The care of vasculitis patients during the ongoing COVID-19 pandemic represents an extraordinary challenge: Once diagnosed, patients require regular follow-up visits to assess treatment responses, track symptoms, perform blood tests, monitor potential adverse events and comorbidities, and re-evaluate treatment indications (1).

At the beginning of the pandemic, risk factors associated with mortality and morbidity associated with COVID-19 infections were unknown and optimal management of immunosuppressive therapy was uncertain. This resulted in widespread anxiety and a high rate of self-isolation among patients diagnosed with rheumatic and musculoskeletal diseases (RMDs) (2, 3).

German (Deutsche Gesellschaft für Rheumatologie, DGRh) and European (European Alliance of Associations for Rheumatology, EULAR) organizations ultimately developed a set of recommendations and provided guidelines to address these concerns (4–7).

Specifically, these guidelines recommended to balance the benefits of regular visits (monitoring of disease activity, complications and comorbidities) and the risks associated with direct in-person consultations. Consequently, extended follow-up intervals could be considered for patients in a stable disease status (4).

Ultimately, vaccinations against COVID-19 were approved and recommended for patients diagnosed with RMDs (7–9). Reported vaccination rates in these patients ranged from 30.2 to 80% between June and October 2021 (10–12).

Against this background, our study aims to examine the impact of COVID-19 on the medical care of vasculitis patients across specialized centers in Germany and to evaluate their overall acceptance of COVID-19 vaccination.

A web-based national survey was developed by rheumatology centers in collaboration with vasculitis patient advocacy groups. An expert panel identified two areas of interest to investigate: (1) medical care of vasculitis patients during the COVID-19 pandemic and (2) vaccine acceptance.

After performing a literature search, members of the panel designed a questionnaire based on the standard operating procedures outlined by the EULAR recommendation task force (13). The questionnaire was shared with members of vasculitis patient advocacy groups who reviewed the draft version and provided critical insight and patient perspectives.

The web-based survey (LimeSurvey, https://www.limesurvey.org/) was distributed nationwide by members of patient advocacy groups and clinical rheumatologists via flyers and mail. The survey was accessible via a QR code or weblink from December 9, 2021, to April 19, 2022. Informed consent was obtained from all participants prior to study participation. Patients with self-reported diagnoses of small and large vessel vasculitis, including granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), microscopic polyangiitis (MPA), giant cell arteritis (GCA) and Takayasu arteritis (TAK) were included in the analysis. Participation was voluntary and there were no incentives provided. Internet protocol (IP) addresses were used to identify potential duplicate entries. Responses from incomplete questionnaires were included in the analysis as appropriate. This resulted in differences of the overall number of patients for different questions within the questionnaire. The respective total numbers are indicated in the text.

The study methodology and results are reported according to the Checklist for Reporting Results of Internet E-Surveys (14).

We report findings from a patient survey focused on the medical care provided to vasculitis patients during the COVID-19 pandemic. To the best of our knowledge, this is the first patient survey designed to assess (1) the changes in medical care provided to vasculitis patients in the COVID-19 pandemic and (2) vaccination acceptance in this patient cohort. While several published reports document the results of surveys that included patients with RMDs, the proportion of vasculitis patients was small and their responses were not analyzed separately; in other studies, only data from the first month of the pandemic were presented (18–22).

Participating patients in this study reported that prescheduled appointments for the disease vasculitis had been canceled due to COVID-19 (10.3%) or had been replaced by digital services (7.1%). The reported proportions are lower than those reported in a survey of rheumatologists throughout Europe that included patients with various RMD in which 82% stated that they had canceled patient appointments due to COVID-19 (23). Therefore, vasculitides might be considered as diseases that require closer monitoring compared to other RMDs.

