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Table 2 MIS-A published case reports

From: Multisystem inflammatory syndrome in adults: a case report and review of the literature

Authors

Age, sex, ethnicity

Past medical history

Signs and symptoms at presentation

Previous COVID-19 infection

Initial COVID-19 testing

ICU stay

Laboratory findings

Imaging studies

Treatments

Outcome

Kofman, 2020 [4]

25, female

None

Fever, dyspnea, sore throat, diarrhea, vomiting, cough, and adenopathy

No

PCR (+)

IgG (+)

Yes

Increased neutrophils, ESR, CRP, d-dimer, ferritin, Tn, and creatinine; lymphopenia

Chest X-ray and CT: No detected abnormalities

CT angiography: dilated main pulmonary artery

CT abdomen/pelvis: acute uncomplicated pancreatitis

Echo: dilated IVC then right ventricular dysfunction

Aspirin, IVIG

Recovery

Fox, 2020 [9]

31, female, African-American

HTN, DM, and obesity (BMI 36.1 kg/m2)

Fever, tachycardia, left-sided neck pain, nausea, vomiting, and parotitis by examination

Yes, 12 days prior

PCR (−)

NR

Elevated d-dimer, lactic acid, CRP, and creatinine

CT neck: bilateral parotid enlargement and swelling of the posterior nasopharynx to the oropharynx

CT chest: bilateral basal GGO plus anterior mediastinal lymphadenopathy

NR

Deceased

Shaigany, 2020 [8]

45, male, Hispanic

No PMH

BMI of 26.6 kg/m2

Fever, diarrhea, sore throat, painful lower extremities, diffuse exanthema, conjunctivitis, periorbital edema, left neck swelling with lymphadenopathy, plaques and papules diffuse, hypotension, tachycardia, and atrial fibrillation

No

PCR (+)

No

Increased neutrophils, low lymphopenia, ESR, CRP, d-dimer, ferritin, Tn, AST, ALT, PCT (3179 ng/mL), IL-6 (117 pg/mL)

Chest X-ray: diffuse interstitial haziness

CT neck with contrast: inflamed edematous lower eyelids and preseptal spaces, reactive lymphadenopathy

ECG: anterolateral ST segment elevation

PCI: normal coronary

TTE: global hypokinesia of the left ventricle with reduced EF of 40

Slit-lamp examination: conjunctivitis and uveitis

Full dose enoxaparin, IVIG (2 g/kg over 2 days), and single dose of IL-6 inhibitor (tocilizumab)

Recovery

Ahsan, 2020 [13]

28, male

Thalassemia minor. BMI of 28.48 kg/m2

High-grade fever (40.6 °C), anorexia, vomiting, nausea, lower limb pain, generalized weakness, red eye, difficult urination, and constipation. Bilateral facial nerve palsy, optic neuritis

Yes, 2 weeks before Ab (+), PCR (−)

Not done

NR

Anemia hypoalbuminemia leukocytosis with neutrophilia

Elevated ESR, ferritin, and CRP

ECG: normal

Chest X-ray: normal

MRI brain and orbit: normal

Ceftriaxone 2 g daily and prednisolone 1 mg/kg/day orally for 6 weeks

Recovery

Bettach, 2021 [14]

54, female

None

Fever, septic shock, GI symptoms, skin rash, heart failure, bilateral acute anterior uveitis

No

PCR (−)

IgG (+)

Yes

NR

Slit-lamp examination: bilateral corneal edema with Descemet’s membrane and keratin precipitates

Fundus examination: small localized intracranial bleed

Fluorescein angiography: no vascular abnormalities

Antibiotics, corticosteroids, and vasopressors. After 2 weeks, topical dexamethasone

Recovery

Razavi, 2020 [15]

23, male, African-American

BMI of 35.4 kg/m2

Fever, fatigue, myalgia, dyspnea, orthopnea, watery diarrhea, and temporal headache. Hypotension, bilateral scleral, and conjunctival injection

Yes, 1 month prior

PCR (−)

IgG (+)

NR

Leukocytosis, lymphocytopenia, high Tn I and BNP (NSTEMI)

