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Acute cerebral vasculopathy in systemic sclerosis

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Abstract

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.

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Acknowledgments

We thank Anne KASMAR, English language editorial assistant, for reviewing the manuscript.

Conflict of interest

The authors declare that they have no conflict of interest.

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Authors and Affiliations

  1. Aix-Marseille Université, Marseille, France

    Benoit Faucher & Brigitte Granel

  2. Internal Medicine Department, Assistance Publique des Hôpitaux de Marseille (APHM), Service de Médecine interne, Hôpital Nord, Chemin des Bourrely, 13915, Marseille Cedex 20, France

    Benoit Faucher & Brigitte Granel

  3. Neurovascular Unit, Assistance Publique des Hôpitaux de Marseille (APHM), Hôpital de La Timone, Service de Neurovasculaire, Marseille, France

    Francois Nicoli

Authors
  1. Benoit Faucher
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  2. Brigitte Granel
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  3. Francois Nicoli
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Correspondence to Benoit Faucher.

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Faucher, B., Granel, B. & Nicoli, F. Acute cerebral vasculopathy in systemic sclerosis. Rheumatol Int 33, 3073–3077 (2013). https://doi.org/10.1007/s00296-012-2614-y

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  • DOI: https://doi.org/10.1007/s00296-012-2614-y

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