[CIS-PAGID] (no subject)

[Charlotte Cunningham-Rundles@mssm.edu]

Hi,

Not sure if this is a clear CVID as he has been on immune suppressants so
that makes the workup difficult to really interpret. The immune studies
might be altered on that account.    Not to say that the Ig did not help,
and not to say he is not immune deficient, it is just not a case that would
be included in collections of CVID subjects, based on what you can knew
about him.  

As for ongoing rx that makes it more a judgment call.  -- and if the
literature suggests it will help. We have used Rituxan regardless of the B
cell numbers in ITP and it works well there.

Charlotte


On 5/23/11 1:08 PM, "Dimitriades, Victoria R." <varsen at lsuhsc.edu> wrote:


> Dear Colleagues-

>  

> As I have seen a recent flurry of activity regarding autoimmunity, I thought I

> could take this opportunity to ask about a patient whom I see with CVID and

> vasculitis:

>  

> Thia is a 60 year old male whom I started to see 3 years ago as a referral

> from his rheumatologist-- 2 years prior he had been diagnosed with microscopic

> polyangitis (with severe kidney and lung disease) and was started on

> cyclophosphamide and prednisone. After several months on cyclophos, he was

> hospitalized for pseudomonal sepsis and  was then changed to methotrexate and

> a (2 year) steroid taper.

> When I saw him, he had a clear recurrent infection history preceding his

> vasculitis (by 10 years) and low (but not absent) IgG and IgA with no

> pneumococcal response. Additionally, he has low T cell subsets and B cell

> percentages (1%) as well. He was started on subcutaneous Ig and is currently

> infection-free, with a prophylactic antibiotic daily. He no longer takes

> prednisone or immunosuppressant meds and his vasculitis has been under control

> for the last three years.

>  

> Recently, because of elevations in his inflammatory markers and ANCA, his

> rheumatologist would like to restart his treatment. Because of his difficulty

> previously with cyclophosphamide and steroids, he would like to start

> Rituximab, 4 doses, once weekly as a solo therapy (this has been recently used

> as a therapy for ANCA-positive vasculitides).

> I have seen Rituximab used for ITP in CVID patients, but does anyone have any

> experience in vasculitis? I assume he will be protected somewhat given his Ig

> replacement and antibiotics, but are there any other precautions I should

> take?

> Furthermore, given his extremely low B cell numbers, will this even be

> effective? (I guess if they are proliferative enough to churn out

> autoantibodies, they may also be likely to be affected...)

>  

> I appreciate any guidance you may have--

>  

>  

> Victoria Dimitriades, MD

> Assistant Professor of Pediatrics

> Divisions of Allergy/Immunology and Rheumatology

> Louisiana State University Health Sciences Center

> Children's Hospital of New Orleans

> 200 Henry Clay Ave

> New Orleans, LA 70118

> (504) 896-9589 (A/I)

> (504) 896-9385 (Rheum)

> 


-------------- next part --------------
An HTML attachment was scrubbed...
URL: <http://seven.pairlist.net/mailman/private/pagid/attachments/20110523/31fb7474/attachment.htm>