High CRP, d-dimer, ferritin, and fibrinogen

Echo: global hypokinesia with reduced EF (40–45%)

Chest X-ray: no focal consolidations

CT chest with contrast: no abnormalities

Cardiac MRI: pericardial effusion and borderline EF (54%)

Antibiotics, IVIG, methylprednisolone, aspirin, enoxaparin

Recovery

Gulersen, 2021 [18]

31, female

Obesity, asthma, pregnant (28 weeks)

Fever, left-sided pleuritic chest pain, shortness of breath. Late-onset hypotension and tachypnea

Yes, 4 weeks prior. PCR (+)

PCR (−)

IgG (+)

Yes

Leukocytosis. Elevated CRP, normal lactate, ferritin, PCT, late-onset increased in cardiac enzymes and inflammatory markers

CT angiography of the chest: normal with no pulmonary embolism or lung pathology detected

TTE: On admission, EF 65–70% with a hyperdynamic left side, rim pericardial effusion, and well-functioning right ventricle. On day 4: global dysfunction of the right and left ventricles with rim pericardial effusion

Non-stress test: reactive fetus

Intravenous heparin, IVIG, dexamethasone (10 mg every 6 hours), mechanical ventilation, inotrope and vasopressor

Extubated on day 8, elective delivery, and discharged home on day 15

Malangu, 2020 [19]

46, male

History of pneumonia

Fever (39.1 °C), atrial fibrillation, mild hypoxia (SatO2 91% on room air), bilateral exudative conjunctival injection, oral mucositis, bilateral cervical lymphadenopathy, and macular skin rash

No

PCR (−)

IgG (+)

NR

Leukocytosis and thrombocytopenia. Elevated d-dimer, CRP, ferritin, LDH fibrinogen. Mildly elevated ALT, AST, kidney injury with hematuria, and proteinuria

CT angiography of the chest: bilateral apical patchy consolidations

Chest X-ray: basal and middle lobe opacities

TTE: left ventricular dysfunction with EF 31% and eccentric hypertrophy

Cardiac MRI: perihilar lymph nodes with no infiltrative lesions

Bronchoscopy: no malignant cells

Antibiotics and apixaban

Recovery

Othenin-Girard, 2020 [20]

22, male, East African

None

Five days of chills, myalgia, asthenia, diarrhea, and abdominal pain. Three weeks of loss of taste and smell sensations, and 1 day of dry cough, odynophagia, and rash (over trunk, extremities, palms)

Yes, 3 weeks prior. IgG (+)

PCR (+)

IgG (+)

Yes

Leukocytosis, elevated CRP (275 mg/L), fibrinogen (8.5 g/L), d-dimer (3322 ng/mL), and creatinine (1.5 mg/dL)

Autoimmune workup: negative ANA, ANCA, and rheumatoid factor

CT abdomen and chest: normal lung parenchyma with pulmonary embolism and inflamed mesenteric lymph nodes

TTE: biventricular dysfunction/endomyocardial biopsy: myocarditis with necrotic foci

Nerve conduction study: mononeuritis multiplex

IVIG, tocilizumab, rituximab, corticosteroids, and cyclophosphamide. Mechanical ventilation and extracorporeal membrane oxygenation (ECMO)

Recovery

Moghadam, 2020 [16]

21, male, Caucasian

None

Seven days of fever (40 °C), watery non-bloody diarrhea, chest tightness, vasoplegic shock, rash, tachypnea, bilateral conjunctivitis, and truncal and palmar rash

No

PCR (−)

IgG (+)

Yes

Leukocytosis, CRP (365 mg/L), PCT (3.4 ng/mL), ferritin (1.282 mg/L), high lactate, Tn (55n ng/L)

Skin biopsy: inflammatory infiltrates

TTE: hyperkinetic left ventricle with preserved EF

CT scan chest and abdomen: compatible with congestive heart failure

Fluid resuscitation, noradrenaline, antibiotics (amikacin and ceftriaxone)

Recovery

Lidder, 2020 [5]

