Background/Purpose:  Despite progress in induction and maintenance therapy in ANCA-associated vasculitis (AAV), relapses remain the main challenge in this disease with a rate above 50%. Few data exist on the clinical features of those relapses. Our goal was to investigate the severity of relapse events (RE) and their mode of presentation in patients with AAV.

Methods:   Patients from a single center diagnosed with AAV – granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA) and microscopic polyangiitis (MPA) – between 1990 and 2014, and experiencing at least one relapse according to Hellmich and al (Ann Rheum Dis 2007; 66: 605-17), were retrospectively investigated. The different organ involvements were registered during each relapse. Presentation at onset and RE were compared for each patient. The BVAS was used to assess the activity of each relapse. Statistical analysis was performed using SAS v9.4. Chi-square test of McNemar and Student test were used on paired time series to compare the proportions, and to compare mean values between initial onset and relapses respectively.

Results:   Ninety-nine patients with AAV were followed in our centre: 53 patients (30 male/23 female, mean age at diagnosis 58,2 years old [49-70]) experiencing at least one relapse during their follow-up were included – 38 GPA patients (72%), 3 MPA (6%), 12 EGPA (22%) – with a total of 96 RE. The rate of RE in our series was 53% with a median time of follow-up of 81.5 months (36-130). The mean time to first RE after initial onset was 25 months. The distribution of patients was as followed: thirty patients experienced one single RE, 14 had 2, 5 had 3, 2 had 4 and 2 had respectively 7 and 8. Fifty-five percent of relapse events had the same initial organ as initial onset. Compared to initial onset, some clinical manifestations were less present: general symptoms (30% vs 69%, p<0.0001) including fever and weight loss; ear-throat-nose (ETN) (53% vs 81%, p<0.0001) including sinusitis, chondritis and conductive hearing loss; lung involvement (59% vs 74%, p=0.0071), with nodules (28% vs 44%, p=0.00071), interstitial infiltrates (13% vs 32%, p=0.004), pleural effusion (3% vs 12%, p=0.014) and alveolar hemorrhage (7% vs 27%, p=0,0002); peripheral neuropathies (7% vs 18%) and cranial nerves involvement (5% vs 20%) (p=0,0007); joints with arthritis or arthralgia alone. Moreover, some organs involvement known as being associated with a worse prognosis at initial onset were significantly less present: kidney with hematuria, proteinuria and acute kidney injury; heart but mostly related to pericarditis (1% vs 22%, p<0.0001), myocarditis being rare (2% vs 3%). Skin, eye and bowel manifestations were not significantly less involved during relapse events, as well as nasal crusting and ulcers, and bloody nasal discharge. The mean BVAS at initial onset was 9,47 (6-11) and 5,09 (3-6) at relapse (p<0,0001). Among the 96 relapse events, 44 (45.8%) had a new organ involvement compared to initial onset. None of these new manifestations were life-threatening.

Conclusion: Our study suggests that global activity of RE in AAV patients is significantly lower than on initial onset. Fewer organs seem to be involved in relapses, as shown by the decrease of the BVAS between diagnosis of the AAV and the RE. This probably reflects the effect of the follow-up visits. RE turn out to begin with the same manifestations as initial onset in most of cases which could facilitate early recognition of relapses.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/relapses-in-patients-with-anca-associated-vasculitis-a-retrospective-study-on-severity-and-organ-involvement-compared-to-initial-onset/