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.

Abstract

OBJECTIVE:

To describe characteristics and outcomes 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 patients with possible alternative diagnoses. We analyzed patient demographics and symptoms, laboratory, radiographic, and histologic findings, and treatments. Studied outcomes included treatment response(s), relapse, death, and disability.

RESULTS:

We included 52 patients (30 males; median 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 patients had signs suggestive of PACNS on conventional cerebral angiography. All but 1 patient received corticosteroids, 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 modified Rankin scale scores, 4 patients (8%) did not respond, 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 enhancements on magnetic resonance imaging (MRI) (8 of 10 [80%] versus 6 of 32 [19%]; P = 0.001) and more common in patients with than in those without seizures at diagnosis (8 of 17 [47%] versus 6 of 35 [17%]; P = 0.04).

CONCLUSION:

In this cohort of patients with PACNS, most patients received corticosteroids and CYC, and mortality was low. Patients with seizures at diagnosis or meningeal enhancements on MRI may be prone to relapse and require a different treatment strategy.

• Authors: Hubert de Boysson, Mathieu Zuber, Olivier Naggara, Jean-Philippe Neau, Françoise Gray, Marie-Germaine Bousser, Isabelle Crassard, Emmanuel Touzé, Pierre-Olivier Couraud, Philippe Kerschen, Catherine Oppenheim, Olivier Detante, Anthony Faivre, Nicolas Gaillard, Caroline Arquizan, Boris Bienvenu, Antoine Néel, Loïc Guillevin, Christian Pagnoux, French Vasculitis Study Group and the French NeuroVascular Society
• Journal: Arthritis & rheumatology (Hoboken, N.J.) (Arthritis Rheumatol) Vol. 66 Issue 5 Pg. 1315-26 (May 2014) ISSN: 2326-5205 [Electronic] United States
• PMID: 24782189 (Publication Type: Journal Article, Multicenter Study, Research Support, Non-U.S. Gov't)
• Copyright: Copyright © 2014 by the American College of Rheumatology.

Chemical References

• Adrenal Cortex Hormones
• Antirheumatic Agents
• Cyclophosphamide

Topics

• Adolescent
• Adrenal Cortex Hormones (therapeutic use)
• Adult
• Aged
• Antirheumatic Agents (therapeutic use)
• Biopsy
• Brain (pathology)
• Cohort Studies
• Cyclophosphamide (therapeutic use)
• Disability Evaluation
• Female
• Follow-Up Studies
• France (epidemiology)
• Humans
• Magnetic Resonance Imaging
• Male
• Middle Aged
• Recurrence
• Treatment Outcome
• Vasculitis, Central Nervous System (diagnosis, drug therapy, epidemiology)
• Young Adult