Background/Purpose
To study the long-term outcomes of 65 patients with non-HBV polyarteritis nodosa (PAN) or microscopic polyangiitis (MPA) enrolled in a prospective, randomized, open-label trial,1 with Five-Factor Score (FFS)-defined poor-prognosis factors, focusing on survival, relapses, clinical and laboratory findings, therapeutic responses and sequelae.
Methods
Patients’ data were updated in 2014. The new Chapel Hill criteria were applied to classify PAN and MPA. The following definitions were used: relapses: recurrence and/or new appearance of ≥1 vasculitis manifestation(s) after remission lasting ≥3 months; failure: no clinical remission with the assigned treatment. Times to relapse and/or death were calculated from treatment onset. For survival analyses, data were censored after 90 months of follow-up.
Results
Mean±SD overall follow-up was 65.6±47.5 months, comparable for PAN and MPA patients. For the 65 patients (41 MPA and 24 PAN), mean age at diagnosis was 55.3±17.0 years, mean Birmingham Vasculitis Activity Score 2003 23.7±8.8; FFS=1, 2 or ≥3 for 27, 30 or 8 patients, respectively; ANCA-positivity: 2 (8.3%) PAN (1 cANCA+ and 1 pANCA+, anti-myeloperoxidase [MPO]– and proteinase–3–negative) and 34 (82.9%) MPA (pANCA+, 90.6% MPO-specific). Patients received 3 methylprednisolone pulses and corticosteroids (CS) and were randomized to receive 6 or 12 intravenous cyclophosphamide (CYC) pulses. After treatment, 53/65 (81.5%, 32 MPA and 21 PAN) entered remission. Treatment was intensified for the 12 nonresponders: 4 achieved remission and 8 died before remission. After remission, 25/57 (43.9%, 18 MPA, 7 PAN) patients relapsed 29.4±27.4 months after starting treatment. The respective 3-, 5- and 7-year overall survival rates were 79.5%, 72.3% and 64.4%, with no significant difference between PAN and MPA patients (p=0.241). Overall survival tended to be shorter for patients given 6 versus 12 CYC pulses (p=0.157). The respective 1-, 3- and 5-year relapse-free–survival rates were 84%, 68.4% and 52.4%, comparable for PAN and MPA patients. The relapse-free–survival difference between patients that received 6 versus 12 CYC pulses tended to decline during follow-up and was no longer significant at 90 months (p=0.07). At the last follow-up visit, 38/65 (58.5%) patients were still alive, 12/38 (31.6%) were still taking CS and 8/38 (21.1%) an immunosuppressant (IS). The mean vasculitis damage index score for the 57 patients with ≥1 remission(s) was 2.3±1.5, with the most frequent sequelae being hypertension (50%), chronic renal failure (creatininemia >150 µmol/L) (44.6%) and neuropathy (23.2%). Notably, 7 patients, all with MPA, progressed to end-stage renal failure and required chronic dialysis; 2 of them received kidney transplants.
Conclusion
For non-HBV PAN or MPA patients with FFS≥1 at diagnosis, overall survival at 7 years reached 64%. Relapses were frequent during long-term follow-up, even for patients who had received 12 CYC pulses at diagnosis, thereby confirming that PAN and MPA patients with FFS≥1 at diagnosis require post-remission maintenance therapy with an IS or biotherapy.
Reference:
1. Guillevin L et al. Arthritis Rheum 2003;49:93–100
Disclosure:
M. Samson,
None;
X. Puéchal,
None;
H. Devilliers,
None;
P. Cohen,
None;
B. Bienvenu,
None;
K. H. Ly,
None;
A. Bruet,
None;
B. Gilson,
None;
M. Ruivard,
None;
E. Pertuiset,
None;
M. Hamidou,
None;
B. Terrier,
None;
C. Pagnoux,
None;
L. Mouthon,
None;
L. Guillevin,
None;
T. French Vasculitis Study Group (FVSG),
None.
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