Rheumatologic disorders in the picu

Abstract

Rheumatologic diseases in children compromise a small but sometimes perplexing portion of patients admitted to the Pediatric Intensive Care Unit. Systemic Lupus Erythematosus (SLE) is the most variable and indiscriminate vasculitis affecting children. SLE may cause devastating injury to virtually any organ system and therefore must often be included in the differential diagnosis of critically ill children. Juvenile Idiopathic Arthritis (JIA) does not commonly require admission to the ICU in itself, but is associated with macrophage activation syndrome (MAS). MAS is a spectrum of hemophagocytic lymphohistiocytosis (HLH) which often calls for intensive care and aggressive therapies. Henoch-Schoenlein Purpura is a small vessel vasculitis affecting children commonly associated with intussusception, but may also give rise to significant cardioplulmonary complications. Kawasaki Disease is the most common vasculitis in children and the recently described Kawasaki Disease Shock Syndrome may require invasive monitoring and therapies. Antiphospholipid Antibody Syndrome, Goodpasture Disease, and Wegener’s Granulomatosis while not common, give rise to complications which also may necessitate critical care. The following chapter discusses these disease entities and other rheumatologic illness affecting children and common complications arising from them which may indicate critical care. Included in the discussion are the most widely accepted diagnostic approaches and therapies, and supporting evidence is described.