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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Giant cell arteritis &#40;GCA&#41; is a systemic medium and large vessel vasculitis&#44; with a wide spectrum of presentations&#44; most frequently secondary to involvement of the cranial arteries&#44; the typical symptoms being headache&#44; jaw claudication&#44; visual loss and stroke&#46; Extracranial arterial involvement is described in 10&#8211;15&#37; of cases&#44;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">1&#44;2</span></a> usually presenting as upper or lower extremity claudication&#44; and rarely with acute critical ischemia as the primary finding&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">We report a severe and sudden case of acute critical ischemia of the arms with digital necrosis as the presenting features of GCA&#44; successfully treated by salvage revascularization surgery&#46; Only large vessels were involved&#44; mainly the subclavian and axillary arteries&#44; with no involvement of the carotid or cranial arteries&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Case report</span><p id="par0015" class="elsevierStylePara elsevierViewall">An 80-year-old Caucasian woman with a history of hypertension described proximal muscle pain in the arms with morning stiffness of the shoulders and neck&#44; for the past eight months&#46; In the previous two months&#44; she had developed worsening bilateral arm claudication&#44; severe pain with restricted mobility&#44; cold extremities and digital necrosis&#46; She had no palpable radial or cubital pulses and no measurable blood pressure&#46; There were no clinical signs of cranial or lower limb artery involvement and no constitutional symptoms were reported&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Investigation</span><p id="par0020" class="elsevierStylePara elsevierViewall">The patient presented normochromic anemia&#44; elevated erythrocyte sedimentation rate &#40;ESR&#41; &#40;120 mm&#47;h&#41; and C-reactive protein &#40;CRP&#41; &#40;123 mg&#47;l&#41;&#44; and negative infectious and autoimmune workup&#46; Contrast computed tomography angiography &#40;CTA&#41; in the arterial phase &#40;<a class="elsevierStyleCrossRefs" href="#fig0005">Figures 1&#8211;3</a>&#41; revealed concentric wall thickening of the aorta extending to the aortic arch branches&#44; particularly the subclavian and axillary arteries&#44; which were severely stenotic with areas of bilateral luminal occlusion&#44; and no involvement of the carotid branches&#59; an aneurysm of the ascending aorta 47 mm in diameter was also detected&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0025" class="elsevierStylePara elsevierViewall">The patient immediately started corticosteroid therapy &#40;prednisone 1 mg&#47;kg daily&#41; and aspirin &#40;100 mg daily&#41;&#44; but there was incomplete resolution of the symptoms with progression of digital ulceration&#44; necrosis and paresthesia&#44; features of acute critical ischemia&#46; Surgical revascularization using a bilateral carotid-humeral bypass was accordingly performed&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">After surgery&#44; corticosteroid therapy was maintained &#40;slowly tapered to 40 mg&#47;day&#41;&#44; and at the six-month follow-up appointment&#44; there was no arm claudication and on physical examination there were palpable radial and cubital pulses and systolic blood pressure of 135 mmHg&#46; Laboratory exams revealed a sustained reduction of serum inflammatory markers&#44; and follow-up CTA &#40;<a class="elsevierStyleCrossRef" href="#fig0020">Figure 4</a>&#41; showed adequate permeability of the bypassed vessels and stability of the aneurysm&#46; The histological result of the biopsied artery showed a transmural inflammatory infiltrate&#46;</p><elsevierMultimedia ident="fig0020"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Discussion</span><p id="par0035" class="elsevierStylePara elsevierViewall">Giant cell arteritis is a chronic inflammatory systemic disease&#44; affecting medium and large arteries&#46; The most common symptoms arise from the preferential involvement of the cranial arteries&#44; especially branches of the external carotid such as the temporal or ophthalmic artery&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">In 10&#8211;15&#37; of patients there is involvement of aortic arch branches&#44; particularly the subclavian and axillary arteries&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">1&#44;2</span></a> This involvement is rarely the presenting feature of the disease&#44; usually resulting from progression or relapse after discontinuation or tapering of corticosteroid therapy&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">1</span></a> One