ARTHRITIS & RHEUMATOLOGY Vol. 66, No. 5, May 2014, pp 1315–1326 DOI 10.1002/art.38340 © 2014, American College of Rheumatology Primary Angiitis of the Central Nervous System Description of the First Fifty-Two Adults Enrolled in the French Cohort of Patients With Primary Vasculitis of the Central Nervous System Hubert de Boysson, 1 Mathieu Zuber, 2 Olivier Naggara, 3 Jean-Philippe Neau, 4 Franc ¸oise Gray, 5 Marie-Germaine Bousser, 5 Isabelle Crassard, 5 Emmanuel Touze ´, 6 Pierre-Olivier Couraud, 7 Philippe Kerschen, 8 Catherine Oppenheim, 3 Olivier Detante, 9 Anthony Faivre, 10 Nicolas Gaillard, 11 Caroline Arquizan, 12 Boris Bienvenu, 13 Antoine Ne ´el, 14 Loı ¨c Guillevin, 15 and Christian Pagnoux, 15 for the French Vasculitis Study Group and the French NeuroVascular Society Objective. To describe characteristics and out- comes of a multicenter cohort of patients diagnosed as having primary angiitis of the central nervous system (PACNS). Methods. In 2010, we initiated a cohort study of adults diagnosed as having PACNS <15 years ago and with followup of >6 months (unless they died earlier of biopsy-proven PACNS). Its first analysis was planned at 2 years. Multidisciplinary investigators verified that appropriate investigations were done and excluded pa- tients with possible alternative diagnoses. We analyzed patient demographics and symptoms, laboratory, radio- graphic, and histologic findings, and treatments. Stud- ied outcomes included treatment response(s), relapse, death, and disability. Results. We included 52 patients (30 males; me- dian age at diagnosis 43.5 years [range 18–79 years]) in whom PACNS was diagnosed between 1996 and 2012. Nineteen (61%) of 31 patients who had undergone brain biopsy had histologic vasculitis (biopsy-proven PACNS), while the other 12 patients had normal or noncontributive biopsy samples. An additional 21 pa- tients had signs suggestive of PACNS on conventional cerebral angiography. All but 1 patient received cortico- steroids, and 44 patients received cyclophosphamide (CYC). After a median followup of 35 months (range 2–148 months) postdiagnosis (1 patient with biopsy- proven PACNS died 2 months after diagnosis), 32 patients responded to treatment with improved modi- fied Rankin scale scores, 4 patients (8%) did not re- spond, 14 patients (27%) had relapse of their disease at least once, and 3 patients (6%) died (1 patient after a relapse). Relapse was more common in patients with than in those without meningeal gadolinium enhance- Supported by an institutional grant from the French Ministry of Health (COVAC, 2009 PHRC 08017). 1 Hubert de Boysson, MD, MSc: Ho ˆpital Cochin, AP-HP, and Universite ´ Paris-Descartes, Paris, France, and Centre Hospitalier Universitaire (CHU) de Caen, Caen, France; 2 Mathieu Zuber, MD, PhD: Groupe Hospitalier Saint-Joseph, AP-HP, and Universite ´ Paris- Descartes, Paris, France; 3 Olivier Naggara, MD, PhD, Catherine Oppenheim, MD, PhD: Universite ´ Paris-Descartes, Sorbonne Paris Cite ´, INSERM UMR S894, and Ho ˆpital St. Anne, AP-HP, Paris, France; 4 Jean-Philippe Neau, MD, PhD: CHU La Mile ´trie, Poitiers, France; 5 Franc ¸oise Gray, MD, PhD, Marie-Germaine Bousser, MD, PhD, Isabelle Crassard, MD: Ho ˆpital Lariboisie `re, AP-HP, and Uni- versite ´ Paris-Diderot, Paris, France; 6 Emmanuel Touze ´, MD, PhD: Ho ˆpital St. Anne, AP-HP, and Universite ´ Paris-Descartes, Paris, France; 7 Pierre-Olivier Couraud, PhD: Institut Cochin, Paris, France; 8 Philippe Kerschen, MD: CHU Henri-Mondor, AP-HP, and Univer- site ´ Paris-Est Cre ´teil, Cre ´teil, France; 9 Olivier Detante, MD, PhD: CHU de Grenoble, Grenoble, France; 10 Anthony Faivre, MD: Ho ˆpital d’Instruction des Arme ´es St. Anne, Toulon, France; 11 Nicolas Gaillard, MD: Centre Hospitalier de Perpignan, Perpignan, France; 12 Caroline Arquizan, MD: Ho ˆpital Gui de Chauliac and Universite ´ Montpellier, Montpellier, France; 13 Boris Bienvenu, MD, PhD: CHU de Caen, Caen, France; 14 Antoine Ne ´el, MD: CHU Ho ˆtel-Dieu, Nantes, France; 15 Loı ¨c Guillevin, MD, Christian Pagnoux, MD, MPH, MSc (current address: Mount Sinai Hospital, University Health Net- work, and University of Toronto, Toronto, Ontario, Canada): Ho ˆpital Cochin, AP-HP, and Universite ´ Paris-Descartes, Paris, France. Dr. Zuber has received speaking fees and/or honoraria from Bayer, Sanofi, Bristol-Myers Squibb, and Euthe ´rapie (less than $10,000 each). Dr. Pagnoux has received consulting fees, speaking fees, and/or honoraria from Hoffmann-La Roche (less than $10,000). Address correspondence to Christian Pagnoux, MD, MPH, MSc, Mount Sinai Hospital, Division of Rheumatology, 60 Murray Street, R 2-222, Toronto, Ontario M5T 3L9, Canada. E-mail: cpagnoux@mtsinai.on.ca. Submitted for publication March 11, 2013; accepted in revised form December 26, 2013. 1315