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

[CIS-PIDD]

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<mailto: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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