Progress in pediatric vasculitis.
OBJECTIVE
To examine recent advances in the pathophysiology and therapy of pediatric vasculitis.


RESULTS
The past 2 years have been marked by significant progress in extending novel techniques to the investigation of the two most common pediatric vasculitis syndromes, Henoch-Schonlein purpura and Kawasaki disease. Study of other vasculitides, such as Wegener granulomatosis, Churg-Strauss syndrome, and microscopic polyangiitis, is impeded by the small number of pediatric patients. Nonetheless, national and international registries are beginning to provide the foundation for generation of testable hypotheses regarding pathogenesis and optimal treatment. Thus, recent data from the study of children suggest that disorders in the control of inflammation, such as those that underlie familial Mediterranean fever and other autoinflammatory diseases, may predispose to vasculitis. Improved knowledge of mechanisms of disease, in turn, should pave the way for more targeted, effective, and tolerable therapies for children with systemic vasculitis.


CONCLUSIONS
International collaboration to study rare disorders such as pediatric vasculitis are demonstrating disorders of inflammatory regulation that predispose to these diseases and may point toward new treatment approaches.
O'Neil
Kathleen M
KM
Division of Pediatric Rheumatology, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma 73104, USA. kathleen-oneil@ouhsc.edu
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use; D000925:Anticoagulants / Q000627:therapeutic use; D002648:Child; D015267:Churg-Strauss Syndrome / Q000209:etiology / Q000628:therapy; D014890:Granulomatosis with Polyangiitis / Q000209:etiology / Q000628:therapy; D006801:Humans; D011695:IgA Vasculitis / Q000209:etiology / Q000628:therapy; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D009080:Mucocutaneous Lymph Node Syndrome / Q000209:etiology / Q000628:therapy; D013625:Takayasu Arteritis / Q000209:etiology / Q000628:therapy; D014657:Vasculitis / Q000209:etiology* / Q000628:therapy*; D020293:Vasculitis, Central Nervous System / Q000209:etiology / Q000628:therapy
D000305:Adrenal Cortex Hormones; D000806:Angiotensin-Converting Enzyme Inhibitors; D000925:Anticoagulants; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents