Hubert de Boysson1, Jean-Jacques Parienti2, Caroline Arquizan3, Grégoire Boulouis4, Nicolas Gaillard3,5, Alexis Régent6, Antoine Néel7, Olivier Detante8, Emanuel Touzé9, Achille Aouba1, Boris Bienvenu1, Loïc Guillevin6, Olivier Naggara4, Mathieu Zuber10, Christian Pagnoux11. 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.
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.
Authors: Simon Schuster; Ann-Kathrin Ozga; Jan-Patrick Stellmann; Milani Deb-Chatterji; Vivien Häußler; Jakob Matschke; Christian Gerloff; Götz Thomalla; Tim Magnus Journal: J Neurol Date: 2019-03-23 Impact factor: 4.849
Authors: L A Benjamin; E Lim; M Sokolska; J Markus; T Zaletel; V Aggarwal; R Luder; E Sanchez; K Brown; R Sofat; A Singh; C Houlihan; E Nastouli; N Losseff; D J Werring; M M Brown; J C Mason; R J Simister; H R Jäger Journal: Brain Commun Date: 2022-06-20