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

Tue Oct 20 09:37:52 EDT 2015

This is an old paper but maybe could help.

Clin Immunol
Immunopathol. 1994 Jan;70(1):60-5.
IgG2 deficiency in primary Sjögren's
syndrome and hypergammaglobulinemic purpura.
Eriksson P1, Almroth G,
Denneberg T, Lindström FD. 
Abstract
Total IgG and IgG subclasses were
studied in 34 patients with primary Sjögren's syndrome and 4 with
hypergammaglobulinemic purpura. Total IgG was elevated in 30/34 patients
with Sjögren's syndrome. IgG1 increase was responsible for the main part
of total IgG increase, contrasting with low levels of IgG2. The
difference in IgG1/IgG2 ratio between 38 patients as a group and 40
normal controls was statistically highly significant, but was not seen
in all patients. Six patients had markedly low levels of IgG2, but only
two had severe repeated respiratory infections. These observations
probably reflect selective autoantibody restriction to the IgG1
subclass. We conclude that patients with Sjögren's syndrome may be IgG2
subclass deficient despite elevated levels of total IgG, but also that
such deficiency in most instances does not cause a tendency to
infections. IgG subclass analysis may be of value to characterize
polyclonal IgG increase, since IgG1 subclass predominance often
indicates autoimmune disease.

Best regards 

Milica Mitrevski,
MD
Clinical Immunology and Allergy Specialist
PhD canditate in
Immunology
Sapienza University of Rome
Department of Clinical
Medicine
Viale dell'Università 37, 00185 Rome, Italy
tel: +39 06
49972036
 +39 349 5622376 
mail: mitmilica at tiscali.it

Il 20.10.2015
12:25 CIS-PIDD ha scritto: 

> 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 [4]
> 
>
www.uniklinik-freiburg.de/cci [5]
> 
> -----Ursprüngliche
Nachricht-----
> Von: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org
[6]] 
> 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 [7]
> 
> Am 20.10.15 11:32 schrieb
"CIS-PIDD" unter :
> 
>> 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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