45, male

None

Five days of fever, red eyes, diarrhea, sore throat, eyelids edematous rash, nonexudative conjunctivitis, and abnormal perioral mucosa

No

PCR (+)

NR

Lymphopenia, elevated CRP, ESR, ferritin, d-dimer, and elevated Tn

TTE: global hypokinesia with reduced EF (40%)

CT neck: unilateral lymphadenopathy

Eye-lubricating medications, topical prednisolone acetate 1%, IVIG, tocilizumab, and triamcinolone ointment for the rash

Recovery

Tung-Chen, 2021, Spain [6]

25, male

None

One-day history of nausea and abdominal pain. One week of fever (38 °C), sore throat, fatigue, anosmia, and orthopnea. Shock at presentation

No

PCR (−)

IgM (+)

IgG (+)

Yes

Lymphopenia (0.43 × 109/L), elevated fibrinogen (> 1200 mg/dL), CRP (337.1 mg/L), TnT I, and BNP

TTE: global hypokinesia with severely impaired left ventricular function (EF 29.7%) and rim pericardial effusion. EF improved after 8 days

CT chest: no abnormalities

Chest X-ray: no abnormalities

ECG: sinus tachycardia with no other abnormalities

Antibiotics, ganciclovir, norepinephrine, milrinone, and diuretics

Recovery

Uwaydah, 2021 [7]

22, male

None

Four days of fever (39 °C), sore throat, diarrhea, nausea, vomiting, myalgia, headache, fatigue, erythematous rash involving the torso, tachycardia, hypotension, edema, and proteinuria

Yes, 40 days prior PCR (+)

PCR (−)

IgG (+)

Yes

Leukocytosis, elevated creatinine, AST (53 U/L), ALT (81 U/L), direct bilirubin, CRP (249 mg/L), ferritin (4357 ng/mL), d-dimer (14 mg/mL), PCT (9 ng/mL), IL-6 (90 pg/mL), low platelets (122) and albumin (16 g/L)

TTE: severe tricuspid regurgitation, pulmonary HTN (46 mmHg), left ventricle dysfunction (EF 45%), and rim pericardial effusion. Normal echo after recovery

CT chest: bilateral moderate pleural effusion and basilar atelectasis

Antibiotics, intravenous hydrocortisone

Recovery

Ahmad, 2021 [21]

26, male, Caucasian

None

Fever, abdominal pain, loose stool, nausea, reduced urine output, hypotension tachypnea (38 breath/minute) and hand/feet rash

PCR (+)

PCR (+)

Abs (+)

Yes

Leukocytosis. Elevated lactic acid (9.7 mg/dL), CRP (246 mg/L), PCT (105.12 ng/mL), d-dimer (2.03), LDH (236 U/L), creatinine (4.66 mg/dL), and urea (38 mg/dL)

Lower limb doppler: left peroneal DVT

Chest X-ray: peribronchial thickening

Noncontrast CT abdomen: perinephric edema and mesenteric lymphadenopathy

TTE: severely impaired left ventricular function (EF 15–20%) as well as right ventricular dysfunction. EF increased to 60% after 10 days

Vasopressors, IVIG, methylprednisolone (250 mg/6 hours), aspirin, anakinra (IL-1 receptor antagonist), mechanical ventilation, and CRRT

Recovery

Li, 2021 [10]

28, male

None

Five days of right-sided neck pain and swelling, enlarged tonsils, tenderness of the right submandibular fever, malaise, tachycardia, pruritic rash

4 weeks prior, PCR (+)

PCR (−)

IgG (+)

NR

Leukocytosis (13,800/mm3), anemia (10.7 g/dL). Elevated hs-Tn I (11,908 ng/L), BNP (1661 pg/mL), CRP (304.2 mg/L), and ferritin (1588 mg/L)

CT neck: cervical lymphadenopathy, more on the right side

TTE: mildly impaired left ventricular function (EF 45–55%)

Cardiac MRI: rim pericardial effusion and slightly impaired right ventricular function

Broad-spectrum antibiotics, fluid resuscitation, beta-blocker, ACE inhibitor

Recovery

Veyseh, 2021 [23]

