BCL2L11 Is Associated With Kawasaki Disease in Intravenous Immunoglobulin Responder Patients.

Abstract

Kawasaki disease (KD) is a self-limited systemic vasculitis of an unknown pathogenesis mainly affecting children <5 years old. KD is a clinically heterogeneous disease; its diagnosis is based on common clinical symptoms, and there is no specific diagnostic test for it. Complete KD is diagnosed when subjects have at least 5 of the following 6 principal clinical signs: fever persisting for ≥5 days, bilateral conjunctival congestion, changes to the lips and oral cavity, polymorphous exanthema, changes to peripheral extremities, and acute nonpurulent cervical lymphadenopathy. The standard treatment for KD is high-dose intravenous immunoglobulin (IVIG), manufactured from normal human immunoglobulin purified from the full plasma of a thousand healthy donors; it reduces the duration of fever and the incidence of coronary artery abnormalities. The anti-inflammatory effects of IVIG are manifested in a wide range of pathological conditions, including immune thrombocytopenia, systemic lupus erythrematosus, Guillain–Barre syndrome, and others. Despite IVIG therapy, however, ≈15% of patients with KD have persistent or recurrent fever with a high risk for coronary artery lesions. In our KD patients data, we also observed significant differences in clinical variables between …