[CIS PIDD] [cis-pidd] granulomatous hepatitis of unknown etiology and low IgG

Fri Apr 8 07:39:57 EDT 2016

I concur with Bodo- I have had extremely good luck with MMF.  I often need steroids at the outset but then can go with MMF as mono therapy.

Kate Sullivan, MD PhD
Wallace Chair
Chief of Allergy Immunology
ARC 1216 CHOP
3615 Civic Center Blvd.
Philadelphia, PA 19104
(p) 215-590-1697
(f) 267-426-0363

On Apr 8, 2016, at 7:33 AM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> wrote:

This data does not speak against CVID, does it?
Anti-TNF would not be the first choice for a steroid-sparing agent in treating granulomatous CVID though.
We prefer MMF, recently abatacept was published efficient for the lung.
Best 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<mailto:bodo.grimbacher at uniklinik-freiburg.de>
www.uniklinik-freiburg.de/cci<http://www.uniklinik-freiburg.de/cci>

Von: 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: Wednesday 17 February 2016 21:28
An: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Betreff: RE: [cis-pidd] granulomatous hepatitis of unknown etiology and low IgG

Hello,

here is an update on a patient's labs I emailed last week:

Regarding patient's B cel panel done at Cincinnati Childrens and the results are below:

Decreased absolute B cells (50 cells/ul).
Increased % total naive (CD27-) B cells  86 % (CD19+CD27+);
Decreased % of total memory B cells 14 % (CD19+CD27+,
Class switchemd memroy B cells are 3.2 % (low CD19+CD27+IgM-IgD- (nl 4.7-22.1);
Transitional B cell populations are normal limits
CD19+CD5+ cells are low at 5%.

She does have low WBC count 2640, %neuto 58%, %mono 12%; and % lym 24%

Her WBC has range from 2000-3000 range with apatietn on 40 mg prednisoen sicne 1/31/15

Team is considering Imfiximab for treatment of CVID granulomatous disease and I am hesitant to recommend this without vaccination and not an absolute low IgA.

She received another IVIG treatment 2/10/16  and IgG is at 569 mg/dl on 2/16/16; IgA and IgM has been normal.

The thought process is that her lymph system is causing her loss of the IgG from the liver team's perspective.

With the B cell panel, I was hoping her trnasitional cells would be elevated and her Class siwthced memory B cells would be <2% but it is not.

Any thoughts?

Tracy Hwangpo

Instructor, Division of Clinical Immunology & Rheumatology

Department of Medicine

thwangpo at uabmc.edu<https://webmail.uabmc.edu/owa/redir.aspx?C=Jvh21Y4R5Ey8El7HpI-8P70cvHtMgtIIoyupOWh-LnQ10U6acu4koIOBQhs3ZMe_AqV8kNSBks8.&URL=mailto%3athwangpo%40uabmc.edu>

________________________________
From: CIS-PIDD [cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>]
Sent: Wednesday, February 10, 2016 12:26 AM
To: CIS-PIDD
Subject: RE: [cis-pidd] granulomatous hepatitis of unknown etiology and low IgG and IgA -??

Hi,

Not yet but I will get them sent out to be done at Children's Cincinnati.  I assume is to look at class switched memory cells %.  She is on 40 mg prednisone currently (10 days already).  I have yet to check a vaccine response on this patient due to her current state of immunosuppression.

She has lymphopenia with WBC 3200, absolute lymphocytes 672 with absolute T cells at 507 cells/ul and absolute B cells at 45 cells/ul with normal distribution of peripheral blood T (CD3 75%) and B cells (CD19 7%)
She had 3.3% CD3+, CD4/8-, TCRalphabeta+ cells (mild increase from our normal values < 2.5%).  This lab was done 4 days in steroid treatment.

Thank you for your advice,
Tracy Hwangpo

MD/PhD

Instructor, Division of Clinical Immunology & Rheumatology

Department of Medicine

thwangpo at uabmc.edu<https://webmail.uabmc.edu/owa/redir.aspx?C=Jvh21Y4R5Ey8El7HpI-8P70cvHtMgtIIoyupOWh-LnQ10U6acu4koIOBQhs3ZMe_AqV8kNSBks8.&URL=mailto%3athwangpo%40uabmc.edu>

________________________________
From: CIS-PIDD [cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>]
Sent: Tuesday, February 09, 2016 10:46 PM
To: CIS-PIDD
Subject: Re: [cis-pidd] granulomatous hepatitis of unknown etiology and low IgG and IgA -??

