virgola82
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A 62 yo man former smoker presents with a 2 year history of progressive dry cough and dyspnea. No occupational or enviromental exposures are noted. Findings on physical exam include bilateral basilar Velcro rales and clubbing. CXR reveals prominent interstitial infiltrates in the middle and lower lung fields. The ANA titer is minimally elevated at 1:40. Serum protein electrophoresis demonstrates a polyclonal increase. Rheumatoid factor is also minimally elevated at 1:40. HRCT demonstrates subpleural honeycombing with thickened alveolar septa in the lower lobes with bilateral lymphnodes of 1.5 cm but only minimal ground-glass opacities. On PFTs VC is 62% of predicted, FEV1 is 69% of predicted and FEV1/FVC ratio is 78% with DLCO 52% of predicted. Which one of the following treatments is likely to result in clinical improvements?
A azathioprine
B Steroids
C Cyclophosphamide
D Colchicine
E No treatment has been proven to be effective
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| changchang
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B steroid
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| sprint123
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Steroids are used for "Farmer's Lung"
HRCT-->Honey combing + ground glass + restrictive pattern of PFT---->Farmer's lung!
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| virgola82
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This is not farmer's lung... It's a restrictive disease associated with neither rheumathologic conditions nor exposures. So it is Interstitial Pulmonary Fibrosis (IFP)/Usual Interstitial Pneumonia (UIP) for which there is no therapy proved to be effective. Steroids are useful in case of Non-Specified Interstitial Pneumonia (NSIP)/IFP which is IFP associated with reumathologic conditions or exposures. Unlike IPF/UIP, NSIP/IPF is associated with ground glass opacities at HRCT as opposed to IPF/UIP in which the major feature is subpleural honeycombing with fibrosis and traction bronchiectasis. Overall prognosis is poor for IPF/UIP (5yr survival 20% with or without steroids) while is better for NSIP/IPF (5yr survival 65-80% with steroid therapy)
So The right answer was E
maybe it was a little too hard: it's from a collection for ABIM test
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| sprint123
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Great question virgola82!
I appreciate your postings!
So IPF unrelated to rheumatolgy or inflammation--->Steroids ineffective
IPF with rheumatologic symptoms--->Steroids..
Is that right? BTW : How to differentiate between Farmer's lung and IPF?
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| virgola82
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This topic is not my beast one anyway according to what I found I'd better conclude
IPF with rheumatologic symptoms--->Steroids
IPF without neither rheumatologic symptoms nor exposure AND HRCT revealing subpleural honeycombing, fibrosis and traction bronchiectasis---->Steroids useless
The differential diagnosis between NSIP/UIP and UIP/IPF is actually resolved with the HRCT response
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| ram3
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steroids big time
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| doyoudig
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UW seems to differ from this explanation
It states Steroids are the best Treatment Option to which 35% of Pt repsond effecively in the 1st 6 month but steroids to not sustain this repsonse, therafter
what do u think?
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| cirus
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I chose ..E No treatment has been proven to be effective .. and actually i thought it is asbestosis as he is an old guy & so by the time he was young & was actively working,asbestos was still bieng used even in agricultural field (insulation & stuff) as his bilateral LNs are quite suggestive..
From what i know nothing works with interstetial lung disease, its irreversible but will stay stationary if exposure is stopped, some mild cases may reverse but thats not comman..
Concerning the rheumatological factor, thats totally new for me so ill have to look it up..
Nice quest though, thanks virgola82
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