Arthritis & Rheumatism, Volume 60,
October 2009 Abstract Supplement
The 2009 ACR/ARHP Annual Scientific Meeting
Philadelphia October 16-21, 2009.
Rheumatic Manifestations in Common Variable Immunodeficiency
Kanahara, Satoko M., Calabrese, Leonard H., Lang, David, Molloy, Eamonn S.
Common variable immunodeficiency (CVID) is a primary humoral immunodeficiency typified by hypogammaglobulinemia and recurrent sinopulmonary infections. A proportion of CVID patients may develop autoimmune diseases, especially autoimmune cytopenias. CVID has been associated with rheumatic diseases, but these have not been well-characterized to date. This study was conducted to evaluate the prevalence, disease course and treatment of rheumatic manifestations in CVID.
Retrospective chart review of patients with diagnosis of CVID from the Rheumatology and Allergy and Immunology Departments at the Cleveland Clinic.
The cohort of 45 CVID patients consisted of 32 females and 13 males, with median age of 51 years (range 19 78). 86% of the patients were Caucasian, 8.8% were African American. The median duration of disease since diagnosis was 5 years (range 1 41). 23/45 (52%) of the patients had at least one recorded history of moderate to severe joint pain, 85% of whom had multiple joint involvement. 14 patients were considered to have fibromyalgia, 9 were diagnosed with osteoarthritis and 5 patients had a clearcut inflammatory arthropathy. There were no proven cases of septic arthritis. Of the patients with inflammatory arthropathy, all 5 were treated with low doses of glucocorticoids, 4 with hydroxychloroquine, and 2 patients with methotrexate. One patient, with a concomitant diagnosis of ulcerative colitis, was sequentially treated with infliximab and abatacept. No patients developed radiographic erosions or joint deformity. There were no serious infectious complications associated with immunosuppressive therapy, although 4/5 patients were receiving immunoglobulin replacement therapy. One patient underwent synovectomy. Two patients with osteoarthritis had successful total knee replacement, without infectious complications.
Moderate to severe joint pain was noted in 23/45 (52%) in this cohort of CVID patients. Inflammatory arthritis was noted in 11% of the patients. In general, only modest immunosuppressive therapy was required for control of joint inflammation, and no patients developed destructive arthritis. As CVID patients may present initially with autoimmune phenomena even before onset of recurrent infections, rheumatologists should be cognizant of this disease association to facilitate diagnosis of CVID, and to minimize use of immunosuppressive therapy. Judicious use of immunosuppressive therapy appears to be well tolerated, once patients are established on immunoglobulin replacement therapy.
To cite this abstract, please use the following information:
Kanahara, Satoko M., Calabrese, Leonard H., Lang, David, Molloy, Eamonn S.; Rheumatic Manifestations in Common Variable Immunodeficiency [abstract]. Arthritis Rheum 2009;60 Suppl 10 :797