I agree that CVID is the most likely explanation, with granulomatous hepatitis and nodular regenerative hyperplasia  being quite common accompaniments of CVID. ?False positive brucella serology. Were B lymphocyte subsets analysed?
Regards Glenn

[photo]
Glenn Reeves
Assoc. Professor, Immunology/Immunopathology
+61 2 49214000<tel:+61%202%2049214000>| +61 2 49214440<https://webmail.uabmc.edu/owa/UrlBlockedError.aspx>| glenn.reeves at gmail.com<mailto:glenn.reeves at gmail.com>| coastalimmunology.com<https://urldefense.proofpoint.com/v2/url?u=http-3A__coastalimmunology.com&d=BQMFaQ&c=o3PTkfaYAd6-No7SurnLtwPssd47t-De9Do23lQNz7U&r=qmVY27Bu3sfBG9T8CX6NMV1ZlRj4STnnvI4pXBOFdCw&m=_NS1sbzh2MvUJaQOloQYCvJ2Y7HXmM_sMcfsUsqpZV8&s=ud7iFlltbte-G57HyaQ8nnXn3fIZNDS6Cz0dyj77RFs&e=>| c/o Pathology North, John Hunter Hospital, Lookout Road, New Lambton Heights, Newcastle, NSW 2300

[http://ws-promos.appspot.com/ga/pixel.png?yes__count=1&e=5732568548769792]
Get a signature like this: Click here!<https://urldefense.proofpoint.com/v2/url?u=http-3A__ws-2Dpromos.appspot.com_r-3Frdata-3DeyJydXJsIjogImh0dHA6Ly93d3cud2lzZXN0YW1wLmNvbS9lbWFpbC1pbnN0YWxsP3dzX25jaWQ9NjcyMjk0MDA4JnV0bV9zb3VyY2U9ZXh0ZW5zaW9uJnV0bV9tZWRpdW09ZW1haWwmdXRtX2NhbXBhaWduPXByb21vXzU3MzI1Njg1NDg3Njk3OTIiLCAiZSI6ICI1NzMyNTY4NTQ4NzY5NzkyIn0-3D-26ws-5Frandom-5Fnumber-3D&d=BQMFaQ&c=o3PTkfaYAd6-No7SurnLtwPssd47t-De9Do23lQNz7U&r=qmVY27Bu3sfBG9T8CX6NMV1ZlRj4STnnvI4pXBOFdCw&m=_NS1sbzh2MvUJaQOloQYCvJ2Y7HXmM_sMcfsUsqpZV8&s=tVX0O-WZ9VuXZHtvmriZG45RNv9CZ3nUox5exqNGe6c&e=>

On 10 February 2016 at 15:28, CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> wrote:

Hello,

I am seeing a 29 year old female w/ recently diagnosed granulomatous hepatitis of unknown etiology (January 2016-1st admission) and low IgG (noted in current/2nd admission).  Prior to coming to university hospital, she had her gallbladder removed and her liver was knicked in Dec 2015 when she was being evaluated locally for abdominal girth.   She was transferred due to ascites and hepatosplenomegaly.

She has extensive work-up with no overt infection found except for a positive IgM antibody to brucellosis.  I saw her for the first time today.  She has had recurrent sinusitis since childhood and had T & A removed at age 17 due to recurrent strep throat.  She had ear infections but did not need PET tubes.  She had possible meningitis at age 19 and was hospitalized for 2 weeks for that and we hope to get her discharge records from that time period to learn more about that hospitalization since her memory is vague regarding this.

I am trying to determine if CVID as a potential diagnosis for the cause of her problems.  She had a biopsy of her liver that showed granulomatous hepatitis on 1/4/16 .  Her CT lung  is fine.  She does have hepatosplenomegaly.  She had LN biopsy done at OSH and the slide reviewed at our university hospital  confirmed  Numerous non-caseating granulomas effacing normal nodal architecture.

Has any folks seen granulomatous hepatits as presenting complaint for CVID or have you seen other immunodeficiency associated with granulomatous hepatitis?

