Adult primary angiitis of the central nervous system: isolated small-vessel vasculitis represents distinct disease pattern

Rheumatology (Oxford). 2017 Mar 1;56(3):439-444. doi: 10.1093/rheumatology/kew434.


Objectives: We aimed to identify whether presentations and outcomes in adult patients with isolated small-vessel primary angiitis of the CNS (PACNS) would differ from other patients with large/medium-vessel involvement.

Methods: In the French PACNS cohort, we compared the characteristics, treatments and outcomes of patients with isolated small-vessel disease (normal CT, MR and/or conventional angiograms, brain biopsy positive for vasculitis) with other patients who had large/medium-vessel involvement (vessel abnormalities on CT, MR or conventional angiograms). A good functional outcome was defined as a modified Rankin scale ⩽2 at last follow-up, regardless of the occurrence of relapse.

Results: Among the 102 patients in the cohort, 26 (25%) had isolated small-vessel PACNS, whereas the 76 others demonstrated large/medium-vessel involvement. Patients with isolated small-vessel PACNS had more seizures (P < 0.0001), cognitive (P = 0.02) or consciousness impairment (P = 0.03) and more dyskinesias (P = 0.002) but less focal deficits (P = 0.0002) than other PACNS patients. They also had more abnormal cerebrospinal fluid analysis (P = 0.008) and gadolinium enhancements on MRI (P = 0.001) but less frequent acute ischaemic lesions (P < 0.0001) than patients with large/medium-vessel involvement. Treatments and modified Rankin scale at last follow-up did not differ between groups. Thirty-two (31%) patients relapsed; 14 (54%) with isolated small-vessel PACNS vs 18 (24%) with large/medium-vessel involvement (P = 0.004). Eight patients died, with no difference between the groups (P = 0.97).

Conclusion: In our cohort, adult patients with isolated small-vessel PACNS presented some distinct disease features and relapsed more often than other PACNS patients who had large/medium-vessel involvement. Functional outcomes and mortality did not differ.

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

Publication types

  • Observational Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Biopsy
  • Brain / pathology
  • Case-Control Studies
  • Cerebral Angiography
  • Cerebral Small Vessel Diseases / complications
  • Cerebral Small Vessel Diseases / diagnostic imaging*
  • Cerebral Small Vessel Diseases / pathology
  • Cerebral Small Vessel Diseases / physiopathology
  • Cognitive Dysfunction / etiology
  • Cohort Studies
  • Consciousness Disorders / etiology
  • Dyskinesias / etiology
  • Humans
  • Magnetic Resonance Imaging
  • Middle Aged
  • Retrospective Studies
  • Seizures / etiology
  • Tomography, X-Ray Computed
  • Vasculitis, Central Nervous System / complications
  • Vasculitis, Central Nervous System / diagnostic imaging*
  • Vasculitis, Central Nervous System / pathology
  • Vasculitis, Central Nervous System / physiopathology
  • Young Adult

Supplementary concepts

  • Primary angiitis of the central nervous system