[CIS PIDD] [cis-pidd] CVID/IVIg/heparin in patient with h/o PE

[CIS-PIDD]

Dear Bodo:

Thank you for the response.

In answer to your questions, our 37 year old patient with MS did have low immunoglobulins before receiving rituximab, and at our recent first visit my strong impression was that she has primary immunodeficiency accompanied by multiple autoimmune disorders (MS, Crohns, dermatomyositis). She is undergoing genetic analysis to see if we can provide further definition.


That noted, my neurology colleagues report that her MS history, exam and imaging are consistent with a classic MS, and she responded to rituximab for 10 months before recurrent symptoms (although another one of our similar patients who most likely DOES have CNS lymphocytic infiltration has remained symptom free on rituximab)- her genetic work-up is negative thus far.


Sincerely,

Katherine




Katherine Gundling, MD, FACP
Clinical Professor
Allergy and Immunology
UC San Francisco
400 Parnassus Ave. Box 0359
San Francisco, CA 94143
Clinic: 415-353-2725
Pager: 415-443-7682
________________________________
From: cis-pidd at lyris.dundee.net <cis-pidd at lyris.dundee.net> on behalf of CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
Sent: Sunday, September 17, 2017 11:49:31 AM
To: CIS-PIDD
Subject: Re: [cis-pidd] CVID/IVIg/heparin in patient with h/o PE

Dear Katherine,
To me, this is an interesting case.
Has she been hypogammaglobulinemic before receiving rituximab, or is the antibody deficiency secondary?
If it were primary, could the MS be a lymphocytic infiltration of the CNS as we see it in CTLA4 or LRBA immune dysregulation syndromes?
Alike, could the CNS phenotype (and the PE) be secondary to DADA2?
Both of these would require a change of treatment:
DADA2 > anti-TNF and no anticoagulation
Tregopathy (CTLA4/LRBA) > abatacept or alike
Did she respond well to rituximab treatment?
Yours Bodo

****************************************
Univ.-Prof. Dr. med. B. Grimbacher

Scientific-Director
CCI-Center for Chronic Immunodeficiency
UNIVERSITÄTSKLINIKUM FREIBURG
Tel.: 0761 270-77731  Fax: -77744
Breisacherstraße 115, 79106 Freiburg
bodo.grimbacher at uniklinik-freiburg.de
www.uniklinik-freiburg.de/cci

Von: <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>> on behalf of CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Antworten an: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Datum: Saturday, 16 September 2017 00:00
An: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Betreff: [cis-pidd] CVID/IVIg/heparin in patient with h/o PE


Dear colleagues:

I have a newly diagnosed, 37 year old CVID patient who needs IVIg before (and after) receiving rituximab for multiple sclerosis. She was diagnosed with PE in 2009 associated with Crohn disease (otherwise no clear etiology), and treated without complication or recurrent problems when anticoagulation was stopped.


Do any of you routinely give prophylactic subQ heparin (or other anticoagulation practice) before IVIg in patients with a history of PE?

Would you do so in this patient? She is ambulatory and has relatively good functional status in spite of the MS.


Your thoughts would be greatly appreciated.


Katherine


Katherine Gundling, MD, FACP
Clinical Professor
Allergy and Immunology
UC San Francisco
400 Parnassus Ave. Box 0359
San Francisco, CA 94143
Clinic: 415-353-2725
Pager: 415-443-7682

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