Labs of interest:

Globulins 0.34 (low, nl 0.6-1) 1/29/16
IgG 194 (low, nl 634-1,700), IgA 64 (low; nl 78-436), IgM 206 (normal) - 1/23/16

IgG 201 (low, nl 634-1,700), IgA 78 (low; nl 66-436), IgM 188 (nl 45-281 ) - 1/29/16

- Brucella IgG wnl, IgM elevated- unreliable  BRUCELLA IgG 0.07, IgM 1.19 H (<1.10)

Cell markers 2/4/16: "there is a moderate lymphopenia with a relatively normal distribution of peripheral blood T and B cells. TCRalphabeta positive CD4/CD8 negative T cells are mildly increased. NKT cells are prominent. Increased T cell HLA-Dr expression indicates activation."

Post IVIG infusion:

1/30/16:
IgG = 619

Steroids started 1/30/16 for presumably sarcoidosis (as a diagnosis of exclusion).

1 week later

2/5/16:
IgG = 322
IgA = 72
IgM = 133

Tetanus IgG Ab = 1.08
Anti-A iso titer = 1:32
Anti-B iso titer = 1:64
CH50 = 70.2
FLEXITEST 1 NEISSERIA MENINGITIDIS IGG VACCINE RESPONSE, MAID
SEROGROUP A <0.5 L ug/mL
SEROGROUP C <0.5 L ug/mL
SEROGROUP Y <0.5 L ug/mL
SEROGROUP W-135 <0.5 L ug/mL

- CT with hepatosplenomegaly but otherwise normal w/o enlarged nodes to biopsy

- Pathology has reviewed lymph node from Pratville - lymph node consumed with granulomas. no normal lymph tissue.

- path report of normal peritoneum without granulomas. AFB stain negative and flow cytometry negative.

- Empirically treating for infiltrative process like sarcoid; also brucella as above concurrently with prednisone 40mg daily, Doxycycline, and Rifampin since 1/31/2016 ( Day 10 )

Thank you for your help,

Tracy Hwangpo

MD/PhD

Instructor, Division of Clinical Immunology & Rheumatology

Department of Medicine

thwangpo at uabmc.edu<https://webmail.uabmc.edu/owa/redir.aspx?C=Jvh21Y4R5Ey8El7HpI-8P70cvHtMgtIIoyupOWh-LnQ10U6acu4koIOBQhs3ZMe_AqV8kNSBks8.&URL=mailto%3athwangpo%40uabmc.edu>

---

You are currently subscribed to cis-pidd as: glenn.reeves at gmail.com<mailto:glenn.reeves at gmail.com>.

To unsubscribe click here: http://cts.dundee.net/u?id=101246636.556f43fd47bd35dc0c45b0f36352c004&n=T&l=cis-pidd&o=3467266<https://urldefense.proofpoint.com/v2/url?u=http-3A__cts.dundee.net_u-3Fid-3D101246636.556f43fd47bd35dc0c45b0f36352c004-26n-3DT-26l-3Dcis-2Dpidd-26o-3D3467266&d=BQMFaQ&c=o3PTkfaYAd6-No7SurnLtwPssd47t-De9Do23lQNz7U&r=qmVY27Bu3sfBG9T8CX6NMV1ZlRj4STnnvI4pXBOFdCw&m=_NS1sbzh2MvUJaQOloQYCvJ2Y7HXmM_sMcfsUsqpZV8&s=gV5Lj0_Ez5BUfp8tP-lG2_-VknM2Awmz0vLu-5cCVCY&e=>

(It may be necessary to cut and paste the above URL if the line is broken)

or send a blank email to leave-3467266-101246636.556f43fd47bd35dc0c45b0f36352c004 at lyris.dundee.net<mailto:leave-3467266-101246636.556f43fd47bd35dc0c45b0f36352c004 at lyris.dundee.net>

---

You are currently subscribed to cis-pidd as: thwangpo at uabmc.edu<mailto:thwangpo at uabmc.edu>.

