MO218: Anca-Associated Vasculitis After SARS-COV-2 Vaccination in a Patient With HLA-DRB1*09: 01 ALLELE: A Case Report and Literature Review
Authors: Kawamura Takuro , Nakazawa Daigo , Komatsumoto Maki , Saori Nishio , Atsumi Tatsuya 
Published: May 2022

Abstract

BACKGROUND AND AIMS
A Coronavirus disease 2019 (COVID-19), caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), presents with severe pneumonia and fatal systemic complications. Currently, SARS-CoV-2 vaccines are effective in reducing the risk of onset and severity of the disease. However, autoimmune diseases, including anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), have been reported as rare complications of the COVID-19 vaccine. Although the mechanism of ANCA vasculitis remains unknown, the genetic background, environmental factors and infections are involved in the development of the disease. Genome-wide association studies have identified several AAV-related haplotypes, including the human leukocyte antigen (HLA)-DRB1*09: 01 allele. Here, we report a case of AAV with a risk HLA allele after SARS-CoV-2 vaccination (Pfizer-BioNTech) and a literature review.

METHOD
Case report: A 71-year-old woman visited a clinic complaining of fever (37.0–37.5ºC) and malaise, 1 week after receiving second dose of COVID-19 vaccine (Pfizer-BioNTech). Two months after her first dose, her serum creatinine (Cr) level had increased from 0.86 mg/dL to 1.2 mg/dL with high titer of MPO-ANCA (280 IU/mL, normal value &lt;3.5 IU/mL). Urinary microscopy revealed a red blood cell count of 30–49/high power field and a urinary protein–creatinine ratio of 1.06 g/gCr. We diagnosed MPO-AAV with manifestations of renal involvement, general symptoms and the presence of ANCA, as a cause of renal progressive glomerulonephritis. A course of corticosteroids and intravenous cyclophosphamide was initiated. After treatment, her general symptoms and urinary abnormalities disappeared, and renal insufficiency was improved as well. Three months later, the MPO-ANCA titer decreased to 27.4 IU/mL. We examined a human leukocyte antigen (HLA) haplotype and her allele was HLA-DRB1*09:01, which is a known risk allele of MPO-ANCA-associated vasculitis.

RESULTS
Review: Until November 30, 2021, we searched PubMed, including the case report study and seven cases have been reported as de novo AAV after SARS-CoV-2 vaccination. The mean age of patients was 72.5 years (three women and four men). The onset of symptomatic symptoms, such as fever, headache and malaise, ranged from the day after the first dose to 2 weeks after the second dose; consequently, renal dysfunction was detected. In six patients (except for our case), histological findings showed pauci-immune crescentic glomerulonephritis. Most patients received initial induction immunosuppressive therapy, including corticosteroids and cyclophosphamide, followed by maintenance therapy. The renal involvement of six patients improved, but one patient with severe renal dysfunction developed end-stage renal disease. Information on HLA allele was not available in any case.

CONCLUSION
This is the first case report of de novo AAV after SARS-CoV-2 vaccination in a patient with AAV susceptible HLA-DRB1*09:01 allele.