Document Detail

Lethal giant cell arteritis with multiple ischemic strokes despite aggressive immunosuppressive therapy.
MedLine Citation:
PMID:  20609853     Owner:  NLM     Status:  MEDLINE    
Two patients with giant cell arteritis (GCA) had a malignant course despite aggressive immunosuppressive therapy. A 63-year-old woman presented with symptoms of headache, jaw claudication, scalp paresthesia, and visual disturbances. A temporal artery biopsy showed GCA. While on prednisone, she suffered ischemic strokes, and serial cerebral angiograms demonstrated bilateral, severe and progressive narrowing of distal vertebral and internal carotid arteries. Despite escalating immunosuppressive therapies, she suffered more infarcts and eventually died. Postmortem examination of arteries showed no active inflammation. A 65-year-old man presented with extrapyramidal symptoms though no symptoms typical of GCA. Imaging showed multiple ischemic strokes. Because serial angiograms demonstrated findings similar to the first patient, he underwent temporal artery biopsy that showed GCA. He died 7 months after his presentation with complications of aggressive immunosuppressive therapy. These two patients confirm that GCA can follow a lethal course despite escalating immunosuppressive therapies. Our two patients were unique in that eventually both anterior and posterior circulations were involved bilaterally in a characteristic location where the arteries penetrate the dura. This pattern should always raise the possibility of GCA and, if confirmed, should prompt aggressive immunosuppressive therapy. The dismal outcomes despite this approach may suggest a non-inflammatory arteriopathy, as seen on necropsy in one of our patients. Such an arteriopathy may require novel therapies to be considered for this severe variant of GCA.
Christine Lu-Emerson; Melanie Walker; Bertrand R Huber; Basavaraj Ghodke; W T Longstreth; Sandeep P Khot
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2010-06-17
Journal Detail:
Title:  Journal of the neurological sciences     Volume:  295     ISSN:  1878-5883     ISO Abbreviation:  J. Neurol. Sci.     Publication Date:  2010 Aug 
Date Detail:
Created Date:  2010-07-08     Completed Date:  2011-01-18     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0375403     Medline TA:  J Neurol Sci     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  120-4     Citation Subset:  IM    
Copyright Information:
Copyright (c) 2010 Elsevier B.V. All rights reserved.
Department of Neurology, University of Washington, Seattle, WA 98104, USA.
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MeSH Terms
Brain Infarction / etiology
Cerebral Angiography
Disease Progression
Giant Cell Arteritis* / complications,  immunology,  therapy
Immunosuppression / methods*
Magnetic Resonance Imaging / methods
Middle Aged
Stroke* / complications,  immunology,  therapy

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