Background/Purpose:

Diagnosing ANCA-associated vasculitides (AAVs) can be challenging. Their clinical presentations are numerous and the time to diagnosis may range from days to years.^1,2 The impact of delayed diagnosis on AAV outcome has not been established. This study was conducted to assess the first-symptom-to-diagnosis times for patients with granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA).

Methods:

Adults diagnosed with new onset GPA or MPA between 2005 and 2015 and followed at Cochin National Referral Center for Vasculitides were selected from the French Vasculitis Study Group (FVSG) Registry. Patients were excluded when the date and first symptom were incomplete. First symptom (systemic vs ENT), clinical and laboratory findings, Birmingham Vasculitis Activity Score (BVAS) at diagnosis, time to diagnosis and Vasculitis Damage Index (VDI) at last visit were collected from the FVSG registry’s database and medical records. The first symptom had been systematically assessed by the treating physician at diagnosis. Data are expressed as median, interquartile range. Pearson and Mann–Whitney tests were used to analyze data.

Results:

Among 257 patients screened, 29 were excluded. The study population comprised 228 patients: 169 with GPA and 59 with MPA (median age at diagnosis 55 years [42–65]; M/F sex ratio 0.81). At diagnosis, their median BVAS was 12 [7–18], 44% had renal involvement and 22% had 2009 Five Factor Scores ≥1. The median time to diagnosis was 5.1 [2.1–16.2] (range 0.1–356) months, for which GPA and MPA did not differ significantly. Among AAV patients’ first symptoms, 174 (76%) were systemic and 54 (24%) were ENT, with longer times to diagnosis when ENT was the first symptom (8.65 vs 4.1 systemic; P<0.0001). The median time from first ENT to first systemic symptom was 6.1 [3.6–33] months. The time to diagnosis was significantly longer in women (6.1 vs 4.1 men; P<0.05) and in the absence of renal involvement (6.1 vs 3.2 renal involvement; P<0.01). No association was found between time to diagnosis and age, date of diagnosis, BVAS at diagnosis, or VDI at last visit. Thirty-nine (17%) patients, mostly with GPA (27, 69%), were diagnosed >24 months after the first symptom, which was mainly arthritis/arthralgias (n=9), retroorbital tumor (n=5), purpura/livedo (n=5), thoracic nodules (n=5), ENT (n=5) or others (n=10). Corticosteroids (4 patients) or immunosuppressants (3 patients) were prescribed before AAV diagnosis.

Conclusion:

The time to AAV diagnosis remains long, especially when the first symptom involves ENT. ANCA testing might shorten the time to diagnosis, and should be considered even in pauci-symptomatic patients.

References:

¹ Yacyshyn E. Joint Bone Spine 2016;83:599.

² Mohammad AJ. Rheumatology (Oxford) 2007;46:1329.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/time-to-diagnosis-of-anca-associated-vasculitides-data-from-french-vasculitis-study-group-registry/