Immunologic Changes of Peripheral Blood Lymphocytes in Kawasaki Disease after Intravenous Gamma Globulin Therapy

Shim, Jung Yeon; Kim, Kyung Mo; Kim, Young Hwue; Hong, Soo Jong

Abstract

PURPOSE: Kawasaki disease(KD) is multi-systemic vasculitis and coronary artery abnormalities can develop up to 30 percent in untreated patients. Several observations suggest that immune activation and the secretion of cytokines from immune effector cells contribute to the pathogenesis of this disease. Though high dose intravenous gamma globulin(IVGG, >1 gm/kg) is effective in reducing the prevalence of coronary artery aneurysms in KD, the mechanism of IVGG is not yet well understood. To investigate the changes of immune effector cells, their activation, and risk factors for coronary artery aneurysms, we measured the peripheral blood lymphocyte subsets and their activation markers in KD patients with coronary artery lesions and compared them with those of patients without coronary artery lesions after IVGG treatment.
METHODS
The study group consisted of 11 patients with acute phase of KD including 4 patients with coronary artery lesions by 2D echocardiography, and 8 age-matched healthy controls. The peripheral blood mononuclear cells were collected by ficoll-hypaque, stained with monoclonal antibodies, and evaluated by flow cytometry. We compared the profile of peripheral blood lymphocyte subsets before IVGG(2 gm/kg, single dose) treatment, and at 5, 14 and 60 days after IVGG treatment.
RESULTS
1) Total T lymphocytes, CD4+ T lymphocytes, CD8+ T lymphocytes, and CD23+ B lymphocytes were decreased, but interleukin 2 receptor positive(IL-2R+) T lymphocytes and IgM+ B lymphocytes were increased in acute phase of KD when compared with normal healthy control. 2) Total T lymphocytes, CD8+ T lymphocytes, CD23+ T lymphocytes were increased and NK cells were decreased after IVGG treatment. 3)Total T lymphocytes and CD8+ T lymphocytes were significantly increased at 5 days and 14 days, respectively after IVGG treatment, and IgM+ B lymphocytes and IL-2R+ T lymphocytes were significantly decreased at 14 days and 60 days, respectively in the patients with coronary artery lesions when compared with those without coronary artery lesions.
CONCLUSIONS
Total T lymphocytes are decreased and T lymphocytes are activated in acute phase of KD, especially in patients with coronary artery lesions and that findings are recovered after IVGG treatment as those without lesions.