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

[CIS-PIDD]

I agree with Stephan, 
although it would be interesting to see how many of these patients really have an underlying monogenic disease. This is still an unanswered questions, since these patients are usually not genetically investigated. 
Do you have a B cell differentiation? In the connective tissue disorders you might see an expansion of plasmablasts and sw mem B cells while this is unusual for the above mentioned PIDs

Best regards

Klaus

Prof. Dr. med. Klaus Warnatz

MEDICAL CENTER - UNIVERSITY OF FREIBURG
Center for Chronic Immunodeficiency - CCI
Department of Rheumatology and Clinical Immunology

Breisacher Str. 117, 79106 Freiburg, Germany
Tel. +49 761 270 77640 / FAX -71000 / Pager 12-7100
klaus.warnatz at uniklinik-freiburg.de

www.uniklinik-freiburg.de/cci


-----Ursprüngliche Nachricht-----
Von: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org] 
Gesendet: Dienstag, 20. Oktober 2015 11:49
An: CIS-PIDD
Betreff: Re: [cis-pidd] adult patient with auto-immunity and recurrent airway infections

If PID, consider APDS (or CTLA-4 and STAT3 GOF - because of the "MS",
STAT1 GOF). Hyper- may precede hypogamma in these conditions.


Beste Grüße 

Prof. Dr. Stephan Ehl
Medizinischer Direktor

UNIVERSITÄTSKLINIKUM FREIBURG
CCI - Center for Chronic Immunodeficiency

Breisacher Str. 117 - 2. OG, 79106 Freiburg i. Brsg., Germany
phone: +49(0)761.270-77300
Sekretariat +49(0)761.270-77550  fax +49(0)761.270-77600
e-mail: stephan.ehl at uniklinik-freiburg.de





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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