COVID-19 associated pediatric vasculitis: A systematic review and detailed analysis of the pathogenesis

Semin Arthritis Rheum. 2022 Aug:55:152047. doi: 10.1016/j.semarthrit.2022.152047. Epub 2022 Jun 9.


Objectives: Coronavirus disease 2019 (COVID-19) caused by SARS-CoV-2, has opened a new era in the practice of pediatric rheumatology since it has been associated with inflammatory complications such as vasculitis and arthritis. In this review, we aimed to present a detailed analysis of COVID-19 associated pediatric vasculitis.

Methods: A systematic review of the English literature was performed through Pubmed/MEDLINE and Scopus up to January 1st, 2022. Articles including data about the patients with 1) onset of vasculitis <18 years of age, 2) evidence of SARS-CoV-2 exposure, 3) evidence of vasculitis diagnosis (imaging, histopathologic evidences or fulfilling the specific diagnostic/classification criteria) were included in the final analysis. Patients with Kawasaki disease-like vasculitis associated with multisystem inflammatory syndrome in children (MIS-C) were excluded.

Results: A total of 25 articles describing 36 patients with COVID-19 associated pediatric vasculitis (median age 13 years; M/F: 2.3) were included. The most frequent phenotype was IgA vasculitis (n=9) followed by chilblains (n=7) and ANCA associated vasculitis (AAV) (n=5). Skin (58.3%) and renal (30.5%) involvements were the most common manifestations of vasculitis. The majority of patients received corticosteroids (40%), while rituximab (14.2%) and cyclophosphamide (11.4%) were the most frequently used immunosuppressive drugs. Remission was achieved in 23 of 28 patients. Five patients (4 with central nervous system vasculitis; 1 with AAV) died.

Conclusion: Although COVID-19 associated pediatric vasculitis is very rare, awareness of this rare entity is important to secure earlier diagnosis and treatment. The clinical features of COVID-19 associated pediatric vasculitis subtypes look similar to those in pediatric vasculitis not associated with COVID-19. Whether COVID-19 is the reason of the vasculitis or only the trigger remains unknown.

Keywords: COVID-19; Pathogenesis; Pediatric vasculitis; SARS-CoV-2.

Publication types

  • Review
  • Systematic Review

MeSH terms

  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis*
  • COVID-19* / complications
  • Child
  • Humans
  • Rituximab
  • SARS-CoV-2
  • Systemic Inflammatory Response Syndrome


  • Rituximab

Supplementary concepts

  • pediatric multisystem inflammatory disease, COVID-19 related