Multisystem Inflammatory Syndrome in an Adult (MIS-A) Successfully Treated with Anakinra and Glucocorticoids

Abstract

During the current SARS-CoV-2 pandemic, a novel syndrome termed “multisystem inflammatory syndrome in children” (MIS-C) has emerged. MIS-C was linked to COVID-19 and shared some features with Kawasaki disease and Toxic Shock Syndrome, with a common pathogenetic substrate of hyperinflammation and cytokine storm. Lately, MIS was also described in adults (≥21 years of age) and named “MIS-A”. There is no consensus about the treatment of MIS-A; successful use of glucocorticoids and immunoglobulins has been reported in case series, but more solid evidence is lacking. Furthermore, the role of biologic agents with proven benefits against COVID-19, MIS-C, or Kawasaki disease is still unexplored. In this report, we detail the clinical picture and the diagnostic process that led to the diagnosis of MIS-A in a 27-year-old man, focusing on its treatment with anakinra and glucocorticoids, which resulted in full recovery. To our knowledge, this is the first report of the successful use of anakinra for MIS-A, a drug that has already proven useful in the treatment of refractive cases of MIS-C. Anakinra may also play a pivotal role for the treatment of MIS-A.

Cardiac involvement in Adult Multisystemic Inflammatory Syndrome related to COVID-19. Two case reports

“…The response to corticosteroids is good in almost all cases described, and intravenous boluses can be used. Other treatments have been tried in patients who do not respond to corticosteroids, including immunoglobulins, anakinra, and tocilizumab [9] .…”

Multisystem Inflammatory Syndrome Associated with SARS-CoV-2 Infection in an Adult: A Case Report from the Maldives

“…The time to develop MIS-C after exposure to SARS-CoV-2 is reported to be within five weeks after infection [ 14 ]. The existing data regarding MIS-A suggest a similar convalescent response in adults [ 15 , 16 , 17 , 18 ]. Nevertheless, reports of a multisystem inflammatory syndrome after vaccination (MIS-V) in individuals who had received immunization after recovering from mild COVID-19, or perhaps non-neutralizing antibodies from other coronaviruses, suggest an alternative pathogenesis involving antibody enhancement that leads to the hyper-inflammatory syndrome [ 19 , 20 , 21 ].…”

Anakinra treatment in multisystemic inflammatory syndrome in children (MIS-C) associated with COVID-19

“…They prescribed anakinra to 23 patients with MIS-C and showed a significant resolution in laboratory parameters ( 16 ). Furthermore, successful results with anakinra in patients with the multisystem inflammatory syndrome in adults were published ( 17 ).…”

“…The median resolution day of the fever was 3 (1-10) days after anakinra treatment. The lymphocyte counts, BNP, CRP, and D-dimer values of the patients became normal on the median day of 5 (1-18), 8.5 (5-53), 10 (1-33), and 11.5 (4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20), respectively. The pattern of improvement in EF…”