Covid-19 Vaccination and Covid 19 Infection in Atypical Hemolytic Uremic Syndrome: One Single Center Study in Taiwan

ABSTRACT

Introduction: Acute kidney injury, microangiopathic hemolytic anemia and thrombocytopenia with multiple organ thrombotic microangiopathy (TMA) are typical characteristic presentation of Atypical hemolytic uremic syndrome(aHUS). Infection, pregnancy, operation, and some medication can be a trigger factor to induce the complement system over activation and induce atypical hemolytic uremic syndrome unstable to a life-threatening condition. Both SARS-CoV-2(Severe Acute Respiratory Syndrome Coronavirus 2) infection and COVID 19 vaccination are reported to be the trigger factors for aHUS. There are no clinical trial enrolled aHUS cases to COVID 19 vaccine or anti SARS-CoV2 agent. Therefore, aHUS became a tough medical issue in this pandemic status. In this study, we evaluate the efficacy and disease activity of aHUS after COVID 19 vaccination. Meanwhile, we analysis the severity of COVID 19 infection in our 21 aHUS cases. Method(s) There are 21 aHUS cases enrolled this study from April 2022 to September 2022. Each cases with regular blood sampling which include hemolysis markers (Hemoglobin, Platelet count, LDH, CH50, haptoglobin, Blood smear), renal function and urine analysis every months. While them had COVID 19 vaccination or COVID 19 infection, the above blood sampling and urine analysis should be followed up two weeks later. Once the aHUS cases became severe condition and need hospitalization, our medical team must visit these cases closely and monitor if any new critical issue happen. We confirmed the serum SARS-CoV-2 Spike IgG and Interferon-gamma (IFNgamma) release assay testing for the vaccination efficacy analysis. Result(s) 21 aHUS cases all had COVID 19 vaccination, 2 cases received 1 dose vaccine, 6 cases received 2 doses vaccine and 13 cases received 3 doses vaccine. Only one case with aHUS unstable after Moderna vaccine injection which is self-limited gradually and didn't need extra dose of anti-complement therapy. Interestingly, this case with stable aHUS disease activity while he switches to Pfizer-BioNTech vaccine as his 2nd dose. The SARS CoV-2 Spike IgG level and IFNgamma level are corelated to the dosage of COVID 19 vaccination, the higher doses with the higher level. The SARS-CoV2 spike IgG and IFNgamma level without lower response to the group with regular anti-C5 treatment. For those complete three dose vaccination cases, mix type of COVID-19 vaccination (AZ/mRNA) with better efficacy trend to fix type of mRNA. During this study period, there are 4 cases with COVID 19 infection. One case (already had 2 doses COVID 19 vaccination) needed hospitalization and improved after remdesivir and dexamethasone treatment who with mild aHUS disease activity progression. Two cases (complete three doses COVID 19 vaccination) with stable aHUS disease activity after Molnupiravir treatment. One case (complete three doses COVID 19 vaccination) refused Molnupiravir treatment and had mild aHUS disease activity progression. Conclusion(s) According to our study, we recommend the aHUS patient to have COVID 19 vaccination and multiple doses are more protective for them. aHUS disease activity should be close monitor especially after COVID 19 vaccination, during COVID 19 infection and after COVID 19 infection. Remdesivir and Molmupiravir are relative safe to use for aHUS cases. No conflict of interestCopyright © 2023