study reported a period of eleven months between diagnosis of temporal artery GCA and the appearance of symptoms resulting from involvement of the upper limb arteries&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">3</span></a> The most common symptom of such involvement is arm claudication and pain &#40;68&#37;&#41;&#44; but in rare cases it can evolve into critical ischemia&#44; digital ulceration and gangrene&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">1</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">Unlike for temporal artery GCA&#44; there are no validated independent criteria for the diagnosis of GCA with involvement of the upper limb arteries&#44; so this relies on clinical suspicion&#44; supported by laboratory and imaging studies&#46; An ESR of over 50 mm&#47;h was one of the first inflammatory markers associated with the disease&#44; and is part of the American College of Rheumatology criteria for GCA diagnosis &#40;86&#46;5&#37; sensitivity and 47&#46;7&#37; specificity&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">4</span></a> Some studies of GCA with upper or lower limb involvement found lower ESR values compared with temporal artery GCA&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">1</span></a> CRP level was observed to be a more sensitive marker of disease activity&#44; and is especially useful to access disease relapse&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">2</span></a> The European League Against Rheumatism guidelines for the management of GCA recommend a temporal artery biopsy in all patients whenever a diagnosis is suspected &#40;level of evidence 3&#44; strength of recommendation C&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">5</span></a> Nevertheless&#44; Brack et al&#46; reported negative temporal artery biopsy in 42&#37; of patients with large-vessel GCA&#46;<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">6</span></a> Assie et al&#46; also observed a lower rate of positive temporal artery biopsy in patients with temporal artery GCA compared to those with involvement of upper and lower limb arteries &#40;69&#37; vs&#46; 95&#37;&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">7</span></a> Biopsy of the affected artery is generally only available if revascularization procedures take place&#44; so the diagnosis and treatment strategy of large-vessel GCA are frequently decided before histological results are available&#46; The biopsy may reveal transmural inflammatory infiltrate with fragmentation of the elastic lamina with or without giant cells in a focal and segmental distribution&#46; False negatives are possible depending on the sample size &#40;ideally 1&#8211;2 cm&#41;&#44; duration of previous corticosteroid therapy and the presence of skip lesions&#46; CTA is an excellent non-invasive exam to assess vessel wall thickness and regularity&#44; also revealing luminal defects such as stenosis&#44; occlusion&#44; dilation and aneurysms&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">As a systemic inflammatory disease the first-line treatment advocated in most guidelines is corticosteroid therapy &#40;1 mg&#47;kg&#47;day&#44; maximum 60 mg daily&#41;&#44; with some authors suggesting high-dose methylprednisolone bolus before oral corticosteroid therapy&#46; Nonetheless&#44; there is scant evidence supporting this strategy in GCA patients with upper limb artery involvement&#46; Our patient immediately started prednisone 60 mg&#47;d&#44; and showed a slight clinical improvement within days&#46; However&#44; the bilateral occlusion of both subclavian arteries was remarkably severe&#44; and progression to critical ischemia was inevitable&#46; The vascular surgery team performed a bilateral carotid-humeral bypass as a salvage revascularization procedure to assure blood flow and limb viability&#46; She continued corticosteroid therapy with slow dose tapering&#46; Surgical intervention in such patients is rarely indicated because of the chronic nature of the disease&#46; Nonetheless&#44; when the occlusion is abrupt there is no time to develop collateral circulation to assure tissue viability and the response to corticosteroids can be inadequate&#44; especially in cases of large vessel involvement&#46;<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">8</span></a> There are some small series reporting the use of balloon angioplasty as a minimally invasive revascularization procedure&#44; with excellent immediate results but with 50&#37; recurrence of stenosis after a median of five months&#46;<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">9</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">Follow-up in these patients is mainly based on the response to corticosteroid therapy&#44; as