Serial immunologic studies in patients with mucocutaneous lymph node syndrome (Kawasaki disease)
Lin, C.Y.; Hwang, B.
Annals of Allergy 59(4): 291-297
1987
ISSN/ISBN: 0003-4738 PMID: 3662131 Document Number: 297996
Twenty cses of mucocutaneous lymph node syndrome (MCLS), from Fall 1984 to April 1986, are included in this study. The serial immunoglobulin levels including IgG, IgA, IgM showed polyclonal increased immunoglobulin levels during the first month. Only one-fourth of patients had high serum IgE levels. There was no correlation with house dust mite antigen skin test and anti-mite IgE levels. By using the polyethylene glycol and ELISA methods, IgE-circulating immune complexes were detectable in 60% of high serum IgE patients. IgG-circulating immune complexes were also detected in 70% by the polyethylene glycol method and 60% by Raji cell method. CH50 and serum properdin factor B level increased during acute febrile phase. Serial T cell subset studies showed OKT4 cells decreased progressively and OKT8 levels were within normal range during the first and second week. By the third week, OKT4 cells increased and OKT8 cells decreased. This progressed to the fifth week and returned to normal range 2 months later. During the acute febrile phase of the first week, Leu2+ 15+ cells increased and Leu2+15- cells decreased. After the second week, the Leu2+15+ cells decreased and Leu2+15- cells increased. This increase continued to the fifth week when the Leu2+15+ cells were at the lowest level and the Leu2+15- cells at the higher level corresponding to the OKT8 change. After the fifth week, the Leu2+15+ cell and Leu2+15- cells returned to normal. Patients had increased antibody-dependent cell-mediated cytotoxic reaction at the first week of the acute febrile stage. It decreased dramatically in the second week and returned to normal l2 months later. These findings indicate abnormal immunoregulation contributes to the pathogenesis of this disease, including decreased antibody-dependent cell-mediated cytotoxic activity, so that in the initial stage the host is unable to clear the inciting pathogen. The reduced suppressor T cells and relatively elevated helper T cells contribute to the increased immunoglobulin levels and detectable circulating immune complexes after the third week of onset of disease. These abnormal immunologic responses may contribute to the development of vasculitis.