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Kawasaki Disease (Mucocutaneous...
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PEDIATRICS Vol. 112 No. 4 October 2003, pp. 986-992

EXPERIENCE AND REASON

Longstanding Obliterative Panarteritis in Kawasaki Disease: Lack of Cyclosporin A Effect

Taco W. Kuijpers, MD, PhD, Maarten Biezeveld, MD, Annemiek Achterhuis, MD, Irene Kuipers, MD, PhD, Jan Lam, MD, C. E. Hack, MD, PhD, Anton E. Becker, MD, PhD and Allard C. van der Wal, MD, PhD

Emma Children’s Hospital, Academic Medical Center, Meibergdreef 9, 1105 AZ, Amsterdam, the Netherlands
Department of Immunopathology, Central Laboratory of the Dutch Red Cross Blood Transfusion Service, 1066 CX, Amsterdam, the Netherlands
Department of Cardiovascular Pathology, Academic Medical Center, 1105 AZ, Amsterdam, the Netherlands

Kawasaki disease is a childhood vasculitis of medium-sized vessels, affecting the coronary arteries in particular. We have treated a therapy-resistant child who met all diagnostic criteria for Kawasaki disease. After the boy was given intravenous immunoglobulins and salicylates, as well as several courses of pulsed methylprednisolone, disease recurred and coronary artery lesions became progressively detectable. Cyclosporin A was started and seemed clinically effective. In contrast to the positive effect on inflammatory parameters, ie, C-reactive protein and white blood cell counts, a novel plasma marker for cytotoxicity (granzyme B) remained elevated. Coronary disease progressed to fatal obstruction and myocardial infarction. Echocardiography, electrocardiograms, and myocardial creatine phosphokinase did not predict impending death. At autopsy an obliterative panarteritis was observed resulting from massive fibrointimal proliferation, affecting the aorta and several large and medium-sized arteries. Immunophenotypic analysis of the inflammatory infiltrates in arteries revealed mainly granzyme-positive cytotoxic T cells and macrophages in the intima and media, as well as nodular aggregates of T cells, B cells, and plasma cells in the adventitia of affected arteries. These findings further endorse the role of specific cellular and humoral immunity in Kawasaki disease. Unremitting coronary arteritis and excessive smooth muscle hyperplasia resulted in coronary occlusion despite the use of cyclosporin A.

Key Words: Kawasaki disease • panarteritis • immunoglobulin • cyclosporin A • granzyme

Abbreviations: IVIG, intravenous immunoglobulin • CsA, cyclosporin A • CRP, C-reactive protein • WBC, white blood cell • LAD, left anterior descending • RCA, right coronary artery • ELISA, enzyme-linked immunosorbent assay • GrA, granzyme A • GrB, granzyme B • NK, natural killer • Ig, immunoglobulin • CTL cytotoxic T lymphocyte

Received for publication Feb 20, 2003; Accepted Jun 6, 2003.


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