assessed by clinical evolution&#44; reduction in inflammatory markers and&#44; in some cases&#44; imaging studies&#46; A treatment course of one to two years is often required&#44; but some patients have a more chronic or relapsing course of the disease and require longer treatment&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Ethical disclosures</span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Protection of human and animal subjects</span><p id="par0060" class="elsevierStylePara elsevierViewall">The authors declare that no experiments were performed on humans or animals for this study&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Confidentiality of data</span><p id="par0065" class="elsevierStylePara elsevierViewall">The authors declare that no patient data appear in this article&#46;</p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Right to privacy and informed consent</span><p id="par0070" class="elsevierStylePara elsevierViewall">The authors declare that no patient data appear in this article&#46;</p></span></span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">Conflicts of interest</span><p id="par0075" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare&#46;</p></span></span>"
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        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Giant cell arteritis &#40;GCA&#41; is a systemic large vessel vasculitis&#44; with extracranial arterial involvement described in 10&#8211;15&#37; of cases&#44; usually affecting the aorta and its branches&#46; Patients with GCA are more likely to develop aortic aneurysms&#44; but these are rarely present at the time of the diagnosis&#46;</p><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">We report the case of an 80-year-old Caucasian woman&#44; who reported proximal muscle pain in the arms with morning stiffness of the shoulders for eight months&#46; In the previous two months&#44; she had developed worsening bilateral arm claudication&#44; severe pain&#44; cold extremities and digital necrosis&#46; She had no palpable radial pulses and no measurable blood pressure&#46;</p><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">The patient had normochromic anemia&#44; erythrocyte sedimentation rate of 120 mm&#47;h&#44; and a negative infectious and autoimmune workup&#46; Computed tomography angiography revealed concentric wall thickening of the aorta extending to the aortic arch branches&#44; particularly the subclavian and axillary arteries&#44; which were severely stenotic&#44; with areas of bilateral occlusion and an aneurysm of the ascending aorta &#40;47 mm&#41;&#46; Despite corticosteroid therapy there was progression to acute critical ischemia&#46; She accordingly underwent surgical revascularization using a bilateral carotid-humeral bypass&#46; After surgery&#44; corticosteroid therapy was maintained and at six-month follow-up she was clinically stable with reduced inflammatory markers&#46;</p><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">GCA&#44; usually a chronic benign vasculitis&#44; presented exceptionally in this case as acute critical upper limb ischemia&#44; resulting from a massive inflammatory process of the subclavian and axillary arteries&#44; treated with salvage surgical revascularization&#46;</p></span>"
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        "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">A arterite de c&#233;lulas gigantes &#40;ACG&#41; &#233; uma vasculite de grandes vasos&#44; com envolvimento extracraniano em 10-15&#37; dos casos&#44; afetando preferencialmente a aorta e os seus ramos&#46; Os aneurismas da aorta ascendente s&#227;o mais frequentes em doentes com ACG&#44; mas s&#227;o raros no momento do diagn&#243;stico&#46;</p><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Apresentamos o caso de uma doente&#44; caucasiana&#44; com 80 anos de idade&#44; que descrevia dor muscular nos membros superiores&#44; com rigidez matinal dos ombros&#44; desde h&#225; oito meses&#46; Nos &#250;ltimos dois meses referia claudica&#231;&#227;o intermitente de agravamento progressivo&#44; dor intensa&#44; extremidades frias e o aparecimento de &#250;lceras digitais&#46; Ao exame objetivo n&#227;o se palpavam pulsos radiais ou cubitais&#44; nem se registavam valores de press&#227;o arterial&#46;</p><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Laboratorialmente&#44; destacava-se anemia normoc&#237;ticanormocr&#243;mica&#44; VS de 120 mm&#47;h e estudo autoimune e infeccioso negativos&#46; A tomografia computorizada com contraste revelou espessamento conc&#234;ntrico da parede da aorta e dos seus ramos&#44; notoriamente art&#233;ria subcl&#225;via e axilar&#44; que apresentavam &#225;reas de estenose e