Table 2 Summary of clinical findings, demographics, and treatment strategies of pediatric-onset AAV after COVID-19 infection

Age, years

16

12

17

17

Sex

Female

Female

Male

Male

Comorbidities

Asthma

None

Asthma and Obesity

None

Chronology with COVID-19

1–2 weeks following infection

2 weeks following infection

2 months following infection

Concurrent

Positive serology

Anti-PR3 and C-ANCA

Anti-MPO and ANCA

Anti-MPO and P-ANCA

Anti-PR3 and C-ANCA

Lung involvement at presentation

Extensive multifocal pulmonary nodules and regions of consolidation with multiple areas of cavitation and central bronchiectasis with diffuse bronchial wall thickening

Dense consolidation in the left lower lobe and patchy infiltrate in the right middle and upper lobes without ground-glass opacities; diffuse alveolar hemorrhage

Extensive heterogeneous infiltrates in both lungs with an unusual fluffy central distribution concerning for diffuse alveolar hemorrhage

Multiple bilateral cavitary lung lesions

Kidney involvement at presentation

Normal kidney function (Cr. 0.79 mg/dL)

Pauci-immune necrotizing and crescentic glomerulonephritis

Renal biopsy showed necrotizing glomerulonephritis with limited immune complex deposition

Normal kidney function (Cr. 0.74 mg/dL)

AAV treatment

Prednisone, rituximab, mycophenolate mofetil

Methylprednisolone, rituximab, cyclophosphamide

Methylprednisolone, plasmapheresis, cyclophosphamide

Methylprednisolone, rituximab, not on maintenance therapy

Antibody titers at presentation

1:40 and a proteinase 3 antibody (PR3) level of 1.4 (normal < 1.0)

1:640 and a perinuclear pattern

Not available

1:640 and a proteinase 3 antibody (PR3) level of 251.9 (normal < 1.0)

Outcomes

Marked improvement of multifocal consolidation, nodularity, and cavitation on CT

Improvement in clinical symptoms

Resolution of DAH and AKI, not requiring outpatient dialysis

Clinically asymptomatic with marked improvement of cavitary lung nodules on CT