[CIS-PAGID] (no subject)

[Dimitriades, Victoria R.]

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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