ABSTRACT
Objective: To describe outcomes of the coronavirus disease 2019 (COVID-19) in stiff person syndrome (SPS). Background: The COVID-19 pandemic has impacted people with autoimmune disorders to varying degrees. Several risk factors for more severe COVID-19 outcomes in different populations have been identified including medical comorbidities and immunosuppressant therapies (IST). These risk factors are often present in people with SPS, however, little is known about the impact of COVID-19 in the SPS population. Design/Methods: Individuals with a diagnosis of SPS seen at Johns Hopkins Hospital are followed as part of a longitudinal observational study. Data collection includes demographics, disease characteristics, laboratory studies, and outcomes. Individuals who tested positive for COVID-19 were identified and information on their disease severity and COVID-19 outcomes were obtained. Results: Fourteen SPS patients were identified as testing positive for COVID-19. Mean (standard deviation) age at COVID-19 diagnosis was 52(±12) years, most were female (86%) and half were non-Caucasian. The most common COVID-19 medical comorbidities noted were obesity(n=4), diabetes(n=3), and cardiovascular disease(n=2). Four individuals were on IST (rituximab, mycophenolate mofetil, or methotrexate). The majority(79%) of patients did not require hospitalization. Three individuals required hospitalization including intensive care and mechanical ventilation, and all had diabetes. One individual on rituximab with underlying diabetes, obesity, and hypertension died from COVID-19 complications. Two others received monoclonal antibody infusions in the outpatient setting. There was one breakthrough infection following full COVID-19 vaccination;occurred 4 months after vaccination and did not require hospitalization. Conclusions: The majority of people with SPS in this case series did not require hospitalization due to COVID-19. However, the individuals that required hospitalization also had underlying comorbidities that have been associated with more severe COVID-19 outcomes in other populations. One breakthrough COVID-19 case was reported thus far, hence, public health preventative measures are critical no matter vaccination status.