oclus&#227;o bilaterais e uma dilata&#231;&#227;o aneurism&#225;tica da aorta ascendente &#40;47 mm&#41;&#46; Apesar da institui&#231;&#227;o imediata de corticoterapia&#44; verificou-se uma progress&#227;o para isquemia cr&#237;tica aguda dos membros superiores&#46; Consequentemente&#44; foi submetida com sucesso a revasculariza&#231;&#227;o cir&#250;rgica atrav&#233;s de um <span class="elsevierStyleItalic">bypass</span> car&#243;tido-umeral&#46; A doente manteve corticoterapia e ao 6&#46;&#176; m&#234;s de <span class="elsevierStyleItalic">follow-up</span> encontrava-se clinicamente est&#225;vel&#44; com regress&#227;o dos par&#226;metros inflamat&#243;rios&#46;</p><p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">A ACG &#233; habitualmente descrita como uma patologia indolente e benigna&#46; No entanto&#44; apresentou-se neste caso como uma isquemia cr&#237;tica aguda dos membros superiores&#44; secund&#225;ria &#224; intensa resposta inflamat&#243;ria das art&#233;rias subcl&#225;vias e axilares&#44; obrigando &#224; revasculariza&#231;&#227;o cir&#250;rgica&#46;</p></span>"
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                      "titulo" => "Atteintes art&#233;rielles des membres sup&#233;rieurs et inf&#233;rieurs au cours de la maladie de Horton"
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                      "titulo" => "Polymyalgia rheumatica and giant-cell arteritis"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:3 [
                            0 => "C&#46; Salvarani"
                            1 => "F&#46; Cantini"
                            2 => "L&#46; Boiardi"
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                    0 => array:2 [
                      "doi" => "10.1056/NEJMra011913"
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                0 => array:2 [
                  "contribucion" => array:1 [
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                      "titulo" => "Subclavian and axillary involvement in temporal arteritis and polymyalgia rheumatica"
                      "autores" => array:1 [
                        0 => array:2 [
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                          "autores" => array:3 [
                            0 => "J&#46;P&#46; Ninet"
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                0 => array:2 [
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                      "titulo" => "The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis"
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                        0 => array:2 [
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                          "etal" => true
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Case report
Acute upper limb ischemia, a rare presentation of giant cell arteritis
Isquemia crítica dos membros superiores, uma apresentação rara de arterite de células gigantes
Luís Almeida-Moraisa,
Corresponding author
lmmorais88@gmail.com

Corresponding author.
, Sofia Galegob, Nélia Marquesb, Tiago Packb, Hugo Rodriguesc, Rodolfo Abreuc, Leonor Vasconcelosc, Hugo Marquesd, António Sousa Guerreirob
a Serviço de Cardiologia, Centro Hospitalar de Lisboa Central, EPE Hospital de Santa Marta, Lisboa, Portugal
b Unidade Funcional Medicina IV, Centro Hospitalar de Lisboa Central, EPE Hospital de Santa Marta, Lisboa, Portugal
c Serviço de Cirurgia Vascular, Centro Hospitalar de Lisboa Central, EPE Hospital de Santa Marta, Lisboa, Portugal
d Serviço de Radiologia, Centro Hospitalar de Lisboa Central, EPE Hospital de Santa Marta, Lisboa, Portugal
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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Giant cell arteritis &#40;GCA&#41; is a systemic medium and large vessel vasculitis&#44; with a wide spectrum of presentations&#44; most frequently secondary to involvement of the cranial arteries&#44; the typical symptoms being headache&#44; jaw claudication&#44; visual loss and stroke&#46; Extracranial arterial involvement is described in 10&#8211;15&#37; of cases&#44;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">1&#44;2</span></a> usually presenting as upper or lower extremity claudication&#44; and rarely with acute critical ischemia as the primary finding&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">We report a severe and sudden case of acute critical ischemia of the arms with digital necrosis as the presenting features of GCA&#44; successfully treated by salvage revascularization surgery&#46; Only large vessels were involved&#44; mainly the subclavian and axillary arteries&#44; with no involvement of the carotid or cranial arteries&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Case report</span><p