Rheumatology International

, Volume 33, Issue 12, pp 3073–3077

Acute cerebral vasculopathy in systemic sclerosis

Short Communication

DOI: 10.1007/s00296-012-2614-y

Cite this article as:
Faucher, B., Granel, B. & Nicoli, F. Rheumatol Int (2013) 33: 3073. doi:10.1007/s00296-012-2614-y


Systemic sclerosis is an autoimmune disease characterized by skin and deep organ fibrosis and obliterative microvasculopathy. Cerebral involvement is currently not recognized as a manifestation of the disease, although several morphologic and functional studies suggested a frequent cerebral involvement in systemic sclerosis. We report a new case of acute cerebral vasculopathy in a patient suffering from systemic sclerosis together with five historical cases identified through a literature review. Cerebral acute vasculopathy most often revealed the disease. Affected patients suffered often from limited or diffuse cutaneous systemic sclerosis. Reversibility of arterial lesions, absence of specific histologic findings, and association with severe peripheral vascular involvement plead for a major role of vasospasm. However, the apparent efficacy of immunosuppressive treatments suggests an association with inflammatory or immune mechanisms. Awareness should be raised because of the severity of the disease, the risk of relapse, and the possible occurrence early in the course of systemic sclerosis.


Scleroderma CREST syndrome Central nervous system vasculitis Stroke Angiography Intracranial vasospasm 

Copyright information

© Springer-Verlag Berlin Heidelberg 2012

Authors and Affiliations

  • Benoit Faucher
    • 1
    • 2
  • Brigitte Granel
    • 1
    • 2
  • Francois Nicoli
    • 3
  1. 1.Aix-Marseille UniversitéMarseilleFrance
  2. 2.Internal Medicine Department, Assistance Publique des Hôpitaux de Marseille (APHM), Service de Médecine interne, Hôpital NordChemin des BourrelyMarseille Cedex 20France
  3. 3.Neurovascular Unit, Assistance Publique des Hôpitaux de Marseille (APHM), Hôpital de La TimoneService de NeurovasculaireMarseilleFrance

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