Central nervous system vasculitis secondary to systemic sclerosis

J Clin Neurosci. 2013 Aug;20(8):1168-70. doi: 10.1016/j.jocn.2012.09.022. Epub 2013 May 8.


Systemic sclerosis (SSc) or scleroderma is a connective tissue disease with a diverse array of clinical manifestations secondary to underlying fibrosis and autoimmunity. Central nervous system (CNS) impairment is uncommon in SSc. Here we report the fourth known patient with CNS vasculitis caused by SSc. In each previous report, the patient was a middle-aged to elderly female. Our patient was 24 years old at the time of presentation, significantly younger than the other reported patients. Importantly, our patient's rapidly progressive clinical course and poor response to immunosuppression have not been reported in patients with CNS vasculitis secondary to scleroderma. Although CNS vasculitis is extremely rare in SSc, our report suggests that clinicians should consider this diagnosis in the differential of SSc patients with neurologic impairment.

Keywords: Central nervous system; Scleroderma; Systemic sclerosis vasculitis.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Cerebral Angiography
  • Electroencephalography
  • Fatal Outcome
  • Female
  • Humans
  • Magnetic Resonance Imaging
  • Scleroderma, Systemic / complications*
  • Tomography, X-Ray Computed
  • Vasculitis, Central Nervous System / diagnosis
  • Vasculitis, Central Nervous System / drug therapy
  • Vasculitis, Central Nervous System / etiology*
  • Young Adult