To unsubscribe click here: http://cts.dundee.net/u?id=98953993.b38e4cc85cdd91c8da89882c6c0418d4&n=T&l=cis-pidd&o=3467277<https://urldefense.proofpoint.com/v2/url?u=http-3A__cts.dundee.net_u-3Fid-3D98953993.b38e4cc85cdd91c8da89882c6c0418d4-26n-3DT-26l-3Dcis-2Dpidd-26o-3D3467277&d=BQMFaQ&c=o3PTkfaYAd6-No7SurnLtwPssd47t-De9Do23lQNz7U&r=qmVY27Bu3sfBG9T8CX6NMV1ZlRj4STnnvI4pXBOFdCw&m=_NS1sbzh2MvUJaQOloQYCvJ2Y7HXmM_sMcfsUsqpZV8&s=1cJ2PnlmnIa3HWLeBgGWoG_1r3wT6KvR0UI_uEk92dg&e=>

(It may be necessary to cut and paste the above URL if the line is broken)

or send a blank email to leave-3467277-98953993.b38e4cc85cdd91c8da89882c6c0418d4 at lyris.dundee.net<mailto:leave-3467277-98953993.b38e4cc85cdd91c8da89882c6c0418d4 at lyris.dundee.net>

---

You are currently subscribed to cis-pidd as: thwangpo at uabmc.edu<mailto:thwangpo at uabmc.edu>.

To unsubscribe click here: http://cts.dundee.net/u?id=98953993.b38e4cc85cdd91c8da89882c6c0418d4&n=T&l=cis-pidd&o=3467454<https://urldefense.proofpoint.com/v2/url?u=http-3A__cts.dundee.net_u-3Fid-3D98953993.b38e4cc85cdd91c8da89882c6c0418d4-26n-3DT-26l-3Dcis-2Dpidd-26o-3D3467454&d=BQMFAw&c=o3PTkfaYAd6-No7SurnLtwPssd47t-De9Do23lQNz7U&r=qmVY27Bu3sfBG9T8CX6NMV1ZlRj4STnnvI4pXBOFdCw&m=f6L4KqRSFNS6uGD_R79yZak0VDPIYEhMp6GQQLfAcs0&s=4a-Mx-a_PEWqBNLeEjLLiRlocwJdiQPQD1rE6vFlJTo&e=>

(It may be necessary to cut and paste the above URL if the line is broken)

or send a blank email to leave-3467454-98953993.b38e4cc85cdd91c8da89882c6c0418d4 at lyris.dundee.net<mailto:leave-3467454-98953993.b38e4cc85cdd91c8da89882c6c0418d4 at lyris.dundee.net>

---

You are currently subscribed to cis-pidd as: bodo.grimbacher at uniklinik-freiburg.de<mailto:bodo.grimbacher at uniklinik-freiburg.de>.

To unsubscribe click here: http://cts.dundee.net/u?id=96396431.4f2a33ac30bfa58d3a76b5fb5b03d33a&n=T&l=cis-pidd&o=3479430

(It may be necessary to cut and paste the above URL if the line is broken)

or send a blank email to leave-3479430-96396431.4f2a33ac30bfa58d3a76b5fb5b03d33a at lyris.dundee.net<mailto:leave-3479430-96396431.4f2a33ac30bfa58d3a76b5fb5b03d33a at lyris.dundee.net>

---

You are currently subscribed to cis-pidd as: sullivank at email.chop.edu<mailto:sullivank at email.chop.edu>.

To unsubscribe click here: http://cts.dundee.net/u?id=99424140.0252c6305bcec2418e0f99fbf64e026c&n=T&l=cis-pidd&o=3574244

(It may be necessary to cut and paste the above URL if the line is broken)

or send a blank email to leave-3574244-99424140.0252c6305bcec2418e0f99fbf64e026c at lyris.dundee.net<mailto:leave-3574244-99424140.0252c6305bcec2418e0f99fbf64e026c at lyris.dundee.net>

---
You are currently subscribed to cis-pidd as: pagid at list.clinimmsoc.org.
To unsubscribe click here: http://cts.dundee.net/u?id=96396833.5a9591ccd1e327fe6bc4d1543298c482&n=T&l=cis-pidd&o=3574251
or send a blank email to leave-3574251-96396833.5a9591ccd1e327fe6bc4d1543298c482 at lyris.dundee.net
-------------- next part --------------
An HTML attachment was scrubbed...
URL: <https://pairlist7.pair.net/pipermail/pagid/attachments/20160408/d6b00c71/attachment-0001.html>