[PAGID] Patient question

[Anita Gewurz]

Chris,

There have been 2 papers on Ureaplasma septic arthritis in CVID since  
1995, probably a long shot.

Anita Gewurz MD <agewurz at rush.edu>
Section of Allergy/Immunology
Department of
	Immunology/Microbiology
Rush University Medical Center
1725 W Harrison - 117
Chicago IL 60612


On Jun 22, 2007, at 9:42 AM, Chris Seroogy wrote:


> Thank you, Mary Ellen.  Here is some more information:

>

> 1. FISH was done for 22q11 deletion and was negative by report (I will

> reconfirm that.)

> 2. CVID diagnosis based on absent IgA and poor polysaccharide response

> (pneumovax, although isohemagglutins fair at 1:8), normal T cell  

> numbers and

> function.  IgM low at 32 and patient has been on IVIG for 3 years  

> for the

> laboratory values and recurrent sinusitis.  So perhaps this  

> diagnosis is

> "soft."

> 3. Allergy to insulin and b-lactams (desensitized to both and she has

> dexamethasone in her insulin pump.)

> 4. Several autoimmune problems:  type I DM, hypothyroidism

> 5. Chronic abdominal pain with ongoing GI evaluation.

> 6. Eczema

> 7. Factor V Leiden deficiency

>

> She is (was) a very active girl and highly intelligent with many  

> talents.

> Her growth has been okay.

>

> Regarding infectious work-up, she had EBV,CMV, HIV, toxo ruled out  

> by PCR or

> antibody testing.  Chest, sinus and abdominal CT with contrast  

> unrevealing ,

> bone scan negative.  The possibility of an infection was raised by  

> another

> one of our colleagues and I am looking into having one of the  

> joints scoped

> for fluid and synovial tissue if possible for a more definitive  

> diagnosis.

> Is enterovirus a possibility and would PCR on the joint fluid  

> provide the

> highest yield?

>

> I appreciate any insights/comments.  Regards, Chris

>

>

>

>

>

> On 6/22/07 9:02 AM, "Conley, Mary-Ellen" <maryellen.conley at STJUDE.ORG>

> wrote:

>

>> Hi Chris,

>> I think it might help to have a little more information about the  

>> patient.  A

>> significant proportion of the patients with Pierre-Robin sequence  

>> have

>> abnormalities of chromosome 22q11.  Do you know if this has been  

>> evaluated?

>> How is her growth and development?  Is she active?  What are the  

>> immunologic

>> findings that support the diagnosis of CVID (serum  

>> immunoglobulins; lymphocyte

>> cell surface markers; signs of autoimmune disease etc)?

>>

>> I am not sure that I would discard infectious etiologies just yet.

>>

>> Mary Ellen

>>

>>

>>

>>

>> Mary Ellen Conley, MD

>> Department of Immunology

>> St. Jude Children's Research Hospital

>> 332 N. Lauderdale

>> Memphis, TN 38105-2794

>> FAX  901-495-3977

>> TEL  901-495-2576

>>

>>

>> -----Original Message-----

>> From: pagid-bounces at list.clinimmsoc.org

>> [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Chris Seroogy

>> Sent: Thursday, June 21, 2007 6:58 AM

>> To: pagid at list.clinimmsoc.org

>> Subject: [PAGID] Patient question

>>

>> Dear Colleagues,

>>

>> I have a rheumatologic question on a very complicated CVID patient  

>> and am

>> hoping some of you have faced this management challenge before.   

>> This is a

>> 13y/o female with long-standing brittle type I DM, CVID (mostly  

>> functional

>> antibody production problems and IgA deficient), Pierre-Robin  

>> sequence, and

>> multiple drug allergies.  She presented to my clinic for second  

>> opinion re:

>> CVID management last Fall and at that time I noted significant  

>> arthritis is

>> several large joints and synovitis was confirmed by MRI and  

>> several bony

>> erosions were seen on plain xray.  Since this time, I have been  

>> treating her

>> arthritis (seronegative) aggressively with poor response.  She is  

>> on MTX and

>> humira, low dose daily prednisone.  She did not respond to Enbrel  

>> for 12

>> weeks, hence was switched to humira.  She receives IVIG every 3  

>> weeks (she has

>> problems with recurrent sinusitis.) Despite this management, she  

>> has had

>> significant progression of her arthritis now involving most joints  

>> and daily

>> pain.  I am not sure where to go next (thoughts are anakinra, bolus

>> steroids--although challenging with her DM.)  She was seen by our  

>> ID group and

>> her clinical picture/findings were not felt to be secondary to an  

>> infection.

>> She also had a negative bone scan.  I welcome any thoughts.

>> Regards, Chris

>>

>>

>> Chris Seroogy, M.D.

>>

>> Assistant Professor

>>

>> Dept. of Pediatrics

>>

>> Mail:  H4/474 CSC, Mailstop 4108

>>

>> Shipping:  H4/431 CSC, Mailstop 4108

>>

>> 600 Highland Ave.

>>

>> Madison, WI  53792

>>

>> phone: 608- 263-2652

>>

>> fax: 608-265-0164

>>

>>

>>

>>

>

>


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