[CIS-PAGID] (no subject)

[Routes, John]

I agree with Dr. Tarrant and worry about secondary immunosuppression as a primary diagnosis rather than CVID. Apart from cytoxan, corticosteroids are a common cause of hypogamm--steroids blunt specific Ab production and increase the catabolism of Igs —in steroid induced hypogamm there is a reduction in serum IgG more than other Ig isotypes. We use rituxin rather frequently in CVID (for a variety of problems) and have not had any problems.


Jack Routes, MD
Professor of Pediatrics, Medicine, Microbiology and Molecular Genetics
Medical College of Wisconsin




________________________________
From: Terri Tarrant <tarra002 at gmail.com>
Reply-To: "pagid at list.clinimmsoc.org" <pagid at list.clinimmsoc.org>
Date: Mon, 23 May 2011 12:32:34 -0500
To: "pagid at list.clinimmsoc.org" <pagid at list.clinimmsoc.org>
Subject: Re: [CIS-PAGID] (no subject)

We have experience with Rituximab in primary CVID, and all patients have done well.  We have used it for RA mostly, but also have one collagenous colitis, several ITP, a LIP, and a CNS granulomatous disesase associated CVID patient that did well without infectious problems.  I wonder given that your patient received cyclophosphamide whether or not he is a secondary immunodeficient from the medication since case reports have been described of vasculitis patients treated with cytoxan, anti-epilepsy medications, etc. who developed hypogam with or without lymphopenia.  We reviewed this topic in SLE (J Rheumatol. <javascript:AL_get(this, 'jour', 'J Rheumatol.');>  2003 Feb;30(2):412-4. PMID 12563705) and found that patients went into remission of their vasculitis if secondary lymphopenia was present.  Have you run flow cytometry on this patient since becoming active with ANCA vasculitis?  I'd be curious to know if the lymphopenia and CD19/CD20 deficiency is still present particularly if the ANCAs have returned in high titer.  If the renal disease is the predominant recurrence, you could ask your pathologist to stain the renal biopsy for anti-CD20 to see if B cells are localizing to the tissue rather than the blood.

Terri Tarrant, MD
Rheumatology, Allergy, Immunology
University of North Carolina

On Mon, May 23, 2011 at 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 <tel:%28504%29%20896-9589>  (A/I)
(504) 896-9385 <tel:%28504%29%20896-9385>  (Rheum)