title: Cerebral vasculopathy is associated with severe vascular manifestations in systemic sclerosis.
abstract: OBJECTIVE
  To investigate brain involvement in patients with systemic sclerosis (SSc).
  
  
  METHODS
  Sixty-three patients with SSc fulfilling the American College of Rheumatology and/or Leroy and Medsger criteria were retrospectively studied, including 30 (47.6%) with limited cutaneous and 27 (42.9%) with diffuse cutaneous SSc. Forty-one patients underwent computed tomography (CT) scan and magnetic resonance imaging (MRI) of the brain, 11 patients only CT scan, and the remaining 11 patients only MRI. Cerebral vasculopathy on MRI and CT scan was defined as absent or mild (score < 1), moderate (1 <or= score < 2), or severe (score >or= 2) on a 4-point scale (0 to 3).
  
  
  RESULTS
  Cerebral vasculopathy was identified on CT scan in 22 patients (moderate in 12 and severe in 10) and on MRI in 38 patients (moderate in 28 and severe in 10). Patients with severe cerebral vasculopathy seen on MRI were more likely to have pulmonary arterial hypertension (PAH; p = 0.003) and showed a tendency to have scleroderma renal crisis (SRC; p = 0.25, test for trend p = 0.097). A similar association was found between severe cerebral vasculopathy seen on CT scan and PAH (p = 0.026) or SRC (p = 0.04). After adjusting for age and hypertension, severe cerebral vasculopathy was still associated with increased risk of severe vascular manifestations [odds ratio (OR) 32, 95% confidence interval (CI) 3.45-297, p = 0.002 for CT scan; OR 26, 95% CI 1.71-394, p = 0.019 for MRI].
  
  
  CONCLUSIONS
  Severe cerebral vasculopathy is associated with severe vascular manifestations in SSc patients. SSc patients with severe vascular complications should undergo neuroradiological imaging assessment of brain involvement.
authors.affiliation: Descartes University, Faculty of Medicine, Department of Internal Medicine and French Reference Centre for Necrotizing Vasculitides and Systemic Sclerosis, Cochin Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), 75679 Paris Cedex 14, France.
authors.forename: Benjamin
authors.initials: B
authors.lastname: Terrier
authors.forename: Frédérique
authors.initials: F
authors.lastname: Charbonneau
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Touzé
authors.forename: Alice
authors.initials: A
authors.lastname: Berezne
authors.forename: Christian
authors.initials: C
authors.lastname: Pagnoux
authors.forename: Stéphane
authors.initials: S
authors.lastname: Silvera
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Meder
authors.forename: Loïc
authors.initials: L
authors.lastname: Guillevin
authors.forename: Catherine
authors.initials: C
authors.lastname: Oppenheim
authors.forename: Luc
authors.initials: L
authors.lastname: Mouthon
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001921:Brain / Q000098:blood supply* / Q000473:pathology*; D002561:Cerebrovascular Disorders / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000453:epidemiology; D007674:Kidney Diseases / Q000453:epidemiology; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D012189:Retrospective Studies; D012307:Risk Factors; D012595:Scleroderma, Systemic / Q000150:complications*; D014057:Tomography, X-Ray Computed; D055815:Young Adult