Intravenous Immunoglobulin in Pediatric Rheumatology: When to Use It and What Is the Evidence.

Intravenous immunoglobulin (IVIG) is given to children with a variety of rheumatologic illnesses. The mechanism of action by which it exerts therapeutic effects is not well understood and likely differs in the medical conditions for which it is given. IVIG is approved by the US Food and Drug Administration and is the standard of care for Kawasaki disease, but most IVIG use in pediatric rheumatology is "off-label. " The literature supports the use of IVIG for juvenile dermatomyositis, although it is unclear whether its use should be limited to those children with more severe or refractory disease. It appears efficacious in the treatment of autoimmune thrombocytopenia secondary to lupus, but its use may be limited by transient responses. Treatment of other categories of pediatric rheumatologic diseases, such as juvenile idiopathic arthritis and non-Kawasaki vasculitides, is not well-established in the literature. This review focuses on current use of IVIG in the treatment of pediatric rheumatologic disorders. [Pediatr Ann. 2017;46(1):e19-e24.].