Periodic fever syndromes, autoinflammatory diseases, Still's disease and MAS/HLH
Pediatric and adult rheumatologists may be asked to evaluate children with unexplained CNS dysfunction that may be driven by immune dysregulation or an autoinflammatory process. Neurologic dysfunction may be a hallmark of monogenic illnesses that rheumatologists may not be comfortable with due to their rarity (such as NOMID/CINCA, MWS, DADA2 and interferonopathies). Additionally, other catastrophic neurologic conditions such as febrile infection-related epilepsy syndrome (FIRES), acute necrotizing encephalitis and acute hemorrhagic leukoencephalitis may have an innate immune etiologies and may lead to rheumatology evaluation. Greater awareness of rheumatic disorders and novel inflammatory conditions may lead to greater collaboration with neurologists and rational utilization of immunomodulatory therapies.