Background/Purpose: Primary angiitis of the central nervous system (PACNS) is rare and only 1 pediatric and 1 adult large cohorts including >30 PACNS patients have been published, both from North America. The French Vasculitis Study Group, in collaboration with the French NeuroVascular Society and Internal Medicine Society, initiated a multicenter cohort study to describe a new and large adult PACNS population.
Methods: Patients with PACNS, diagnosed recently or within the past 20 years, with follow-up >6 months after diagnosis (unless they died earlier) and negative diagnostic work-ups for alternative diagnoses or secondary CNS vasculitis, were eligible for inclusion. A multidisciplinary committee (internists, neuroradiologists, neurologists and pathologists) systematically analyzed all medical charts to validate patients’ diagnoses, characteristics and outcomes. Poor outcomes were defined as no disease control and/or PACNS relapse or death.
Results: Eighteen months after cohort initiation, 52 patients (30 M/22 F; median age at diagnosis, 43.5 [18–79] yr) from 21 French hospitals have been included. Thirty-one (60%) underwent brain biopsy, which showed vasculitis features in 19 (37% biopsy-proven PACNS). All 12 patients with normal or non-contributive biopsies and the remaining 21 without brain biopsies, had persistent (>6 months) conventional cerebral angiography suggesting PACNS (33 [63%] angiography-diagnosed PACNS). The most frequent initial manifestations were focal neurological deficits (83%), headaches (54%), cognitive impairment (35%), aphasia (35%) and/or seizures (33%). Compared to biopsy-proven PACNS, angiography-diagnosed PACNS patients had more frequent focal neurological deficits (p=0.004) and bilateral infarctions on MRI (p=0.04) but less frequent encephalitic manifestations, like seizures or cognitive disorders (p=0.003 and 0.04, respectively). Twenty-two (52%) of the 42 MRI with gadolinium injection showed enhanced-parenchymatous/meningeal (22/10) lesions. All but 1 patient received corticosteroids (CS) and 44 cyclophosphamide (CYC). At the time of this analysis (median follow-up, 35 [6–148] mo), 3 (6%) patients had died; 20 (51%) of the 49 survivors had poor outcomes (13 [27%] relapsed, 7 [13%] had no disease control) and 40 (77%) had some persistent neurological damage. Patients with gadolinium leptomeningeal enhancement responded promptly to therapy but relapsed more often (80%) than those without (14%; p<0.0001). Multivariable analysis retained only intraparenchymatous and meningeal gadolinium uptakes at diagnosis as independent predictors of poor prognosis or relapse (HR=2.45 [95% CI, 0.99–6.08] and 1.88 [95% CI, 0.80–4.46], respectively).
Conclusion: In this PACNS cohort, more than half the patients responded to CS–CYC and had good outcomes at 3 yr, but neurological damage was frequent. Leptomeningeal and intraparenchymatous enhancements on MRI were associated with a poorer outcome. Comparisons of the different ongoing cohorts should help identify subgroups with poorer outcomes.
Disclosure:
H. de Boysson,
None;
M. Zuber,
None;
O. Naggara,
None;
J. P. Neau,
None;
F. Gray,
None;
M. G. Bousser,
None;
I. Crassard,
None;
E. Touze,
None;
P. O. Couraud,
None;
P. Kerschen,
None;
C. Oppenheim,
None;
O. Detante,
None;
A. Faivre,
None;
N. Gaillard,
None;
C. Arquizan,
None;
B. Bienvenu,
None;
A. Neel,
None;
L. Guillevin,
None;
C. Pagnoux,
None;
« Back to 2012 ACR/ARHP Annual Meeting
ACR Meeting Abstracts - https://acrabstracts.org/abstract/primary-angiitis-of-the-central-nervous-system-description-of-the-first-52-adult-patients-enrolled-in-the-french-covac-cohort/