[CIS-PAGID] (no subject)

[Verbsky, James]

Victoria

I agree with the prior comments.  I have had a similar patient with Wegener's who received a year of cytoxan, and then flared.  At this point his IgG was about 200 (1 year off cytoxan, only on cell-cept that he wasnt taking).  His albumin was very low.  I attributed his hypogam to protein loss (Kidney, also not uncommon to have GI loss in ANCA associated vasculitis), but because of the concern of retreating with another course of cytoxan, I gave him Rituxan.  The NEJM articles show equal effectiveness to cytoxan.  He has done well as I kept him on IVIG.  I would expect the adverse reactions to be the same as patients receiving Rituxan for other reasons.

I would try Rituxan even if B cells are low.  I frequently see low B counts in any inflammatory condition (For example..we have looked at a number of patients with systemic JRA..all had low B cells..all hypergam)..I assume this is just homing or redistribution from the blood.

Did your patient have low albumin prior to the diagnosis of MPA?  With protein loss, cytoxan, steroids..its going to be hard to tell whether this was CVID or just secondary immunosuppression effects.  Regardless..Rituxan is an reasonable option..my patient did well and has been put back in remission.

Best

James Verbsky MD/PhD
Assistant Professor of Pediatrics and Microbiology and Molecular Genetics
Medical College of Wisconsin
Milwaukee, WI  53226


________________________________
From: pagid-bounces at list.clinimmsoc.org [pagid-bounces at list.clinimmsoc.org] On Behalf Of Charlotte Cunningham-Rundles at mssm.edu [charlotte.cunningham-rundles at mssm.edu]
Sent: Monday, May 23, 2011 4:37 PM
To: PAGID
Subject: Re: [CIS-PAGID] (no subject)

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<https://owamail.mcw.edu/owa/UrlBlockedError.aspx>> 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)