[CIS PIDD] Primary immune deficiency and alpha-1 antitrypsindeficiency?

[Berger, Melvin]

I would be very concerned that the PIDD may play a role similar to one of the effects attributed to cigarette smoking in A1AT deficiency disease: providing (even a low-grade) pro-inflamatory stimulus which would attract increased numbers of neutrophils, which would then create an elastase burden in excess of the local inhibitory capacity. Therefore I suggest that he be monitored very closely for signs of systemic and pulmonary inflammation. If he seems to be having subclinical airways or alveolar infection, maximal effort should be made to control this- like a CF regimen of antibiotics and pulmonary toilet, and relatively high vs usual doses of IgG replacement. If he has uninhibited active elastase in induced sputum or BAL, he should definitely be on A1AT augmentation. 
 
Melvin Berger, M.D., Ph.D.
Adjunct Professor of Pediatrics and Pathology
Case Western Reserve University
Cleveland, OH 44106

________________________________

From: pagid-bounces at list.clinimmsoc.org on behalf of Yesim Yilmaz Demirdag
Sent: Thu 9/6/2012 3:47 PM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS PIDD] Primary immune deficiency and alpha-1 antitrypsindeficiency?







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