ABSTRACT
Hemolytic uremic syndrome (HUS) is classically described as a triad of nonimmune hemolytic anemia, thrombocytopenia, and acute kidney injury. Atypical HUS (aHUS) is a rare variant of the disease, and it accounts for 5-10% of the cases. It has a poor prognosis, with a mortality rate exceeding 25% and a more than 50% chance of progressing into end-stage kidney disease (ESKD). Genetic or acquired dysregulation of the alternative complement pathway is highly implicated in the pathogenesis of aHUS. Multiple triggers for aHUS have been described in the literature, including pregnancy, transplantation, vaccination, and viral infections. Herein, we report a case of a previously healthy 38-year-old male who developed microangiopathic hemolytic anemia and severe kidney impairment one week after receiving the first dose of AstraZeneca SARS-CoV-2 vaccine. A diagnosis of aHUS was made after excluding other causes of thrombotic microangiopathies. Treatment with plasma exchange, prednisone, and rituximab (375 mg/m2) once weekly for four doses resulted in improvement of his hematological parameters. However, he progressed to ESKD.
ABSTRACT
Background Similar to coronavirus disease 2019 (COVID-19), the pathogenesis of inflammatory rheumatic diseases includes cytokines dysregulation and increased expression of pro-inflammatory cytokines. Although current data from international studies suggest that rheumatic diseases are associated with a higher risk of COVID-19 infection and worse outcomes, there is limited literature in Saudi Arabia. This study aims to evaluate the outcomes and length of hospital stay of COVID-19 patients with inflammatory rheumatic diseases in Saudi Arabia. Method This was a single-center retrospective cohort study that included 122 patients with inflammatory rheumatic diseases and documented coronavirus disease 2019 (COVID-19) infection from 2019 to 2021. Patients with suspected COVID-19 infection, non-inflammatory diseases, such as osteoarthritis, or inflammatory diseases but without or with weak systemic involvement, such as gout, were excluded. Results The vast majority (81.1%) of the patients were females. Rheumatoid arthritis was the most common primary rheumatological diagnosis. The admission rate was 34.5% with an overall mortality rate of 11.5%. Number of episodes of COVID-19 infection, mechanical ventilation, cytokine storm syndrome, secondary bacterial infection, number of comorbidities, rituximab, diabetes mellitus, hypertension, chronic kidney disease, and heart failure were significantly associated with a longer hospital stay. Additionally, hypertension, heart failure, rituximab, mechanical ventilation, cytokine storm syndrome, and secondary bacterial infection were significantly associated with higher mortality. Predictors of longer hospitalization were obesity, number of episodes of COVID-19 infection, mechanical ventilation, number of comorbidities, and chronic kidney disease, whereas, hypertension was the only predictor of mortality. Conclusion Obesity, number of episodes of COVID-19 infection, mechanical ventilation, number of comorbidities, and chronic kidney disease were significantly associated with higher odds of longer hospitalization, whereas, hypertension was significantly associated with higher odds of mortality. We recommend that these patients should be prioritized for the COVID-19 vaccine booster doses, and rituximab should be avoided unless its benefit clearly outweighs its risk.