id="par0015" class="elsevierStylePara elsevierViewall">An 80-year-old Caucasian woman with a history of hypertension described proximal muscle pain in the arms with morning stiffness of the shoulders and neck&#44; for the past eight months&#46; In the previous two months&#44; she had developed worsening bilateral arm claudication&#44; severe pain with restricted mobility&#44; cold extremities and digital necrosis&#46; She had no palpable radial or cubital pulses and no measurable blood pressure&#46; There were no clinical signs of cranial or lower limb artery involvement and no constitutional symptoms were reported&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Investigation</span><p id="par0020" class="elsevierStylePara elsevierViewall">The patient presented normochromic anemia&#44; elevated erythrocyte sedimentation rate &#40;ESR&#41; &#40;120 mm&#47;h&#41; and C-reactive protein &#40;CRP&#41; &#40;123 mg&#47;l&#41;&#44; and negative infectious and autoimmune workup&#46; Contrast computed tomography angiography &#40;CTA&#41; in the arterial phase &#40;<a class="elsevierStyleCrossRefs" href="#fig0005">Figures 1&#8211;3</a>&#41; revealed concentric wall thickening of the aorta extending to the aortic arch branches&#44; particularly the subclavian and axillary arteries&#44; which were severely stenotic with areas of bilateral luminal occlusion&#44; and no involvement of the carotid branches&#59; an aneurysm of the ascending aorta 47 mm in diameter was also detected&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0025" class="elsevierStylePara elsevierViewall">The patient immediately started corticosteroid therapy &#40;prednisone 1 mg&#47;kg daily&#41; and aspirin &#40;100 mg daily&#41;&#44; but there was incomplete resolution of the symptoms with progression of digital ulceration&#44; necrosis and paresthesia&#44; features of acute critical ischemia&#46; Surgical revascularization using a bilateral carotid-humeral bypass was accordingly performed&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">After surgery&#44; corticosteroid therapy was maintained &#40;slowly tapered to 40 mg&#47;day&#41;&#44; and at the six-month follow-up appointment&#44; there was no arm claudication and on physical examination there were palpable radial and cubital pulses and systolic blood pressure of 135 mmHg&#46; Laboratory exams revealed a sustained reduction of serum inflammatory markers&#44; and follow-up CTA &#40;<a class="elsevierStyleCrossRef" href="#fig0020">Figure 4</a>&#41; showed adequate permeability of the bypassed vessels and stability of the aneurysm&#46; The histological result of the biopsied artery showed a transmural inflammatory infiltrate&#46;</p><elsevierMultimedia ident="fig0020"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Discussion</span><p id="par0035" class="elsevierStylePara elsevierViewall">Giant cell arteritis is a chronic inflammatory systemic disease&#44; affecting medium and large arteries&#46; The most common symptoms arise from the preferential involvement of the cranial arteries&#44; especially branches of the external carotid such as the temporal or ophthalmic artery&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">In 10&#8211;15&#37; of patients there is involvement of aortic arch branches&#44; particularly the subclavian and axillary arteries&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">1&#44;2</span></a> This involvement is rarely the presenting feature of the disease&#44; usually resulting from progression or relapse after discontinuation or tapering of corticosteroid therapy&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">1</span></a> One study reported a period of eleven months between diagnosis of temporal artery GCA and the appearance of symptoms resulting from involvement of the upper limb arteries&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">3</span></a> The most common symptom of such involvement is arm claudication and pain &#40;68&#37;&#41;&#44; but in rare cases it can evolve into critical ischemia&#44; digital ulceration and gangrene&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">1</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">Unlike for temporal artery GCA&#44; there are no validated independent criteria for the diagnosis of GCA with involvement of the upper limb arteries&#44; so this relies on clinical suspicion&#44; supported by laboratory and imaging studies&#46; An ESR of over 50 mm&#47;h was one of the first inflammatory markers associated with the disease&#44; and is part of the American College of Rheumatology criteria for GCA diagnosis &#40;86&#46;5&#37; sensitivity and 47&#46;7&#37; specificity&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">4</span></a> Some studies of GCA with upper or lower limb