At the beginning of the pandemic, patients and physicians faced substantial uncertainty due to the lack of data regarding the clinical course of COVID-19 in patients diagnosed with RMDs, most notably those managed with immunosuppressive therapy. The first findings to emerge suggested that rituximab therapy was associated with an increased risk of severe COVID-19 (8, 24, 25). Consequently, recommendations for a more stringent risk-benefit analysis were brought forward. However, recent studies have highlighted the extraordinary efficacy of rituximab for induction of remission and maintenance therapy in small vessel vasculitides (26–28). Rituximab was the most commonly used immunosuppressive drug in our patient cohort for patients diagnosed with AAV and remained the treatment of choice for 75.9% of the participants despite the pandemic.

For other immunosuppressive therapies, it was recommended to continue necessary treatments because ongoing disease activity was considered to pose a greater risk than COVID-19 infections (8). Only 17.6% (n = 18) of the respondents of our study reported changes in their therapeutic regimens (i.e., shifts, reductions, or discontinuations) due to COVID-19. Our results are consistent with previous reports that documented no major changes in the management of vasculitis patients during the COVID-19 pandemic (20, 29). This might be explained by the profound importance of current drug regimens used to prevent flare-ups in order to prevent irreversible damage or death in patients with uncontrolled vasculitis (30, 31).

Interestingly, the proportion of respondents who had already contracted and recovered from COVID-19 at the time the survey data was collected was slightly lower than that reported in the general population. These differences might be explained by the high proportion of participants who reported concerns regarding their potentially higher susceptibility to COVID-19. These individuals may have more stringently reduced their in-person social interactions and taken other safety precautions (2). Although the level of concern regarding their potentially higher susceptibility to COVID-19 was high in our cohort, a meta-analysis of data collected from studies that documented the prevalence of immunosuppression among patients with COVID-19 (n = 10,049) revealed that these patients were at no increased risk of contracting this infection (32). However, the meta-analysis did not explore the impact of different immunosuppressive therapies and diseases.

Overall, the study participants exhibited a positive attitude toward vaccinations. The majority (n = 92/104; 88.5%) stated they had never refused any vaccinations. The rate of COVID-19 vaccination was exceptionally high compared to previously reported studies focused on RMD patients (10–12) as well as in the general population in Germany¹.

To interpret the COVID-19 vaccination rates in our cohort, patients were asked about other recommended vaccinations. We used these data to compare their rates of vaccination with individuals in the general population. Apart from the high COVID-19 vaccination rate in our study population, our findings also revealed a generally high rate of vaccination among members of our study, e.g. against influenza and pneumococcus (two- and three-fold higher, respectively). Compared to 2018 (i.e., before the COVID-19 pandemic), Harrison et al. (33) reported lower vaccination rates against influenza in RMDs patients. The increased acceptance of vaccination, in general, might be due to the increased information, public discussion and awareness of the need for vaccination during the COVID-19 pandemic as already observed by Starrostzik (34).

Among the strengths of our study, we note the comparatively large cohort size, patient recruitment based at multiple centers and collaboration with vasculitis patient advocacy groups to develop the survey. We do recognize the potential bias inherent in an online survey. In addition, there seems to be a bias toward patients with small-vessel vasculitis, as this disease is less common than large-vessel vasculitis in the general population (35, 36), but more prevalent in our cohort. This also explains the large proportion of patients reporting a therapy with rituximab, which is used as one standard therapy in ANCA-associated vasculitis. Since our survey was voluntary, more patients with concerns about COVID-19 infections may have participated in the survey.

Participants of our self-reported survey experienced changes in medical care during the COVID-19 pandemic. Compared to the general population, acceptance of vaccinations, especially against COVID-19, was considerably high on our cohort.

The raw data supporting the conclusions of this article will be made available by the authors, without undue reservation.

The studies involving human participants were reviewed and approved by the Ethics Committee of the Medical Faculty of the University of Duesseldorf, Germany (2021-1405). The patients/participants provided their written informed consent to participate in this study.

AK and TF performed the data analyses. AK and GC drafted the first version of the manuscript. All co-authors were involved in the critical interpretation of the results, discussed the findings together, critically reviewed the manuscript and approved its final version. All authors designed the study and contributed to data collection.