[PAGID] CGD + Granulomatous lung disease

[Berger, Melvin]

You may have to bite a bullet and try infliximab or another TNF antagonist, perhaps after first trying IFN-g
 
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 Church, Joseph
Sent: Fri 9/24/2010 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

 

________________________________

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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<Lungs.pdf>

 

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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