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Erschienen in: Rheumatology International 12/2013

01.12.2013 | Short Communication

Acute cerebral vasculopathy in systemic sclerosis

verfasst von: Benoit Faucher, Brigitte Granel, Francois Nicoli

Erschienen in: Rheumatology International | Ausgabe 12/2013

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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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Metadaten
Titel
Acute cerebral vasculopathy in systemic sclerosis
verfasst von
Benoit Faucher
Brigitte Granel
Francois Nicoli
Publikationsdatum
01.12.2013
Verlag
Springer Berlin Heidelberg
Erschienen in
Rheumatology International / Ausgabe 12/2013
Print ISSN: 0172-8172
Elektronische ISSN: 1437-160X
DOI
https://doi.org/10.1007/s00296-012-2614-y

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