[PAGID] CGD + Granulomatous lung disease

[Richard Wasserman]

Joe,
Consider granuibacter. It is somewhat less likely without the extrapulmonary
invasion but it isn't easy to culture.

Greenberg DE, Shoffner AR, Zelazny AM, Fenster ME, Zarember KA, Stock F,
Ding L, Marshall-Batty KR, Wasserman RL, Welch DF, Kanakabandi K, Sturdevant
DE, Virtaneva K, Porcella SF, Murray PR, Malech HL, Holland SM.
Emerg Infect Dis. 2010 Sep;16(9):1341-8.

Richard

On Fri, Sep 24, 2010 at 9:51 AM, Church, Joseph <JChurch at chla.usc.edu>wrote:


>  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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-- 
Richard L. Wasserman, MD, PhD
DallasAllergyImmunology
7777 Forest Lane, Suite B-332
Dallas, Texas 75230
Office (972) 566-7788
Fax (972) 566-8837
Cell (214) 697-7211
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