[CIS PIDD] [cis-pidd] CVID and severe pain

[Benjamin Soule]

I have a 46yo female patient with CVID who has been successfully treated from an infection standpoint with IVIG and prophylactic azithromycin.  She had been doing well for a year when she began to develop some vague joint and muscle pains.  Initially the pain resolved after the IVIG infusions for a week or two then slowly came back.  This benefit has decreased over time and now she has little or no benefit from her IVIG infusions.  On several occasions she has been given oral steroids.  These also helped with the pain initially, but are also no longer particularly effective.
She does not have any objective signs of joint inflammation (swelling, erythema, etc) and her ANA and other autoimmune antibodies have been negative.  Creatinine kinase and other markers of myositis have also been normal.  She has difficulty localizing the pain, but physical exam does not reveal specific weakness and she has been evaluated by a neurologist who has been unable to identify a cause for the pain.
Her other history includes treatment for non-hodgkins lymphoma at age 29, and premature ovarian failure.  She was diagnosed with CVID at age 35 and started on IVIG at that time though she had had recurrent infections since childhood.  More recently, at around the time the pain began, she had a slight increase in her creatinine that resolved without intervention.  She also had some GI symptoms (nausea, cramping, vomiting and diarrhea) that resolved when she started a gluten free diet (antibody testing and biopsy negative for celiac).  
After 8 months of worsening muscle and joint pain, she has now developed night sweats and headaches with no associated weight loss.  PET/CT was performed that did not revealed any apparent recurrence or new lymphoma.  She also had an episode of fleeting blindness for which she had an emergent temporal artery biopsy that did not reveal temporal arteritis.  She had an episode of chest pain for which she was admitted to the hospital and felt to have pleuritis.  Given these symptoms, we elected to try Rituximab for presumed autoimmune inflammatory neuropathy.  After the first infusion, she had complete resolution of her symptoms for three weeks.  The symptoms slowly returned and she was given a second infusion which was also effective for about three weeks.  A third infusion was less successful with resolution of symptoms for only 2 weeks and no further doses have been given.
I am wondering if anyone has seen anything similar or has any other ideas for evaluation and management.  She is essentially unable to work or take care of her children because of the pain which has continued to progress.
Thank you.

Benjamin Soule, MDUniversity of Pennsylvania - Allergy and Immunology SectionChildren's Hospital of Philadelphia - Allergy and Immunology DepartmentPhiladelphia, PA
 		 	   		  
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