Those who were initially misdiagnosed waited nearly four years before receiving a vasculitis diagnosis, compared with 1.6 years for patients who were not initially misdiagnosed."
Creating ways to speed the process of getting an accurate diagnosis would help the health outcomes of patients with ANCA-associated vasculitis and other types of vasculitis, a study says.
Delays in obtaining an accurate diagnosis are linked to unemployment, the need to travel long distances to a medical center, long wait times to see a specialist, and an initial misdiagnosis.
Therefore, greater awareness about the disease, easier healthcare access, and better healthcare provider education is "of the utmost importance," researchers say.
Their study, "Diagnostic delays in vasculitis and factors associated with time to diagnosis," was published in the Orphanet Journal of Rare Diseases.
Vasculitis encompasses a diverse set of rare disorders affecting blood vessels. These conditions often appear similar to other, better known illnesses, leading to delayed diagnoses, which in turn affect the course and outcomes of a patient's disease.
A team of researchers from various U.S. institutions sought to address these delays by determining how much time tends to pass between the onset of vasculitis symptoms and diagnosis, identifying what causes delays, and understanding how patients perceived the consequences of a delayed diagnosis.
The investigators recruited members of the Vasculitis Patient-Powered Research Network to participate in two online surveys. The first (Stage 1) consisted of open-ended questions about which factors patients believed contributed to their vasculitis diagnosis. The second (Stage 2) incorporated those answers into questions about the onset of symptoms, tests done, the types of healthcare providers seen, and the type of provider and test that confirmed a vasculitis diagnosis.
In sum, 375 individuals responded to Stage 1 and 456 replied to Stage 2. Most respondents had AAV, but other forms of vasculitis included Behçet's disease, giant cell arteritis, central nervous system vasculitis, and polyarteritis nodosa.
Overall, unemployment, traveling over one hour to reach a medical center, an initial misdiagnosis, and delays in seeing a specialist all associated with longer times to diagnosis.
Stage 1 responses indicated that patients believed access to specialists and to specialist facilities have had the greatest impact on how quickly they were diagnosed. Respondents who reported provider-related delays in diagnosis felt that their provider often lacked knowledge about vasculitis.
Conversely, respondents with prompt diagnoses attributed this to easily-accessed specialists, who worked well in a care team, were willing to refer people to other providers, and were willing to conduct diagnostic tests.
In the Stage 2 survey, most respondents (85%) said that they saw a healthcare provider within three months of their first symptoms and the median time to diagnosis was seven months. Half (50%) were diagnosed within one year and 75% were diagnosed within two years.
Nearly three-quarters (73%) of participants were initially misdiagnosed, with the most common misdiagnoses being infection (33%) and autoimmune disease (29%). Respondents received a median of five incorrect diagnoses before vasculitis was correctly identified.
Those who were initially misdiagnosed waited nearly four years before receiving a vasculitis diagnosis, compared with 1.6 years for patients who were not initially misdiagnosed.
Two-fifths of vasculitis diagnoses occurred in hospitals, while 2% happened in vasculitis specialty care centers. Also, 60% of participants visited the emergency room at least once before diagnosis.
Symptoms also affected time to diagnosis. Individuals experiencing genital ulcers, blood in their stool, scalp tenderness, mouth sores, and nausea or digestive issues tended to have longer delays, while those with bleeding in their lungs were diagnosed faster.
Most respondents (82%) claimed that a delayed diagnosis negatively affected their health, mostly through a worsening of their condition (55%), losing their job (16%), or becoming disabled (11%).
The study's findings suggest that patient-related and healthcare system-related factors contributed to delays, and that patients will benefit from establishing ways to reach an accurate diagnosis faster.
"These conclusions," the researchers wrote, "indicate 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."
Vasculitis presenting in the skin presents with hemorrhage into the skin with blood vessel inflammation. The physical exam finding for this is called "palpable purpura". It is often is missed. Even more so in patients of color as red or purple on brown looks like a deep brown. On www.projectimpact.org there are image comparisons of diagnoses presenting in dark skin vs light. One of the examples is immunoglobulin A vasculitis formerly called Henoch Schoenlein Purpura. The pictures are worth a thousand words in describing the challenges for non-experts. Hope this is helpful background.