In this mixed-methods study, tangible factors for optimizing the mentor-mentee relationship were identified and will inform the development of an adult rheumatology inter-institutional mentoring program.
Background
Patients with vasculitis, a set of rare diseases, encounter delays in obtaining an accurate diagnosis which can lead to substantial morbidity and increased mortality. This study sought to describe the diagnostic journey of patients with vasculitis and identify factors associated with time to diagnosis.
Methods
Patients with vasculitis enrolled in an online registry completed a two-stage study: Stage 1: survey of open-ended questions about patients’ diagnostic journeys and perceived factors associated with rapid or delayed diagnosis; Stage 2: survey with specific questions based on data from Stage 1 and additional investigator-identified factors.
Results
375 patients with vasculitis participated in Stage 1; 456 patients participated in Stage 2. 85% of patients were seen by a healthcare provider within 3 months of the onset of symptoms. The median time to diagnosis of vasculitis was 7 months. 313/456 (73%) of patients were misdiagnosed initially. 40% of diagnoses were made in a hospital setting; 2% of diagnoses were made at a specialized vasculitis center. 60% of patients had at least 1 visit to an emergency room prior to diagnosis. Unemployment, time to travel to a medical center > 1 h, initial misdiagnosis, and delays in seeing a specialist were all associated with longer times to diagnosis. 373/456 (82%) of patients reported that a delayed diagnosis had negative consequences on their health.
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.
Variability in systemic lupus erythematosus (SLE) disease manifestations is well recognized. Lupus disease activity can range from mild to severe. Fever is a common manifestation of SLE and occurs in 36%–86% of patients. In the Modified Systemic Lupus Erythematosus Disease Activity Index (M-SLEDAI), fever is taken into account as disease activity scoring. Assessment of lupus patients with fever is an important diagnostic challenge, since the initial clinical presentation of a patient with lupus is very similar to the acute febrile phase of an infection. The attribution of fever to SLE holds only after other causes are excluded.
Haptoglobin levels are measured in systematic lupus erythematosus patients as part of the workup for anemia, with low levels indicating hemolysis. Haptoglobin is an acute phase protein. We present 2 lupus patients who were found to have low haptoglobin levels in the absence of other evidence of hemolysis.
Glomerulonephritis (GN) in lupus is generally an immune complex glomerulonephritis from the deposition of immunoglobulin and complements. Pauci-immune GN is the most common cause of rapidly progressive GN and is frequently associated with an anti-nuclear cytoplasmic antibody (ANCA). We report a patient with a history of systemic lupus erythematosus who presented with worsening proteinuria and was subsequently diagnosed with pauci-immune GN on renal biopsy, in the absence of ANCA.
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