What are the complications of systemic-onset juvenile idiopathic arthritis (JIA)?

Updated: Jul 25, 2019
  • Author: David D Sherry, MD; Chief Editor: Lawrence K Jung, MD  more...
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The complications that may occur in systemic-onset JIA are pericarditis, hemolytic anemia, macrophage activation syndrome (MAS), and endarteritis. Patients with pericarditis often present with orthopnea and respond to intravenous (IV) corticosteroid treatment.

MAS is a rare, but important, complication, in which all 3 bloodlines become rapidly decreased. Hypofibrinogenemia, thrombocytopenia, and elevated aspartate aminotransferase levels and markedly elevated ferritin levels are hallmarks. Hypotension, central nervous system (CNS) disease, and marked hepatosplenomegaly may be noted as complications of a release of massive amounts of cytokines.

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