LAUSR

Abstract Whilst they are rare paediatric rheumatological emergencies can be life-threatening. They may occur in a patient with or without a known underlying rheumatological diagnosis. The signs and symptoms of a rheumatological emergency may be subtle and non-specific and can easily be missed, especially in a patient without a known underlying connective tissue disease or vasculitis. Progression can be rapid and without prompt recognition and treatment such emergencies can be catastrophic. This paper discusses the presenting features and the approach to management of several rheumatological emergencies including Macrophage Activation Syndrome (MAS), Pulmonary-Renal Syndrome, neonatal heart block, central nervous system (CNS) vasculitis and Catastrophic Antiphospholipid Syndrome (CAPS). Awareness of these conditions facilitates prompt recognition, guides appropriate investigations and ensures timely initiation of treatment. This is essential to help prevent avoidable morbidity and mortality.