Background/Purpose:
Polyarteritis nodosa (PAN) and microscopic polyangiitis (MPA) are 2 vasculitides characterized by necrotizing inflammation of the vessel wall. They share several clinical features and may be treated similarly. Nonsevere manifestations, as defined by the Five-Factor Score (FFS), respond to corticosteroids (CS) alone. This study aimed to describe the long-term follow-up of PAN and MPA patients without poor-prognosis factors.
Methods:
Data from patients included in a prospective trial1 were updated in 2012. New Chapel Hill criteria were applied to classify PAN and MPA. The following definitions were used: relapses, the recurrence and/or new appearance of ≥1 vasculitis manifestation(s) after remission lasting ≥3 months; major relapses, the emergence of major organ involvement (FFS≥1, 30% creatinine-level rise, pulmonary hemorrhage, threatened vision, new multifocal neurological lesions or mononeuritis multiplex, gastrointestinal hemorrhage or perforation and/or gangrene); failure, the absence of clinical remission with the assigned treatment. Times to relapse and/or death were calculated from that of treatment onset. Time to first event (failure, minor or major relapse and/or death) defined the disease-free survival.
Results:
Among the 124 patients screened, 6 were excluded (2 FFS≥1, 4 other vasculitides). Mean±SD overall follow-up was 98.2±41.9 months. For the 118 patients (61 MPA and 57 PAN), mean age at diagnosis was 55.6±16.5 years, mean Birmingham Vasculitis Activity Score 2003 11.8±5.5; ANCA-positivity: 3 (5.3%) PAN (cANCA+, anti-proteinase-3 and -myeloperoxidase negative) and 31 (50.8%) MPA (pANCA+, 77.4% myeloperoxidase-specific). After CS alone, 97/118 (82.2%, 49 MPA and 48 PAN) achieved remission; 21/118 (17.8%, 12 MPA, 9 PAN) failed on CS and received a second- or third-line therapy with immunosuppressant(s) (IS) that achieved remission in 19 cases (11 MPA, 8 PAN), and 2 patients (1.7%, 1 PAN, 1 MPA) died before remission. After remission, 61/116 (52.6%, 35 MPA, 26 PAN) patients relapsed 25.6±27.9 months after starting treatment, 30 (25.9%, 20 MPA, 10 PAN) experiencing ≥1 major relapse after 47.8±36.2 months of follow-up. The respective 5-, 7- and 8-year overall survival rates were 92%, 85% and 81%, with no significant difference between PAN and MPA patients (p=0.289). Relapse-free survival and major relapse-free survival tended to be shorter for MPA than PAN patients (p=0.174 and 0.06, respectively). Disease-free survival was significantly shorter for MPA than PAN patients (p=0.021). Throughout follow-up, 46.6% of patients required ≥1 IS. At the last follow-up visit, 44% were still taking CS, 15% an IS and the mean vasculitis damage index score was 1.9±1.9, with the most frequent sequelae being peripheral neuropathy, hypertension and osteoporosis.
Conclusion:
For PAN or MPA patients with FFS=0 at diagnosis, overall survival at 120 months was good, with first-line CS alone able to achieve remission in >80% of them. However, relapses remained frequent, especially of MPA, meaning that 46.6% of the patients required immunosuppressant(s).
Disclosure:
M. Samson,
None;
X. Puechal,
Pfizer Inc,
5,
Roche Pharmaceuticals,
5;
H. Devilliers,
None;
C. Ribi,
None;
P. Cohen,
None;
B. Bienvenu,
None;
C. Pagnoux,
None;
L. Mouthon,
None;
L. Guillevin,
None;
F. V. S. G. FVSG,
None.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/long-term-follow-up-of-118-polyarteritis-nodosa-and-microscopic-polyangiitis-without-poor-prognosis-factors/