[CIS-PAGID] IVIG reaction

[Patel, Niraj C]

Dear Colleagues,

I saw this patient for the first time this week, and she has extreme difficulty tolerating IVIG infusions.
45 yo female with lupus since 1994, history of pericarditis, antiphospholipid syndrome, oral ulcers and peripheral neuropathy. She received epratuzumab (antiCD22) for lupus in June 2008 (IgG level prior was 610).  She was started on IVIG in March 2009 for low IgG 530 (normal IgA, IgM) and chronic sinusitis despite. No antibody to vaccines was done.  She initially tolerated IVIG (400mg/kg) for several months (IgG levels in 700-800), until she began developing headaches, vomiting, fever.  No laryngeal swelling, wheezing, or hives.  Despite premedication with 50mg Benadryl, changing IVIG formulations, 20mg demamethasone the night prior and 20mg the morning of infusion, decadron (unknown dose) prior to infusion, and rate slowed to 70cc/hr (15 hour-long infusion), her symptoms worsened. She had aseptic meningitis in May 2011 and Nov 30 2011 thought due to IVIG, although the latter episode occurred 6 days after infusion and no lumbar puncture done either time. Symptoms included fever, neck pain, vomiting, photophobia and was hospitalized for 1 week each time and treated with high-dose steroids.

During the almost 2 years on IVIG, she noted remarkable improvement in sinus symptoms and had just 1 sinusitis during this time period (compared to chronic nasal symptoms and antibiotics at least once monthly prior to IVIG).  She stopped her IVIG after Nov 30 2011 due to adverse reaction and her chronic nasal symptoms returned after 4-6 weeks.  CT of sinus this week was negative except scant sphenoid fluid and endoscopy of nasal passages was normal (she was on levoquin at that time).  Most recent labs on 1/26/12:

IgG 563 (791-1643)
IgA 89 (66-436)
IgM 75 (43-279)
WBC 9,100
ALC 1,065
CD19B 53 (90-660)
CD3T 809 (690-2,540)
CD4T 405 (410-1,590)
CD8T 362 (195-1,140)
CD56/16 181 (90-590)

1) Would you restart Ig replacement? Try subQ in a monitored setting?
2) Hold on Ig replacement therapy until more definitive evidence of a chronic infectious process?
3) Could an autoantibody to Ig be present in this setting?  If so, offer rituximab?

Thank you in advance for your help.

Niraj

Niraj Patel, MD MS

Department of Pediatrics
Infectious Diseases and Immunology
 Levine Children's Hospital
Carolinas Medical Center
PO Box 32861
Charlotte, NC 28232-2861

Tel:   (704) 381-6803
Fax:  (704) 381-6841
Appt: (704) 381-8840

Email: niraj.patel at carolinashealthcare.org<mailto:niraj.patel at carolinashealthcare.org>


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