[PAGID] CGD + Granulomatous lung disease

[Kobrynski, Lisa]

Have you been able to do PCR to look for atypical mycobacteria, MAI ?
Has he been treated with any of the macrolides previously?
Did he not tolerate IFNgamma?  If you think that MAI is a possibility, the IFNgamma could be helpful as well.
Lisa


Lisa Kobrynski, MD, MPH
Associate Professor of Pediatrics
Section, Allergy/Immunology

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From: pagid-bounces at list.clinimmsoc.org [pagid-bounces at list.clinimmsoc.org] On Behalf Of Church, Joseph [JChurch at chla.usc.edu]
Sent: Friday, September 24, 2010 10:51 AM
To: pagid at list.clinimmsoc.org
Subject: Re: [PAGID] CGD + Granulomatous lung disease

Thanks, Kate.  He received 2+ months of iv vori and is still on high dose po itra.  The problem is that on “a little” steroid (0.5mg/kg/day) his lung functions are slipping.  JC

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From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Kate Sullivan
Sent: Friday, September 24, 2010 7:25 AM
To: pagid at list.clinimmsoc.org
Subject: Re: [PAGID] CGD + Granulomatous lung disease

This looks more homogeneous than a typical fungla infection but I would still vote to treat for fungus plus a little steroid.

Kate
On Sep 23, 2010, at 9:00 PM, Church, Joseph wrote:


Colleagues:

I am following a 10yo boy with X-CGD.  He developed progressive pulmonary infiltrates while on TMP-Sulfa and Itraconazole prophylaxis.  He was not and is not on interferon-gamma.  Despite 2+ months of parenteral antibiotics and anti-fungals these did not improve.  An open lung biopsy revealed extensive granulomatous infiltration, but all studies for bacterial, mycobacterial and fungal organisms were negative.  Please see the attachment for the CT and biopsy findings.

Given the “sarcoidosis-like” lung picture (De Ravin et al. Pediatrics 2006;117:e590) and lack of response to standard antimicrobial therapy, he was treated with a brief course of steroids and his lung functions improved.  In an attempt to avoid long term steroids he was given methotrexate for 6 months, but had no clinical or spirometric response.  Subsequently, he improved on daily steroids + methotrexate, but when I tried to taper him to 0.5mg/kg/day, his lung functions again dropped.

We are looking for a HSC donor, but because of his very mixed ethnicity we haven’t come close in two years of searching.

Any suggestions?

Joe Church
Childrens Hospital Los Angeles

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Kathleen Sullivan MD PhD
Professor of Pediatrics
Chief, Division of Allergy Immunology
The Children's Hospital of Philadelphia
(p) 215-590-1697
(f) 267-426-0363





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