Background/Purpose: Despite recent advancements in the evaluation and management of vasculitis, patients with vasculitis continue to encounter diagnostic delays. These delays are often associated with substantial morbidity and mortality. This study sought to describe the diagnostic journey of patients with vasculitis from the onset of symptoms to diagnosis, and identify factors associated with time to diagnosis.

   Methods: Patients enrolled in an online registry completed a two-stage mixed-methods study: Stage 1: Survey consisting of open-ended questions about patients’ diagnostic journeys and the perceived factors associated with rapid or delayed diagnosis; and Stage 2: Survey with specific questions based on data from Stage 1 and additional investigator-identified factors.  Multivariate linear regression analysis was used to identify factors associated with time to diagnosis. Factors were divided into patient-related factors and healthcare-related factors. Patient Research Partners participated in idea conception, study design, and patient-engagement.

   Results: 375 patients with vasculitis participated in Stage 1 and 456 patients participated in Stage 2. The median age (IQR) was 59 (24). 72% were females and 94% Caucasians. The majority (74%) of patients sought medical attention within 3 months of their symptoms and 85% were seen by a healthcare provider within 3 months. The mean time to diagnosis of vasculitis (± SD) was 3.3 ± 7 years with IgA-vasculitis having the shortest time (0.3 ± 0.5 years) and Behçet’s disease having the longest time (20.4 ± 15 years) (Table 1). 313/456 (73%) of patients were misdiagnosed initially (33% were diagnosed with infections, 29% with an autoimmune disease, and 11% with a “fake” illness). The median (IQR) number of misdiagnoses was 5 (3). 40% of diagnoses were made in a hospital setting (35% inpatient and 5% emergency room [ER]). Only 2% of diagnoses were made at a specialized vasculitis center. More than half of patients had at least 1 ER visit prior to diagnosis, with 5% of patients having > 10 ER visits. Unemployment, time to travel to a medical center greater than 1 hour, being initially misdiagnosed, and delays in seeing a specialist were all associated with longer times to diagnosis (Table 1). 373/456 (82%) of patients reported that a delayed diagnosis had negative consequences on their health: 55% of patients thought it made their condition worse, 16% lost their job, and 11% became disabled.

   Conclusion: Patients with vasculitis encounter substantial delays in achieving an accurate diagnosis and these delays are associated with negative health consequences. Both patient-related factors and healthcare-related factors are associated with diagnostic delays. Future efforts should focus on mechanisms to address modifiable factors and shorten delays in diagnosis for patients with new-onset vasculitis.