involvement found lower ESR values compared with temporal artery GCA&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">1</span></a> CRP level was observed to be a more sensitive marker of disease activity&#44; and is especially useful to access disease relapse&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">2</span></a> The European League Against Rheumatism guidelines for the management of GCA recommend a temporal artery biopsy in all patients whenever a diagnosis is suspected &#40;level of evidence 3&#44; strength of recommendation C&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">5</span></a> Nevertheless&#44; Brack et al&#46; reported negative temporal artery biopsy in 42&#37; of patients with large-vessel GCA&#46;<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">6</span></a> Assie et al&#46; also observed a lower rate of positive temporal artery biopsy in patients with temporal artery GCA compared to those with involvement of upper and lower limb arteries &#40;69&#37; vs&#46; 95&#37;&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">7</span></a> Biopsy of the affected artery is generally only available if revascularization procedures take place&#44; so the diagnosis and treatment strategy of large-vessel GCA are frequently decided before histological results are available&#46; The biopsy may reveal transmural inflammatory infiltrate with fragmentation of the elastic lamina with or without giant cells in a focal and segmental distribution&#46; False negatives are possible depending on the sample size &#40;ideally 1&#8211;2 cm&#41;&#44; duration of previous corticosteroid therapy and the presence of skip lesions&#46; CTA is an excellent non-invasive exam to assess vessel wall thickness and regularity&#44; also revealing luminal defects such as stenosis&#44; occlusion&#44; dilation and aneurysms&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">As a systemic inflammatory disease the first-line treatment advocated in most guidelines is corticosteroid therapy &#40;1 mg&#47;kg&#47;day&#44; maximum 60 mg daily&#41;&#44; with some authors suggesting high-dose methylprednisolone bolus before oral corticosteroid therapy&#46; Nonetheless&#44; there is scant evidence supporting this strategy in GCA patients with upper limb artery involvement&#46; Our patient immediately started prednisone 60 mg&#47;d&#44; and showed a slight clinical improvement within days&#46; However&#44; the bilateral occlusion of both subclavian arteries was remarkably severe&#44; and progression to critical ischemia was inevitable&#46; The vascular surgery team performed a bilateral carotid-humeral bypass as a salvage revascularization procedure to assure blood flow and limb viability&#46; She continued corticosteroid therapy with slow dose tapering&#46; Surgical intervention in such patients is rarely indicated because of the chronic nature of the disease&#46; Nonetheless&#44; when the occlusion is abrupt there is no time to develop collateral circulation to assure tissue viability and the response to corticosteroids can be inadequate&#44; especially in cases of large vessel involvement&#46;<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">8</span></a> There are some small series reporting the use of balloon angioplasty as a minimally invasive revascularization procedure&#44; with excellent immediate results but with 50&#37; recurrence of stenosis after a median of five months&#46;<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">9</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">Follow-up in these patients is mainly based on the response to corticosteroid therapy&#44; as assessed by clinical evolution&#44; reduction in inflammatory markers and&#44; in some cases&#44; imaging studies&#46; A treatment course of one to two years is often required&#44; but some patients have a more chronic or relapsing course of the disease and require longer treatment&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Ethical disclosures</span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Protection of human and animal subjects</span><p id="par0060" class="elsevierStylePara elsevierViewall">The authors declare that no experiments were performed on humans or animals for this study&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Confidentiality of data</span><p id="par0065" class="elsevierStylePara elsevierViewall">The authors declare that no patient data appear in this article&#46;</p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Right to privacy and informed consent</span><p id="par0070" class="elsevierStylePara elsevierViewall">The authors declare that no patient data appear in this article&#46;</p></span></span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">Conflicts of interest</span><p id="par0075" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare&#46;</p></span></span>"
