[CIS-PAGID] CVID and vasculitis

[Dimitriades, Victoria R.]

Thank you all for your input!
 
I should clarify- his cyclophosphomide therapy lasted approximately four months and methotrexate was for six months. It has now been two years since those treatments ended and one year since any prednisone. We entertained the ideas of both T and B cell suppression from these meds early on, but now contininue to show low T/B cells and a low IgA and IgM (which is new). Would you think this suppression could last this long? Additionally, he had a very clear history of recurrent pneumonias and sinus infections which started well before his vasculitis issues (requiring admissions and IV antibiotics). 
 
In regards to lymphocyte subpops during vasculitis flare- we have not had this issue in this patient since he has been with us. He had not had them done during his initial diagnosis, either. However, I will repeat them now to see if they have changed in any way. His renal function is also stable, but I will also keep a biopsy stain in mind if needed...
 
Thanks again-
 
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)

________________________________

From: pagid-bounces at list.clinimmsoc.org on behalf of Terri Tarrant
Sent: Mon 5/23/2011 12:32 PM
To: 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. 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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