Case Presentation: A 58 year-old woman with a past medical history of bipolar disorder, pulmonary embolism, opioid use disorder, and hypothyroidism presented to the hospital after being found unresponsive. Review of systems were only positive for confusion and bilateral lower extremity pain/swelling. Initial vital signs included a blood pressure of 79/48 mmHg, a heart rate of 114 beats per minute, and were otherwise within normal limits. On physical examination, the patient had 1+ lower extremity edema bilaterally and was alert and oriented to self and place, but appeared lethargic. Examination was otherwise unremarkable. Complete blood count, complete metabolic panel, and venous blood gas did not reveal any abnormal values. CT of the head showed no intracranial abnormality, but CT of the chest showed findings concerning for aspiration pneumonia, so empiric antibiotics were initiated. Blood pressure was initially responsive to intravenous fluid boluses, however hypotension persisted requiring administration of norepinephrine. Mental status continued to wax and wane and the patient became hypoxemic requiring 2-3L oxygen. Infectious workup showed blood cultures positive for S. pneumoniae and COVID-19 test was positive, so Remdesivir was started. Lumbar puncture was performed but cerebrospinal fluid analysis was not consistent with infection. Transthoracic echocardiogram did not reveal any vegetations. Despite appropriate antimicrobial therapy, the patient worsened clinically with fevers up to 39.4°C and leukocytosis of 31.4 K/mcL (from 6.8 on admission). Repeat imaging showed new findings of aortitis with a pericardial effusion, and inflammatory markers were elevated (ESR= 125mm/hr, CRP= 37mg/dL). The patient met CDC criteria for Multisystem Inflammatory Syndrome in Adults (MIS-A) with one primary criteria (aortitis), two secondary criteria (encephalopathy and shock), and elevated inflammatory markers with a positive COVID-19 test, so treatment with methylprednisolone and IVIG was initiated with subsequent clinical improvement including resolution of fevers, leukocytosis, and hypotension. Repeat imaging showed a marked reduction in aortic wall thickness and mental status returned to baseline.
Discussion: While COVID-19 related aortitis has been reported, it is a rare manifestation of the disease [1]. The proposed mechanism is virions infecting endothelial cells leading to neutrophilic and mononuclear infiltration with subsequent apoptosis [1]. Spontaneous resolution of COVID-19 aortitis has been reported and prognosis is usually excellent with steroid treatment [2, 3]. Another unique aspect of this case is the coexistence of MIS-A (which is an uncommon complication of COVID-19). While one study showed approximately 82% of MIS-A patients have cardiovascular involvement, pathology such as myocarditis remains more common [4]. Aortitis as a feature of MIS-A is very rare and has been reported in one case report [5]. While there are no consensus guidelines on treatment of MIS-A, the American College of Rheumatology suggests treatment with 1 dose of IVIG 2 g/kg body weight along with Methylprednisolone 1 mg/kg body weight followed by an oral glucocorticoid taper once the patient has improved clinically and is nearing discharge [6].
Conclusions: MIS-A remains an uncommon complication of COVID-19 with varied presentations including encephalopathy, shock, and rarely aortitis. This case highlights the importance of prompt diagnosis and treatment given the severity of the condition.