[CIS-PAGID] (no subject)

[Terri Tarrant]

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 (A/I)

> (504) 896-9385 (Rheum)

>

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