key: cord-0702881-5iw1pr6y
authors: Chalasani, Varun; Parameswaran, Padmini; Cherico, Adriani; Villgran, Vipin; Lowther, Holly; Marco, Joanna
title: SARS-CoV-2 multisystem inflammatory syndrome in an adult presenting with polyarthritis treated with anakinra
date: 2021-09-11
journal: Rheumatology (Oxford)
DOI: 10.1093/rheumatology/keab697
sha: a995c6b3061f5127543fecdcf1426f38999930ee
doc_id: 702881
cord_uid: 5iw1pr6y

nan

broadened. Transthoracic echocardiogram revealed biventricular global systolic dysfunction. Norepinephrine, dopamine and vasopressin were started. He became anuric and continuous renal replacement therapy was started. He developed progressive laboratory abnormalities, with his ferritin level peaking at 25 622 ng/ml (reference range 30-400), leucocyte count at 55 000 K/ll (reference range 4.4-11.3), procalcitonin at 272 ng/ml, ESR >130 mm/h (reference range 0-25) and CRP at 32.8 mg/dl (reference range <0.5).

Extensive evaluation for bacterial, viral or fungal infection was negative. ANA and subserologies, RF, myositis panel and ANCA panel were negative. Flow cytometry for lymphoma/leukaemia, T and NK cell levels, and genetic screening for clonality were within normal limits. IL-2R alpha levels were elevated at 8595 U/ml (reference range 241-846).

COVID-19 MIS-A was considered, and on day 5, treatment was started with methylprednisolone 1 g i.v. daily for five days, and anakinra 100 mg every 48 h (dose adjusted for renal insufficiency). The patient had rapid clinical improvement on this combination therapy. In the following week, the patient was titrated off vasopressor support, extubated and transitioned to intermittent haemodialysis. Within 1 month, he made a full renal recovery. A repeat transthoracic echocardiogram also showed normal biventricular functioning. Laboratory abnormalities began to downtrend and eventually normalized. Anakinra and prednisone were gradually tapered. At last follow-up, he had ongoing memory impairment but no other persistent symptoms or organ dysfunction. Table 1 [4, 5] compares the Yamaguchi criteria for AOSD and the Centers for Disease Control criteria for COVID-19 MIS-A. There was a question of AOSD with MAS given the initial presentation of fever and inflammatory arthritis, with subsequent hyperferritinemia and organ dysfunction. However, the patient lacked the classic rash, pharyngitis and lymphadenopathy of AOSD, so in the setting of recent COVID-19 infection and new multiorgan failure, we favoured MIS-A. Additionally, cardiac dysfunction without pericarditis or myocarditis is rare in AOSD/MAS but exceedingly common in COVID-19 MIS-A. Although AOSD/MAS and COVID-19 MIS-A are both marked by hyperinflammation and multiorgan dysfunction, other clinical features and a history of recent COVID-19 infection may be essential to differentiate between the diagnoses.

The unusual presenting symptom of inflammatory arthritis has not yet been described in the literature for COVID-19 MIS-A. Additionally, although most cases of MIS-A in the literature were treated with IVIG, glucocorticoids and tocilizumab, this case is unique for the successful use of anakinra for COVID-19 MIS-A. Rheumatologists should be aware of this condition, which can mimic other severe systemic diseases Funding: No specific funding was received from any bodies in the public, commercial or not-for-profit sectors to carry out the work described in this article.

Disclosure statement: The authors have declared no conflicts of interest.

Data derived from public domain resources, with information to access these resources provided in the references section. 

Case series of multisystem inflammatory syndrome in adults associated with SARSCoV-2 infection -United Kingdom and United States

Multisystem inflammatory syndrome in children and COVID-19 are distinct presentations of SARS-CoV-2