[CIS PIDD] [cis-pidd] Sever joint pain with gamma globulin infusions

Wed Mar 18 14:00:33 EDT 2015

I would suggest considering copper deficiency as this may be playing a role in the myalgias/neuralgias (I must credit my astute allergy fellow, Cosby Stone, MD, with this hypothesis). The only excipient listed in the package insert for gamunex is glycine which complexes with copper and could thereby decrease levels further around the time of infusions. The patient would be at increased risk for copper deficiency given prior gastric bypass surgery. She may also be supplementing with Fe and/or zinc which compete with copper for GI transport in the gut.

Please note that serum copper levels could be falsely elevated in this patient due to it being an acute phase reactant. You could check ceruloplasmin levels as well which can be low in copper deficiency.

>From a practical standpoint, we would recommend a trial of copper supplementation 4 mg daily the week before and the week following her next infusion. She likely would not need to continue this dose for a prolonged period of time.

Best,

John Fahrenholz, MD
Allergy and Immunology Section
Vanderbilt University School of Medicine
Nashville, TN
john.m.fahrenholz at vanderbilt.edu
phone 615-936-2727
fax 615-936-8493

________________________________
From: Howard Lederman [hlederm1 at jhmi.edu]
Sent: Wednesday, March 18, 2015 10:51 AM
To: CIS-PIDD
Subject: Sever joint pain with gamma globulin infusions

We have a 56 y/o lady with straightforward CVID (hypogammaglobulinemia with IgG 340 mg/dL; complete lack of response to vaccines) and problems with respiratory tract infections.    She has a complex medical history including gastric bypass, type 1 diabetes, congestive heart failure, and a recent pulmonary embolism and thrombophlebitis. She had no h/o joint pain.

We began replacement therapy with weekly subcutaneous infusions of Hizentra. She experienced severe joint pain dramatically limiting her mobility, and nausea.  We changed her to therapy to Gamunex, but she again developed disabling joint pain and impaired mobility She had similar problems of severe joint pain with Gamunex.

We referred her to a rheumatologist to be certain that she did not have an underlying arthritis (rheumatoid or other) that had presented coincidentally.  The rheumatologist noted:

She was pain free until she started on the Hizentra in April and had bilateral leg weakness about a month later. She has pain and weakness with changing from a seated position. She needs to push off with her arms. She has pain and weakness with stairs. With changing position the pain is in the knees and ankles. She has hip pain with rolling over onto her side. Her Hizentra was changed to Gamunex around June-July but her symptoms did not improve. She has been a diabetic on insulin for about 20 years. She has a history of peripheral neuropathy, on gabapentin for about 2 years. Her sugars are all over: low to 300s. She has a history of lumbar disk disease with bulging disks, stable for many years. She had an EMG in the 1980s. No swollen joints. She has edema in the legs. No knee x-rays. No physical therapy. She has been told that she has an elevated lactic acid over the past 2 years. She sees Dr. Birnbaum this month. At one time she was told that she had fibromyalgia. Appetite and weight are stable. She avoids analgesics.

The relevant part of her Px:  Neuro exam non-focal with negative straight leg raise, strength 5/5. Musculoskeletal:  A comprehensive musculoskeletal exam was performed for all joints of each upper and lower extremity and assessed for swelling, tenderness and range of motion.   No evidence of synovitis in the small joints of the hands, wrists, shoulders, elbows, hips, knees or ankles. Knees with crepitus. Tenderness over the buttocks, lateral trochanters, iliotibial band. Pes planus.

She has findings of osteoarthritis of the knees and I suspect a metabolic myopathy from diabetes. She also has a history of lumbar disk disease and has musculoskeletal buttock and leg pain. I see no evidence of inflammatory arthritis or connective tissue disease.

We discontinued SQ IgG infusions, and her joint pain, impaired mobility and weakness completely resolved in less than 2 weeks.  We then treated her with Gammagard and the symptoms promptly recurred, along with GI distress (constipation and diarrhea).

I have never had a patient with such severe joint pain with IgG infusions,  I am at a loss in how to proceed.  Does anyone have suggestions?

Howard

Howard M. Lederman, M.D., Ph.D.
Professor of Pediatrics, Medicine and Pathology
Division of Pediatric Allergy and Immunology
Johns Hopkins Hospital - CMSC 1102
600 N. Wolfe Street
Baltimore, MD 21287-3923
Phone: 410-955-5883
Fax: 410-955-0229
Email: Hlederm1 at jhmi.edu<mailto:Hlederm1 at jhem.jhmi.edu>

WARNING: E-mail sent over the Internet is not secure.
Information sent by e-mail may not remain confidential.

DISCLAIMER: This e-mail is intended only for the
individual to whom it is addressed. It may be used only in accordance with applicable laws. If you received this e-mail by mistake, please notify the sender and destroy the e-mail.

---

You are currently subscribed to cis-pidd as: john.m.fahrenholz at vanderbilt.edu<mailto:john.m.fahrenholz at vanderbilt.edu>.

To unsubscribe click here: http://cts.dundee.net/u?id=96396680.a5e0e4cb9bd0b7e926bd402dd0c48c50&n=T&l=cis-pidd&o=2672027

(It may be necessary to cut and paste the above URL if the line is broken)

or send a blank email to leave-2672027-96396680.a5e0e4cb9bd0b7e926bd402dd0c48c50 at lyris.dundee.net<mailto:leave-2672027-96396680.a5e0e4cb9bd0b7e926bd402dd0c48c50 at lyris.dundee.net>

---
You are currently subscribed to cis-pidd as: pagid at list.clinimmsoc.org.
To unsubscribe click here: http://cts.dundee.net/u?id=96396833.5a9591ccd1e327fe6bc4d1543298c482&n=T&l=cis-pidd&o=2672747
or send a blank email to leave-2672747-96396833.5a9591ccd1e327fe6bc4d1543298c482 at lyris.dundee.net
-------------- next part --------------
An HTML attachment was scrubbed...
URL: <https://pairlist7.pair.net/pipermail/pagid/attachments/20150318/e2c81a71/attachment-0001.html>