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

[CIS-PIDD]

?       ?       ?

I am pretty sure that Erwin Gelfand published a case report of this combination in the late 1970's or early 1980's.



Howard
Howard M. Lederman, M.D., Ph.D.
Professor of Pediatrics, Medicine and Pathology
Division of Pediatric Allergy and Immunology
Johns Hopkins Hospital - CMSC 1102
600 N. Wolfe Street
Baltimore, MD 21287-3923
Phone: 410-955-5883
Fax: 410-955-0229
Email: Hlederm1 at jhmi.edu<https://mobile.johnshopkins.edu/OWA/redir.aspx?C=02aee18ab96e42ab8cb61c09ecb79487&URL=mailto%3aHlederm1%40jhem.jhmi.edu>

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________________________________
From: CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
Sent: Tuesday, December 1, 2015 5:54 PM
To: CIS-PIDD
Subject: [cis-pidd] cystic fibrosis and CVID

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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