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

Wed Aug 17 03:00:33 EDT 2016

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
Tel.: 0761 270-77731  Fax: -77744
Breisacherstraße 115, 79106 Freiburg
bodo.grimbacher at uniklinik-freiburg.de
www.uniklinik-freiburg.de/cci

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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