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

[CIS-PIDD]

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


[image: photo]
*Glenn Reeves*
Assoc. Professor, Immunology/Immunopathology
+61 2 49214000 | +61 2 49214440 <fax:> | glenn.reeves at gmail.com |
coastalimmunology.com | c/o Pathology North, John Hunter Hospital, Lookout
Road, New Lambton Heights, Newcastle, NSW 2300
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On 10 February 2016 at 15:28, CIS-PIDD <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>
>
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