliximab