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Rheumatology (Oxford). 2017 Oct 1;56(10):1684-1693. doi: 10.1093/rheumatology/kex047.

Maintenance therapy is associated with better long-term outcomes in adult patients with primary angiitis of the central nervous system.

Author information

1
Department of Internal Medicine, Caen University Hospital, University of Caen-Basse Normandie.
2
Biostatistics and Clinical Research Unit, Caen University Hospital, Caen.
3
Department of Neurology, Montpelier University Hospital Gui de Chauliac, INSERM UMR 894, Montpellier.
4
Department of Neuroradiology, Sainte-Anne Hospital Center, AP-HP, University Paris-Descartes, INSERM UMR 894, Paris.
5
Department of Neurology, Perpignan Hospital, Perpignan.
6
Department of Internal Medicine, Cochin Hospital Center, AP-HP, Paris.
7
Department of Internal Medicine, Nantes University Hospital, Nantes.
8
Department of Neurology, Grenoble Alpes University Hospital, Grenoble.
9
Department of Neurology, Caen University Hospital, University of Caen-Basse Normandie, Caen.
10
Department of Neurology, Saint-Joseph Hospital Center, AP-HP, Université Paris-Descartes, INSERM UMR S 919, Paris.
11
Vasculitis Clinic, Division of Rheumatology, Mount Sinai Hospital, Toronto.

Abstract

Objective:

We aimed to analyse the effect of maintenance therapy after induction on the outcomes of adult patients with primary angiitis of the CNS (PACNS).

Methods:

We analysed long-term outcomes (relapse, survival and functional status) of patients enrolled in the French multicentre PACNS cohort who achieved remission after induction treatment and with ⩾12 months' follow-up, according to whether or not they received maintenance therapy. Good outcome was defined as relapse-free survival and good functional status (modified Rankin scale ⩽ 2) at last follow-up.

Results:

Ninety-seven patients [46 (47%) female, median age: 46 (18-78) years at diagnosis] were followed up for a median of 55 (5-198) months. Induction treatment consisted of glucocorticoids in 95 (98%) patients, combined with an immunosuppressant in 80 (83%) patients, mostly CYC. Maintenance therapy was prescribed in 48 (49%) patients, following CYC in 42 of them. Maintenance therapy was started 4 (3-18) months after glucocorticoid initiation. At last follow-up, good outcomes were observed in 32 (67%) patients who had received maintenance therapy vs 10 (20%) who had not (P < 0.0001). Thirty-two (33%) patients experienced relapse [10 (22%) had received maintenance therapy while 22 (45%) had not, P = 0.01]; four subsequently died from relapse. In the multivariate analysis, maintenance therapy was the only independent predictor of good outcome [odds ratio (OR) = 7.8 (95% CI: 3.21, 20.36), P < 0.0001].

Conclusion:

The results of this long-term follow-up study suggest that maintenance therapy in adults with PACNS is associated with better functional outcomes and lower relapse rates. Further studies are needed to confirm these findings.

KEYWORDS:

MRI; biopsy; large/medium-vessel; primary angiitis of the central nervous system; relapse; small-vessel

PMID:
28340158
DOI:
10.1093/rheumatology/kex047
[Indexed for MEDLINE]

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