[PAGID] CGD + Granulomatous lung disease

[Church, Joseph]

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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