TY - JOUR
T1 - First report of a patient meeting criteria for both multisystem inflammatory syndrome in children and adult onset Still’s disease
AU - Alexandri, Maya
AU - Patel, Julisa
AU - Paul, Eli
AU - Coule, Lynne W.
N1 - Funding Information:
None.
Publisher Copyright:
© 2022, This is a U.S. Government work and not under copyright protection in the US; foreign copyright protection may apply.
PY - 2022/12
Y1 - 2022/12
N2 - Background: COVID-19 is associated with a postinfectious hyperinflammatory disorder, multisystem inflammatory syndrome in children (MIS-C), that shares characteristics with still’s disease, known as systemic juvenile idiopathic arthritis (SJIA) in children younger than 16, and adult onset Still’s disease (AOSD) in children 16 and older. Both MIS-C and SJIA/AOSD can be complicated by macrophage activation syndrome (MAS), a potentially fatal condition of cytokine storm. Case presentation: We present a 16 year-old male who developed quotidian fever, headache, conjunctival injection, sore throat, nausea and vomiting, diarrhea, rash, and symmetrical polyarticular arthralgia/arthritis 4 weeks after exposure to SARS-CoV-2 and 2 weeks after his first vaccination against COVID-19. Our patient’s laboratory results were significant for elevated inflammatory markers and acute phase reactants. He met criteria for diagnosis with both MIS-C and AOSD. After receiving first-line treatment for both diseases, IVIG and methylprednisolone, our patient improved. Conclusion: MAS is a life-threatening rheumatological emergency, and physicians must be able to identify diseases, like MIS-C and AOSD, that may be complicated by MAS. Our patient’s distinguishing feature on presentation was symmetrical polyarticular arthralgia/arthritis, which has not been associated with MIS-C. Simultaneously, AOSD—which is associated with polyarticular arthralgia/arthritis—is only now being recognized as a possible post-infectious entity in the aftermath of COVID-19 infection. In patients like our own, who meet criteria for both MIS-C and AOSD, administering first line treatment for both diseases may be best practice.
AB - Background: COVID-19 is associated with a postinfectious hyperinflammatory disorder, multisystem inflammatory syndrome in children (MIS-C), that shares characteristics with still’s disease, known as systemic juvenile idiopathic arthritis (SJIA) in children younger than 16, and adult onset Still’s disease (AOSD) in children 16 and older. Both MIS-C and SJIA/AOSD can be complicated by macrophage activation syndrome (MAS), a potentially fatal condition of cytokine storm. Case presentation: We present a 16 year-old male who developed quotidian fever, headache, conjunctival injection, sore throat, nausea and vomiting, diarrhea, rash, and symmetrical polyarticular arthralgia/arthritis 4 weeks after exposure to SARS-CoV-2 and 2 weeks after his first vaccination against COVID-19. Our patient’s laboratory results were significant for elevated inflammatory markers and acute phase reactants. He met criteria for diagnosis with both MIS-C and AOSD. After receiving first-line treatment for both diseases, IVIG and methylprednisolone, our patient improved. Conclusion: MAS is a life-threatening rheumatological emergency, and physicians must be able to identify diseases, like MIS-C and AOSD, that may be complicated by MAS. Our patient’s distinguishing feature on presentation was symmetrical polyarticular arthralgia/arthritis, which has not been associated with MIS-C. Simultaneously, AOSD—which is associated with polyarticular arthralgia/arthritis—is only now being recognized as a possible post-infectious entity in the aftermath of COVID-19 infection. In patients like our own, who meet criteria for both MIS-C and AOSD, administering first line treatment for both diseases may be best practice.
KW - Adult onset Still’s disease
KW - AOSD
KW - Case report
KW - COVID-19
KW - Macrophage activation syndrome
KW - MAS
KW - MIS-C
KW - Multisystem inflammatory syndrome in children
KW - Novel coronavirus disease 2019
KW - SJIA
KW - Still’s disease
KW - Systemic juvenile idiopathic arthritis
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U2 - 10.1186/s41927-022-00320-9
DO - 10.1186/s41927-022-00320-9
M3 - Article
AN - SCOPUS:85144992812
SN - 2520-1026
VL - 6
JO - BMC Rheumatology
JF - BMC Rheumatology
IS - 1
M1 - 87
ER -