Patients with vasculitis experience significant delays in achieving an accurate diagnosis, which is associated with negative health outcomes. Both patient- and health care-related factors are associated with a delayed diagnosis of vasculitis, according to results of an analysis published in the Orphanet Journal of Rare Diseases.
The aims of the current study were to identify the average amount of time between the onset of the first vasculitis symptom and the time of diagnosis; to recognize factors associated with the time of vasculitis diagnosis; and to understand patients’ perceptions of the consequences linked to a delayed diagnosis of vasculitis.
Patients who were members of the Vasculitis Patient-Powered Research Network (VPPRN), an online registry, were invited to join the study. The study was carried out as a 2-stage survey.
Stage 1 was a short, open-ended qualitative survey that included questions about patients’ diagnostic journeys and perceived factors that they thought contributed to their rapid or delayed vasculitis diagnosis. Stage 2 was a survey that included specific questions based on data derived from stage 1, along with additional investigator-identified factors.
A total of 375 patients with vasculitis responded to the initial stage 1 survey. Of these individuals, the majority had a form of antineutrophil cytoplasmic autoantibody (ANCA) vasculitis; the remaining participants had Behçet disease, central nervous system vasculitis, immunoglobulin A (IgA), and giant cell arteritis. Survey responses showed that although some participants received a prompt diagnosis, many of them experienced delays in diagnosis, with a median time to diagnosis of 7 months.
Overall, 456 patients participated in the stage 2 survey. Mean patient age of this cohort was 56±15 years; 72% of the patients were women. Similar to the stage 1 survey, the median time to diagnosis of vasculitis was 7 months. Of individuals who were evaluated, 50% were diagnosed with vasculitis within 1 year and 75% were diagnosed within 2 years. The time that elapsed before a diagnosis varied according to the type of vasculitis. Participants with IgA vasculitis had the shortest diagnosis time, with a median of 1 month, whereas those with Behçet disease had the longest time of diagnosis, with a median of 205 months.
A total of 73% (n=313) of the patients in the stage 2 survey were misdiagnosed initially. Overall, 40% of diagnoses were made in a hospital setting and 2% at a specialized vasculitis center. Prior to diagnosis, approximately 60% of the participants reported at least 1 visit to a hospital emergency department. Further, 82% of participants reported that experiencing a delayed diagnosis had a negative impact on their health. Initial misdiagnosis, unemployment, time of more than 1 hour involved in traveling to a medical center, and delays encountered in consulting with a specialist were all associated with longer times to diagnosis.
A major limitation of the current study was the fact that the study population was derived from the online VPPRN, which includes a disproportionate number of women Caucasian patients from North America with ANCA-associated vasculitis, thus decreasing the generalizability of the findings.
Researchers concluded that “…healthcare providers should be better educated on the various ways in which vasculitis can present clinically and what approaches to diagnosis are appropriate in cases of potential vasculitis. Increasing awareness and understanding of vasculitis disorders will hopefully help to streamline the referral process and help eliminate delays in diagnosis.”
Disclosure: One study author declared affiliations with biotech, pharmaceutical, and/or medical device companies. Please see the original reference for a full list of the author’s disclosures.
Sreih AG, Cronin K, Shaw DG, et al; for the Vasculitis Patient-Powered Research Network. Diagnostic delays in vasculitis and factors associated with time to diagnosis. Orphanet J Rare Dis. 2021;16(1):184. doi:10.1186/s13023-021-01794-5