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

Wed Dec 2 17:37:23 EST 2015

Dear all, we absolutely agree with Isabelle. IVIG/scIG seems to be useful in this subgroup of CF patients with hypogammaglobulinemia. 

Pere. 

Pere Soler Palacín, MD, PhD, MSc. 
Pediatric Infectious Diseases and Immunodeficiencies Unit. Hospital Universitari Vall d'He bron. 
Vall d'Hebron Research Institute (VHIR) 
Assistant Professor. Universitat Autònoma de Barcelona (UAB) 
Pg. de la Vall d'Hebron, 119-129 
08035 Barcelona. Spain. 
Tel. 0034934893140 / Fax 0034934893039 

psoler at vhebron.net / 34660psp at comb.cat 
Web: www.upiip.com 
ORCID ID: http://orcid.org/0000-0002-0346-5570 
Scopus Author ID: http://www.scopus.com/authid/detail.url?authorId=55923378300 
ResearchGate: http://www.researchgate.net/profile/Pere_Soler-Palacin 
Linked In : http://es.linkedin.com/pub/pere-soler-palac%C3%ADn/73/918/b16 

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De: "CIS-PIDD" <cis-pidd at lists.clinimmsoc.org> 
Per: "CIS-PIDD" <cis-pidd at lyris.dundee.net> 
Enviats: Dimecres, 2 de Desembre 2015 0:06:03 
Assumpte: RE: [cis-pidd] cystic fibrosis and CVID 

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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