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

[CIS-PIDD]

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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