43, female

None

Fever, hypotension, tachycardia, erythematous rash, diarrhea, and cramping abdominal pain

No

PCR (−)

Yes

High WBCs, CRP, ferritin, d-dimer, fibrinogen, LDH, AST, and ALT

TTE: reduced EF (toxic cardiomyopathy), EF improved after IVIG and steroids

Antibiotics, vasopressors, IVIG, and intravenous solumedrol

Recovery

Diakite, 2021, [17]

33, male

HTN

Fever, diarrhea, chest pain, dyspnea, conjunctivitis, and cheilitis. Hypotension, tachycardia, and elevated hepatojugular reflux

Possible 6 weeks prior

PCR (−)

IgG (+)

NR

Leukocytosis (21,000/mm3), anemia (10.7 g/dL), high AST, ALT, creatinine, CRP, d-dimer, BNP, and Tn

TTE: global hypokinesia, reduced EF (20%), and dilated IVC. Cardiac MRI revealed improved cardiac function after a week of treatment

Coronary CT: aneurysms involving the right coronary, interventricular artery, and the left circumflex

Dobutamine, norepinephrine, IVIG, aspirin, prednisolone

Recovery

Bastug, 2021, Turkey [24]

40, male, Caucasian

None

Fever (39 °C), tachycardia, tachypnea, abdominal pain, diarrhea, and skin rash

23 days prior

PCR (−)

IgM (+)

IgG (+)

NR

Lymphopenia, leukocytosis as well as high liver function tests, ferritin, d-dimer, troponin, BNP, CRP, fibrinogen, PCL, and IL-6

CT abdomen: inflamed intestine and mesentery, mesenteric lymphadenopathy, and effusion

TTE: global hypokinesia, reduced left ventricle function (EF 45%), and mild pericardial effusion. EF increased to 60% and the effusion resolved after treatment

Antibiotics, methylprednisolone, IVIG, full-dose enoxaparin

Recovery

Sokolovsky, 2021, [31]

36, female, Hispanic

None

Fever, vomiting, abdominal pain, diarrhea, arthralgia, rash hypotension, and tachycardia

No

PCR (+)

Abs(+)

NR

Elevated liver enzymes, direct bilirubin, albumin, CRP, ferritin, d-dimer, ESR, and hyponatremia (115 mmol/L)

TTE: normal EF (65%) and moderate tricuspid regurgitation

CTA coronaries: normal with rim pericardial effusion

CT chest: trace pleural effusion

Steroids, acetylcysteine, IVIG, aspirin

Recovery

Julius, 2021, [11]

59, female, Caucasian

HTN and dyslipidemia

Fever, right cervical lymph node swelling, odynophagia, hypotension, and rash (neck and chest)

20 days prior, PCR (+)

PCR (+)

Yes

Slightly elevated AST, ALT; high Tn, CRP, and ferritin

CT neck: enlarged right nodes with one exhibiting liquefaction

EKG: ST elevation in V1 and V2

Antibiotics, steroids, norepinephrine, epinephrine, terlipressin mechanical ventilation

Deceased

Parpas, 2021 [32]

67, male

HTN, cirrhosis

Dyspnea weakness, weight loss, anorexia, nausea, extremities edema, tachycardia, and cognitive impairment

68 days prior

PCR (−)

Abs (+)

NR

Low sodium (109 mEq/L) and albumin (3 g/dL), leukocytosis (35,000/mm3). High d-dimer, LDH, and PCL

Chest X-ray: bilateral basal infiltrative lesions

CT chest: lung atelectasis/collapse

TTE: Pulmonary HTN, and grade I diastolic dysfunction

Duplex of lower limbs: no DVT

Renal biopsy: moderate to severe acute tubular necrosis

Antibiotics, unfractionated heparin, dexamethasone, and hemodialysis

Recovery

Pérez, 2021, [25]

88, male

HTN, dyslipidemia, essential tremors

Hypoxia (saturation 87%), dyspnea, and peripheral edema

54 days prior

PCR (+)

Abs (+)

PCR (−)

IgM (+)

