Identifying relevant determinants of in-hospital time to diagnosis for ANCA-associated vasculitis patients

Abstract

Objectives: Diagnosing patients with ANCA-associated vasculitis (AAV) can be challenging owing to its rarity and complexity. Diagnostic delay can have severe consequences, such as chronic organ damage or even death. Given that few studies have addressed diagnostic pathways to identify opportunities to improve, we performed a clinical audit to evaluate the diagnostic phase.

Methods: This retrospective, observational study of electronic medical records data in hospitals focused on diagnostic procedures during the first assessment until diagnosis.

Results: We included 230 AAV patients from nine hospitals. First assessments were mainly performed by a specialist in internal medicine (52%), pulmonology (14%), ENT (13%) or rheumatology (10%). The overall median time to diagnosis was 13 [interquartile range: 2-49] days, and in patients primarily examined by a specialist in internal medicine it was 6 [1-25] days, rheumatology 14 [4-45] days, pulmonology 15 [5-70] days and ENT 57 [16-176] days (P = 0.004). Twenty-two of 31 (71%) patients primarily assessed by a specialist in ENT had non-generalized disease, of whom 14 (64%) had ENT-limited activity. Two hundred and nineteen biopsies were performed in 187 patients (81%). Histopathological support for AAV was observed in 86% of kidney biopsies, 64% of lung biopsies and 34% of ENT biopsies.

Conclusion: In The Netherlands, AAV is diagnosed and managed predominantly by internal medicine specialists. Diagnostic delay was associated with non-generalized disease and ENT involvement at presentation. Additionally, ENT biopsies had a low diagnostic yield, in contrast to kidney and lung biopsies. Awareness of this should lead to more frequent consideration of AAV and early referral for a multidisciplinary approach when AAV is suspected.

Keywords: ANCA-associated vasculitis; diagnostic delay; health-care usage; patient trajectory; pauci-immune glomerulonephritis.

Fig. 1

Reported results of performed biopsies Supportive = supported the diagnosis of ANCA-associated vasculitis (AAV). Non-supportive = excluded the diagnosis of AAV and, by definition, supported or confirmed a diagnosis other than AAV. Non-specific = neither unable to exclude the diagnosis of AAV nor was it able to support or confirm another diagnosis. n: number of patients.

Fig. 2

Kaplan–Meier curve of the time to diagnosis (A) Time to diagnosis in all patients with generalized disease (black) vs non-generalized disease (orange). (B) Time to diagnosis in all patients assessed primarily by specialists from internal medicine (blue), rheumatology (red), ENT (green) and pulmonology (orange).