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        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Giant cell arteritis &#40;GCA&#41; is a systemic large vessel vasculitis&#44; with extracranial arterial involvement described in 10&#8211;15&#37; of cases&#44; usually affecting the aorta and its branches&#46; Patients with GCA are more likely to develop aortic aneurysms&#44; but these are rarely present at the time of the diagnosis&#46;</p><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">We report the case of an 80-year-old Caucasian woman&#44; who reported proximal muscle pain in the arms with morning stiffness of the shoulders for eight months&#46; In the previous two months&#44; she had developed worsening bilateral arm claudication&#44; severe pain&#44; cold extremities and digital necrosis&#46; She had no palpable radial pulses and no measurable blood pressure&#46;</p><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">The patient had normochromic anemia&#44; erythrocyte sedimentation rate of 120 mm&#47;h&#44; and a negative infectious and autoimmune workup&#46; Computed tomography angiography revealed concentric wall thickening of the aorta extending to the aortic arch branches&#44; particularly the subclavian and axillary arteries&#44; which were severely stenotic&#44; with areas of bilateral occlusion and an aneurysm of the ascending aorta &#40;47 mm&#41;&#46; Despite corticosteroid therapy there was progression to acute critical ischemia&#46; She accordingly underwent surgical revascularization using a bilateral carotid-humeral bypass&#46; After surgery&#44; corticosteroid therapy was maintained and at six-month follow-up she was clinically stable with reduced inflammatory markers&#46;</p><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">GCA&#44; usually a chronic benign vasculitis&#44; presented exceptionally in this case as acute critical upper limb ischemia&#44; resulting from a massive inflammatory process of the subclavian and axillary arteries&#44; treated with salvage surgical revascularization&#46;</p></span>"
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        "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">A arterite de c&#233;lulas gigantes &#40;ACG&#41; &#233; uma vasculite de grandes vasos&#44; com envolvimento extracraniano em 10-15&#37; dos casos&#44; afetando preferencialmente a aorta e os seus ramos&#46; Os aneurismas da aorta ascendente s&#227;o mais frequentes em doentes com ACG&#44; mas s&#227;o raros no momento do diagn&#243;stico&#46;</p><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Apresentamos o caso de uma doente&#44; caucasiana&#44; com 80 anos de idade&#44; que descrevia dor muscular nos membros superiores&#44; com rigidez matinal dos ombros&#44; desde h&#225; oito meses&#46; Nos &#250;ltimos dois meses referia claudica&#231;&#227;o intermitente de agravamento progressivo&#44; dor intensa&#44; extremidades frias e o aparecimento de &#250;lceras digitais&#46; Ao exame objetivo n&#227;o se palpavam pulsos radiais ou cubitais&#44; nem se registavam valores de press&#227;o arterial&#46;</p><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Laboratorialmente&#44; destacava-se anemia normoc&#237;ticanormocr&#243;mica&#44; VS de 120 mm&#47;h e estudo autoimune e infeccioso negativos&#46; A tomografia computorizada com contraste revelou espessamento conc&#234;ntrico da parede da aorta e dos seus ramos&#44; notoriamente art&#233;ria subcl&#225;via e axilar&#44; que apresentavam &#225;reas de estenose e oclus&#227;o bilaterais e uma dilata&#231;&#227;o aneurism&#225;tica da aorta ascendente &#40;47 mm&#41;&#46; Apesar da institui&#231;&#227;o imediata de corticoterapia&#44; verificou-se uma progress&#227;o para isquemia cr&#237;tica aguda dos membros superiores&#46; Consequentemente&#44; foi submetida com sucesso a revasculariza&#231;&#227;o cir&#250;rgica atrav&#233;s de um <span class="elsevierStyleItalic">bypass</span> car&#243;tido-umeral&#46; A doente manteve corticoterapia e ao 6&#46;&#176; m&#234;s de <span class="elsevierStyleItalic">follow-up</span> encontrava-se clinicamente est&#225;vel&#44; com regress&#227;o dos par&#226;metros inflamat&#243;rios&#46;</p><p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">A ACG &#233; habitualmente descrita como uma patologia indolente e benigna&#46; No entanto&#44; apresentou-se neste caso como uma isquemia cr&#237;tica aguda dos membros superiores&#44; secund&#225;ria &#224; intensa resposta inflamat&#243;ria das art&#233;rias subcl&#225;vias e axilares&#44; obrigando &#224; revasculariza&#231;&#227;o cir&#250;rgica&#46;</p></span>"
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