IgG (+)

NR

Creatinine (2.14 mg/dL), proteinuria (> 600 mg/dL), and low albumin 3 g/dL

High LDL, CRP, and d-dimer

Chest X-ray: typical COVID-19 picture and pleural effusion

Renal biopsy: findings suggesting acute IgA-dominant infection-associated glomerulonephritis

Intravenous furosemide, intravenous methylprednisolone

Recovery

Balan, 2021, [33]

46, male

Obesity (BMI 42 kg/m2)

Hypotension, hypoxia tachypnea, right hemiparesis, ataxia, and left hemianesthesia

60 days prior

PCR (−)

Abs (+)

Yes

Elevated ferritin, CRP, LDH, PCT, high creatinine (4.1 mg/dL) and Tn

TTE: normal EF and elevated right ventricular pressures

CT chest: bilateral apical and basal as well as right middle ground-glass opacities

Norepinephrine, antibiotics unfractionated heparin, dexamethasone, tocilizumab, hemodialysis

Deceased

Mieczkowska, 2021, [22]

32, male

None

Fever, tachycardia, right-sided swollen groin lymph nodes, diarrhea, and palms and soles rash

Two months prior

PCR (−)

IgG (+)

No

Elevated AST, ALT, and direct bilirubin. Elevated inflammatory markers (CRP, ferritin, PCL, IL-6, ESR, and d-dimer)

TTE: EF 55% and pericardial effusion

CT: lymphadenopathy of the right groin

Enoxaparin and intravenous methylprednisolone

Recovery

Mieczkowska, 2021, [22]

43, female

None

Fever, myalgia, headache, cough, and skin rash. Hypotension, cardiomyopathy, and acute kidney injury

No

PCR (−)

Serology (+)

NR

Leukocytosis (21,500/mm3).

Elevated ESR, CRP, ferritin, and d-dimer. Elevated AST, ALT, and ALP

Chest X-ray: right basal pneumonia

Abdominal ultrasound: pericholecystic fluid, hepatomegaly, and steatosis

TTE: EF 40%

Vasopressors, antibiotics, intravenous heparin, methylprednisolone

Recovery

Hékimian, 2021 [12]

40, male

DM (BMI 26 kg/m2)

Apyretic, dyspnea, severe asthenia

No

PCR (+)

IgG (−)

Yes

Elevated PCT, CRP, ferritin

Elevated AST, ALT, and ALP

Elevated LDH, CPK

Peak troponin 439 ng/L

Peak BNP 6025 pg/mL

Chest CT: severe multifocal PNA

TTE: EF 45%

Mechanical ventilation, dobutamine, norepinephrine, ECMO

Recovery

Hékimian, 2021 [12]

19, female

None (BMI 24 kg/m2)

Fever, dyspnea, cough

No

PCR (−)

IgG (+)

Yes

Elevated CRP, ferritin, LDH

Peak troponin 10,652 ng/L

Peak BNP 2585 pg/mL

Chest CT: mild infiltrates

TTE: EF 30%

Mechanical ventilation, dobutamine, norepinephrine, ECMO

Recovery

Hékimian, 2021 [12]

22, male

DM, asthma (BMI 38 kg/m2)

Fever, dyspnea, cough, severe asthenia

No

PCR (−)

IgG (−)

Yes

Elevated CRP, ferritin, LDH

Peak troponin 166 ng/L

Chest CT: severe infiltrates

TTE: EF 30%

Mechanical ventilation, ECMO

Recovery

Hékimian, 2021 [12]

19, male

None (BMI 22 kg/m2)

Fever, headache, diarrhea, dyspnea, severe asthenia

No

PCR (−)

IgG (+)

Yes

Elevated CRP, ferritin, LDH

Peak troponin 806 ng/L

Peak BNP 26,956 pg/mL

Chest CT: negative

TTE: EF 15%

Dobutamine, norepinephrine

Recovery

Hékimian, 2021 [12]

16, male

None (BMI 18 kg/m2)

Fever, anosmia, abdominal pain, rash to hands and feet, conjunctivitis, strawberry tongue, adenopathy, severe asthenia, chest pain

