[CIS PIDD] [cis-pidd] cystic fibrosis and CVID

[CIS-PIDD]

Dear colleagues,

yes, we experience the same phenomenon in our CF cohort, with a tendency to
hypogammaglobulinemia especially in children bellow 6 years of age.
Interestingly, such hypogammaglobulinemia is mostly caused by IgG2
deficiency. We also use immunoglobulin substitution with a very good effect
in these patients. We did not consider hypogammglobulinemia to be
"CVID-like" as IgG2 titers usually increased with age and infections. Also
no idea about the underlying mechanism.

Anna Sediva

On Wed, Dec 2, 2015 at 12:06 AM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
wrote:

> Dear Christine, Dear Kate,
>
>
>
> In the Leuven CF cohort we also tend to see hypogamma more frequently than
> expected and it is not at all related to nutritional status. We have been
> supporting several patients with profound hypogamma with immunoglobulin
> substitution and at least subjectively this has led to improvement with
> respect to the number of respiratory exacerbations. No idea about the
> mechanism though.
>
>
>
> Isabelle Meyts
>
> University Hospitals Leuven
>
>
>
>
>
>
>
> *Van:* CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
> *Verzonden:* dinsdag 1 december 2015 23:59
> *Aan:* CIS-PIDD <cis-pidd at lyris.dundee.net>
> *Onderwerp:* Re: [cis-pidd] cystic fibrosis and CVID
>
>
>
> I think the mechanism of hypogam is not clear.  As you correctly point
> out, there is a body of literature describing this association.  We still
> see it in spite of much improved nutrition do I don't think it is purely
> nutrition.
>
> Kate Sullivan
>
> Sent from my iPhone
>
>
> On Dec 1, 2015, at 5:56 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
> wrote:
>
> Dear Colleagues,
>
>
>
> Have any of you seen the coexistence of CF and CVID?  I will be seeing a
> 10y/o diagnosed at 5 years of age with CF (compound heterozygous pathogenic
> mutations).  She had recurrent lung and sinus disease leading up to the
> diagnosis.  She has no pancreatic exocrine dysfunction per pulmonary.
> Because of continuing lung infections that seemed out of proportion, she
> had an immune evaluation demonstrating IgG (low 300s mg/dl), IgM, and IgA
> 2SD below lower limit of normal. Normal vaccine titers to protein antigens
> but poor response to polysaccharide (pneumovax).  She was started on SCIG.
>
>
>
> Reviewing the literature, I find reports of hypogammaglobulinemia in CF
> patients but it has genereally been attributed to poor nutrition or
> hypothesized poor mucosal antigen presentation.
>
>
>
> Wondering if anyone has seen this in their practice and if so what was the
> clinical course?
>
>
>
>
> Christine M. Seroogy MD,  FAAAAI
> Associate Professor
> University of Wisconsin School of Medicine and Public Health
> Department of Pediatrics
> Division of Allergy, Immunology & Rheumatology
> 1111 Highland Avenue
> 4139 WIMR
> Madison, WI  53705-2275
> phone: 608-263-2652
> fax: 608-265-0164
>
>
>
>
>
>
>
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-- 
Prof. Anna Sediva, M.D., Ph.D.
Department of Immunology
2nd School of Medicine, Charles University
University Hospital Motol
V Uvalu 84
Prague, 15006
Czech Republic

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