[CIS PIDD] [cis-pidd] AR-CGD and Hidradenitis

Wed Aug 17 03:20:16 EDT 2016

Hi
Yes-
Gulbu Uzel published a case series of complications associated with tnf blockade in cgd patients. 
http://www.ncbi.nlm.nih.gov/m/pubmed/21058909/
Jen
Sent from my iPhone

> On Aug 17, 2016, at 3:00 AM, "CIS-PIDD" <cis-pidd at lists.clinimmsoc.org> wrote:
> 
> This is interesting, Jen.
> Is there data out to support this?
> (I am not a CGD person, but I am interested in the indications of
> anti-TNFa)...
> Yours, Bodo
> 
> ****************************************
> Univ.-Prof. Dr. med. B. Grimbacher
> 
> Scientific-Director
> CCI-Center for Chronic Immunodeficiency
> UNIVERSITÄTSKLINIKUM FREIBURG
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> 
> 
> Am 16/08/16 22:39 schrieb "CIS-PIDD" unter <cis-pidd at lists.clinimmsoc.org>:
> 
>> 
>> 
>> I would avoid using anti anti-TNF agents in CGD patients
>> 
>> Jen
>> 
>> ________________________________________
>> From: CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
>> Sent: Tuesday, August 16, 2016 2:28 PM
>> To: CIS-PIDD
>> Subject: RE: [cis-pidd] AR-CGD and Hidradenitis
>> 
>> In the US, adalimumab (Humira) is approved for hidradenitis suppurativa...
>> 
>> Tony Infante
>> 
>> -----Original Message-----
>> From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
>> Sent: Tuesday, August 16, 2016 1:05 PM
>> To: CIS-PIDD
>> Subject: [BULK] AW: [cis-pidd] AR-CGD and Hidradenitis
>> 
>> Dear Joe and Dave,
>> 
>> I never had a case like this, but had very good results in CGD related
>> inflamamtion/infection (panniculitis, liver abscess, thymic mass) with
>> low dose prednisolone (1-2 mg/kg).
>> 
>> Best regards, Fabian
>> 
>> Fabian Hauck, MD, PhD
>> 
>> Attending physician / Head Immunodeficiency Unit and Immunological
>> Diagnostics Laboratoy Pediatrics / Pediatric Hematology and Oncology /
>> Immunology (DGfI)
>> 
>> Dr. von Hauner Children's Hospital
>> Klinikum der Universität München
>> Lindwurmstr. 4, 80337 München
>> Germany
>> 
>> Tel.: +49 89 4400-53931
>> Fax: +49 89 4400-53964
>> E-Mail: fabian.hauck at med.uni-muenchen.de
>> ________________________________________
>> Von: CIS-PIDD [cis-pidd at lists.clinimmsoc.org]
>> Gesendet: Dienstag, 16. August 2016 19:32
>> An: CIS-PIDD
>> Betreff: Re: [cis-pidd] AR-CGD and Hidradenitis
>> 
>> Hi Joe,
>> 
>> I am very interested in your case and the responses from colleagues.  I
>> have basically a duplicate case.  I am not sure what to do with her.
>> 
>> She is a 21 year old who came to use with Hidradenitis Supurativa s/p
>> resection (non-healing wound) in her right axilla.  She was set up to
>> donate marrow for her little brother who had AR CGD.  We did a DHR on
>> her...  and her DHR was also abnormal.  Mutation analysis documented
>> homozygous NCF1 mutations.  She was placed on itraconazole, bactrim and
>> actimmune.  She is compliant.  I treated her with a prolonged course of
>> doxycycline - which helped her left axilla (the one that was not operated
>> on) as she some draining wounds on that side.  The problem is the right
>> side that had the surgery.  It is a clean wound that is approxiamtely 1.5
>> inches in length and a mm or so deep.
>> 
>> Her inflammatory markers have remained mildy elevated ESR 60-70s.  CRP
>> also elevated.  We scanned her chest looking of other things - negative.
>> I did a MR of her right axilla - no evidence of a deeper infection.  I
>> tried a course of corticosteroids for a few months which helped a tiny
>> bit, but she got pretty toxic.  I put in a PICC line and placed her on
>> ertapenem for about 1.5 months since a swab from the lesion grew serratia
>> and staph.  The wound did not improve on antibiotics and nor did her ESR
>> and CRP.
>> 
>> Our plastics team offered her a "flap" procedure assuming this is just
>> hidradenitis.  I was not that excited about that as we may run into more
>> wound healing issues.  I wanted to make sure we have exhausted other
>> options.
>> 
>> -Dave
>> 
>> 
>>>>> "CIS-PIDD" <cis-pidd at lists.clinimmsoc.org> 8/16/2016 10:19 AM >>>
>> Colleagues:
>> 
>> I am consulting on a 19yo F with autosomal recessive-CGD (NCF-1
>> mutation).  She has been remarkably well.  She presented at ~10yo with
>> cervical adenitis and severe gingivitis.  Since that time she has been
>> treated with TMP-SMX, itraconazole and interferon-gamma.  She has had no
>> other episodes of adenitis or any other infection.  1 year ago she
>> developed bilateral axillary hidradenitis suppurativa.   She has had
>> excisional surgery on the left side.  Currently, both areas are scarred
>> with minimal discharge.
>> 
>> Any suggestions?
>> 
>> Joe Church, MD
>> Children's Hospital Los Angeles
>> 
>> 
>> 
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