No

PCR (+)

IgG (+)

Yes

Elevated CRP, ferritin, LDH

Peak Troponin 2545n ng/L

Chest CT: mild infiltrates

TTE: EF 20%

Mechanical ventilation, dobutamine, norepinephrine, IVIG

Recovery

Hékimian, 2021 [12]

16, female

None (BMI 24 kg/m2)

Fever, headache, abdominal pain, rash to hands and feet, dyspnea, severe asthenia

Yes, anosmia and cough 1 month prior

PCR (−)

IgG (+)

Yes

Elevate CRP, ferritin, and LDH

Peak troponin 64 ng/L

Peak BNP 1689 pg/mL

Chest CT: negative

TTE: EF 45%

None

Recovery

Hékimian, 2021 [12]

17, male

Moderate aortic regurgitation (BMI 32 kg/m2)

Fever, headache, abdominal pain, diarrhea, dyspnea, severe asthenia, conjunctivitis

No

PCR (+)

IgG (+)

Yes

Elevated ferritin and LDH

Peak troponin 138 ng/L

Peak BNP 35,000 pg/mL

Chest CT: mild pulmonary edema

TTE: EF 20%

Mechanical ventilation, dobutamine, norepinephrine, IVIG, corticosteroids 2 mg/kg/day

Recovery

Hékimian, 2021 [12]

25, female

None (BMI 23 kg/m2)

Fever, headache, abdominal pain, dyspnea, severe asthenia, myalgias, arthralgias, adenopathy

No

PCR (−)

IgG (+)

Yes

Elevated CRP, ferritin, LDH

Peak troponin 2542 ng/L

Peak BNP 24,540 pg/mL

Chest CT: negative

TTE: EF 50%

Nasal cannula

Recovery

Hékimian, 2021 [12]

17, female

None (BMI 18 kg/m2)

Chest pain, dyspnea

No

PCR (+)

IgG (+)

Yes

Elevated CRP, ferritin, LDH

Peak troponin 4905 ng/L

Peak BNP 3362 pg/mL

Chest CT: pulmonary edema

TTE: 20%

Mechanical ventilation, dobutamine, norepinephrine, ECMO, IVIG, corticosteroids 2 mg/kg/day

Deceased

Hékimian, 2021 [12]

37, male

HTN (BMI 35 kg/m2)

Fever, headache, diarrhea, severe asthenia

No

PCR (−)

IgG (+)

Yes

Elevated ferritin, LDH

Peak troponin 1164 ng/L

Peak BNP 35,000 pg/mL

Chest CT: Negative

TTE: EF 45%

IVIG, corticosteroids 2 mg/kg/day

Recovery

Hékimian, 2021 [12]

29, female

None (BMI 22 kg/m2)

Fever, abdominal pain, diarrhea, rash, conjunctivitis, severe asthenia

Yes, 1 month earlier

PCR (−)

IgG (+)

Yes

Elevated CRP, ferritin, LDH

Peak troponin 200 ng/L

Peak BNP 21,298 pg/mL

Chest CT: negative

TEE: EF 50%

IVIG

Recovery

  1. PMH past medical history, HTN hypertension, BMI body mass index, BPM beats per minute, MIS-A multisystem inflammatory syndrome in adults, PCT procalcitonin, AST aspartate transaminase, ALT alanine transaminase, ALP alkaline phosphatase, CRP C-reactive protein, ESR erythrocyte sedimentation rate, LDH lactate dehydrogenase, EKG electrocardiogram, CAP community-acquired pneumonia, PNA pneumonia, HD hospital day, ANA antinuclear antibodies, ANCA antineutrophil cytoplasmic antibodies, OD once daily, Tn troponin, BNP brain natriuretic peptide, DVT deep vein thrombosis, TTE transthoracic echocardiogram, EF ejection fraction, MRI magnetic resonance imaging, MV mechanical ventilation, CRRT continuous renal replacement therapy, IVIG intravenous immunoglobulins, LMWH low molecular weight heparin, Abs antibodies, SatO2 saturation of O2