Acute cerebral vasculopathy in systemic sclerosis
| Autor: | Francois Nicoli, Benoit Faucher, Brigitte Granel |
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| Rok vydání: | 2012 |
| Předmět: |
CREST Syndrome
Adult medicine.medical_specialty Pathology Immunology Disease Scleroderma Rheumatology Fibrosis Internal medicine medicine Immunology and Allergy Humans Stroke Autoimmune disease Scleroderma Systemic business.industry Vasospasm medicine.disease Cerebrovascular Disorders Treatment Outcome Prednisone Female business Immunosuppressive Agents |
| Zdroj: | Rheumatology international. 33(12) |
| ISSN: | 1437-160X |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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