[CIS PIDD] [cis-pidd] adult patient with auto-immunity and recurrent airway infections

[CIS-PIDD]

Dear Esther,
My assessment would be primarily immune dysregulation (autoimmunity).
I would not hesitate to give her immunosuppressive treatment e.g. for her
MS (including rituximab), but I also like the idea of trying with IVIG
first, as you might catch two birds with one stone?
Your patient has both: Sjögren¹s (which btw also predisposes her to the
upper resp. infections) AND a selective IgG2 deficiency.
She should be monitored for B cell lymphoma.
Thinking about genetics, I would exclude CTLA4 and stop there. Everything
else is far-fetched...
Yours, Bodo

****************************************
Univ.-Prof. Dr. med. B. Grimbacher
 
Scientific-Director
CCI-Center for Chronic Immunodeficiency
UNIVERSITÄTSKLINIKUM FREIBURG
Tel.: 0761 270-77731  Fax: -77744
Engesserstraße 4, 79108 Freiburg
bodo.grimbacher at uniklinik-freiburg.de
www.uniklinik-freiburg.de/cci
 
and 
 
Consultant Immunologist
Institute of Immunity & Transplantation
Dept of Immunology
Royal Free Hospital
UNIVERSITY COLLEGE LONDON
Pond Street
London NW3 2QG
b.grimbacher at ucl.ac.uk

www.centreforimmunodeficiency.com






Am 20/10/15 11:32 schrieb "CIS-PIDD" unter <cis-pidd at lists.clinimmsoc.org>:

>A 63-year-old woman who has been diagnosed with Sjögren syndrome and
>multiple sclerosis was referred to me because of recurrent upper and
>lower airway infections since 10 years, chronic cough, and
>bronchiectasis with the question whether she has immunodeficiency. She
>has not been using any immunosuppressive medication for many years, nor
>has she ever been treated with IVIG because of her MS.
>Her lab shows auto-antibodies, hypergammaglobulinemia with IgG2
>deficiency, and partial polysaccharide antibody deficiency (details in
>the attachment, Ig's in g/l).
>Should we consider her to be a patient with primarily auto-immune
>disease, or a patient with a form of primary immunodeficiency, and if
>so, which?
>Your suggestions are welcome!
>Esther de Vries, 's-Hertogenbosch/Tilburg, the Netherlands
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