[CIS PIDD] Sever joint pain with gamma globulin infusions

[Howard Lederman]

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>

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