[CIS PIDD] Fwd: [cis-pidd] oral Ig Therapy

[Clinical Immunology Society]

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From: CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
Date: Tue, Mar 13, 2018 at 2:53 PM
Subject: [cis-pidd] oral Ig Therapy
To: CIS-PIDD <cis-pidd at lyris.dundee.net>


All,

One of my partners here at Wake has a CVID patient that needs to be
considered for oral Ig therapy and has some questions.  Below is the case
and questions.





I am asking for advice on the management of this patient:

42 year old female with CVID (initially diagnosed in 1998, on IVIG),
bronchiectasis with pulmonary granulomas, diabetes, chronic anemia, GERD,
depression/anxiety.  Repeat CT of the chest in 9/2012 with the following
findings:



Regarding immune replacement, she was on Gammagard for many years with IgG
trough levels around ~800-1000.  A few years ago, her trough level began
decreasing, and Gammagard dose was increased up to max of 90gm q3 weeks
(weight ~100kg).  She continued to have low IgG troughs levels on this
dose, so she was transitioned to subcutaneous immunoglobulin (55gm/month to
start, now in the process of increasing to 65gm/month as still suboptimal
replacement – IgG level 544 at steady state) to hopefully have more stable
levels.



Unfortunately, at her last visit with me (10/25/17), she reported chronic
diarrhea and weight loss (17lbs, somewhat intentional, but more than
expected given her diet and workout schedule).  GI pathogen panel with
+norovirus.  I discussed with a colleague in ID, and this was suspected to
be chronic norovirus given longstanding symptoms without any acute change
in diarrhea/nausea or other symptoms of acute VGE (fever, etc).  We decided
to treat with 3 months of ribavirin, which she has now completed.  In the
interim, she had short lived improvement in diarrhea/nausea with ribavirin
and discontinuation of metformin, but now symptoms are back.  She was
hospitalized recently with paresthesias and tetany and found to have
hypocalcemia, hypomagnesemia, and hypokalemia.  These electrolyte
abnormalities were attributed to chronic diarrhea, and now she is on
replacement electrolytes including magnesium (which is likely making
diarrhea worse).  She does improve with Imodium, but only temporarily.
Norovirus was negative during her admission.  She also had normal
parathyroid and thyroid studies.



She also tells me that she often has incontinence of stool (usually at
night while sleeping) and has severe episodes of nausea.  She has lost her
appetite and is losing weight (no longer trying to lose weight and down* 23
more lbs* since her visit with me in October).

 She says she has not had a formed stool in > 6 months.  She denies blood
in the stool.  She had a colonoscopy in ~2014, normal per her report. She
also remotely had an EGD, also reportedly normal.  I checked stool
enterovirus PCR and this was positive.  I have referred her to GI here,
appointment in April.

Questions:

As far as management of her Enterovirus, I am planning on using oral
immunoglobulin.  I have seen recommendations for 150-300mg/kg dosing for
enteroviral shedding in immunocompromised patients.  Is this dose what I
should use?  Also, is this typically administered directly into the
jejunum/ileum/etc via enteral tube to avoid gastric degradation of
immunoglobulin? Is one dose sufficient, or are serial doses usually
required?

I have seen recommendations for exome sequencing for CVID patients with
enteropathy to guide management (like with abatacept, etc).  Do you think
this is necessary now, or should I wait until after the oral IG to see if
she has resolution of symptoms with treatment of Enterovirus?

Are there any specific other interventions/tests/diagnostics that I should
request from GI when they see her?





Jason W Caldwell DO FAAAAI

Associate Professor of Internal Medicine and Pediatrics

Section of Pulmonary, Critical Care, Allergic and Immunological Diseases

Program Director of Allergy/Immunology Fellowship

Wake Forest University School of Medicine

Office: 336-716-5166

Administrative: 336-716-4843

Pager: 336-806-8330

jcaldwel at wakehealth.edu <jcaldwel at wfubmc.edu>

[image: Wake Forest School of Medicine]



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