TY - JOUR
T1 - Giant cell arteritis restricted to the limb arteries
T2 - An overlooked clinical entity
AU - Berti, Alvise
AU - Campochiaro, Corrado
AU - Cavalli, Giulio
AU - Pepe, Gino
AU - Praderio, Luisa
AU - Sabbadini, Maria Grazia
AU - Dagna, Lorenzo
PY - 2015/4/1
Y1 - 2015/4/1
N2 - Objective: Giant cell arteritis (GCA) is a systemic vasculitis typically affecting temporal arteries. In at least 15% of cases, GCA also features inflammation of the aorta and its primary branches. Large-vessel inflammation restricted to proximal limb arteries in the absence of temporal and aortic involvement (Limb Restricted, LR) is rare and not well described in literature. Hence, we aim to characterize this neglected clinical entity. Methods: We describe a series of three cases of LR-GCA. All patients were older than 50. years, had increased erythrocyte sedimentation rate (ESR), normal cholesterol and triglycerides serum levels, negative temporal artery biopsy, suggestive F-18 fluorodeoxyglucose positron emission tomography (FDG-PET) findings, and responded to immunosuppressive therapy. We also reviewed all published cases of LR-GCA (76 cases), for a total of 79 patients. Results: Limb claudication was reported in 87% of the patients, and cranial symptoms and polymyalgia rheumatica in 20%. Constitutional symptoms were never reported. Median ESR levels were 66.5. mm/1. h. Upper and lower limb arteries were involved in 86% and 9% of the patients respectively, and the remaining 5% had simultaneous upper and lower limb vessel involvement. Conventional angiography was performed in 63% of the cases, color-doppler ultrasound in 20%, FDG-PET in 14%, and computed tomography angiography in 3%. Conclusion: If temporal biopsy and aortic imaging are negative for GCA in patients older than 50. years with bilateral limb claudication, elevated ESR, and suggestive vascular radiological findings, LR-GCA should be suspected. Upper limb arteries are more frequently involved. Since constitutional symptoms are typically absent in LR-GCA, differential diagnosis with atherosclerotic plaques may be challenging.
AB - Objective: Giant cell arteritis (GCA) is a systemic vasculitis typically affecting temporal arteries. In at least 15% of cases, GCA also features inflammation of the aorta and its primary branches. Large-vessel inflammation restricted to proximal limb arteries in the absence of temporal and aortic involvement (Limb Restricted, LR) is rare and not well described in literature. Hence, we aim to characterize this neglected clinical entity. Methods: We describe a series of three cases of LR-GCA. All patients were older than 50. years, had increased erythrocyte sedimentation rate (ESR), normal cholesterol and triglycerides serum levels, negative temporal artery biopsy, suggestive F-18 fluorodeoxyglucose positron emission tomography (FDG-PET) findings, and responded to immunosuppressive therapy. We also reviewed all published cases of LR-GCA (76 cases), for a total of 79 patients. Results: Limb claudication was reported in 87% of the patients, and cranial symptoms and polymyalgia rheumatica in 20%. Constitutional symptoms were never reported. Median ESR levels were 66.5. mm/1. h. Upper and lower limb arteries were involved in 86% and 9% of the patients respectively, and the remaining 5% had simultaneous upper and lower limb vessel involvement. Conventional angiography was performed in 63% of the cases, color-doppler ultrasound in 20%, FDG-PET in 14%, and computed tomography angiography in 3%. Conclusion: If temporal biopsy and aortic imaging are negative for GCA in patients older than 50. years with bilateral limb claudication, elevated ESR, and suggestive vascular radiological findings, LR-GCA should be suspected. Upper limb arteries are more frequently involved. Since constitutional symptoms are typically absent in LR-GCA, differential diagnosis with atherosclerotic plaques may be challenging.
KW - Brachial GCA
KW - Claudication FDG-PET
KW - Extracranial GCA
KW - Large-vessel GCA
KW - Limb-restricted GCA
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U2 - 10.1016/j.autrev.2014.12.005
DO - 10.1016/j.autrev.2014.12.005
M3 - Article
C2 - 25528219
AN - SCOPUS:84923007062
VL - 14
SP - 352
EP - 357
JO - Autoimmunity Reviews
JF - Autoimmunity Reviews
SN - 1568-9972
IS - 4
ER -