Isquemia de extremidades inferiores secundaria a arteritis de la arteria temporal

Giant cell arteritis is more common in women older than 60 years, is associated with systemic inflammation symptoms and mainly involves the aortic arch and cranial arteries, specially the temporal artery. Symptomatic lower extremity arterial stenosis or occlusion is uncommon and can lead to limb loss. We report a 73-year-old woman presenting with a one-month history of lower extremity intermittent claudication of sudden onset. She also complained of fever, malaise, headache and weight loss. A non-invasive vascular study showed moderate femoral popliteal occlusive disease, with and abnormal ankle-brachial index (0.68 and 0.83 on right and left sides, respectively). An angio-computed tomography showed thickening of the aortic wall and severe stenosis in both superficial femoral arteries. Steroidal treatment was started, and a temporal artery biopsy was performed confirming giant cell arteritis. Six weeks after steroid therapy the patient had a complete remission of symptoms. A serologic exacerbation was subsequently treated with a humanized monoclonal antibody against the interleukin-6 receptor Tocilizumab, obtaining long time remission.

Giant cell arteritis: a diagnostic challenge in an older adult / Arterite de células gigantes: um desafio diagnóstico em um idoso

Giant cell arteritis is a large-vessel vasculitis usually seen in older adults. The inflammatory process results in systemic, ophthalmic, and neurological lesions. It is difficult to diagnose in older adults and may present as a medical emergency. Here, we report the case of an 83-year-old woman who presented with bitemporal headache, jaw claudication, glossodynia, failure to thrive, and amaurosis fugax. The findings supported the hypothesis of giant cell arteritis. Despite receiving treatment, the patient died of an acute myocardial infarction. Headache in older adults raises the possibility of giant cell arteritis, especially when combined with an ophthalmic emergency. Many symptoms indicate the condition, but the diagnosis may be challenging, especially for the generalist physician.

A arterite de células gigantes é uma vasculite de grandes vasos geralmente observada em adultos mais velhos. O processo inflamatório resulta em lesões sistêmicas, oftalmológicas e neurológicas. É de difícil diagnose em adultos mais velhos e pode se apresentar como uma emergência médica. Apresentamos o caso de uma mulher de 83 anos que apresentou cefaleia bitemporal, claudicação da mandíbula, glossodinia, incapacidade de prosperar e amaurose fugaz. Os resultados apoiaram a hipótese de arterite de células gigantes. Apesar de receber tratamento, o paciente morreu por infarto agudo do miocárdio. Dor de cabeça em idosos aumenta a possibilidade de arterite de células gigantes, especialmente quando combinada com uma emergência oftalmológica. Muitos sintomas indicam a condição, mas o diagnóstico pode ser desafiador, especialmente para o médico generalista.

Annuloaortic ectasia and arteritis: clinical features, treatments of choice, and causative relations

Abstract The roles that aortitis plays in the development of annuloaortic ectasia (AAE) remain uncertain, while clinical features of AAE in arteritis are largely unknown. This study was designed to highlight the clinical features of AAE, the treatments of choice, and the causative relations between aortitis and AAE. The morphology of the aortic valve leaflets was normal in half of the patients, while the valves were thin and overstretched in the other half. Most patients had an aortic aneurysm. Half of the patients had severe aortic valve insufficiency, and one-quarter of them had dilation of the sinuses of Valsalva. Takayasu arteritis was prone to develop coronary artery lesions, whereas giant cell arteritis were not. Aortic branch lesions in Takayasu arteritis were stenotic or occlusive in 92.9% of the patients, while in giant cell arteritis, they were all dilated lesions. Most patients (94.7%) required surgical treatment with steroid therapy. However, long-term follow-up results showed a higher anastomotic dehiscence rate, particularly in patients with Takayasu arteritis. Further morphometric and pathological research on AAE in arteritis should be undertaken, and more feasible measures should be warranted for preventing postoperative anastomotic dehiscence.

Arteritis de células gigantes con compromiso de aorta y grandes vasos / Aortitis and large vessel involvement in giant cell arteritis

La arteritis de células gigantes (ACG) es una vasculitis sistémica de grandes vasos que no solo puede afectar a vasos craneales, si no que cada vez más frecuentemente se ha asociado a compromiso de vasos extracraneales. Este compromiso puede ser asintomático por lo que puede pasar inadvertido al estudio clínico básico y llevar a importantes complicaciones. Se presentan dos casos clínicos de ACG, ambos con aortitis y uno, además, con compromiso de grandes vasos del cayado aórtico y del cuello. Se discuten aspectos fisiopatológicos, clínicos y terapéuticos, con el objetivo de vislumbrar estrategias de abordaje de futuros pacientes con presentaciones similares.

Giant cell arteritis (GCA) is a age related, large vessels systemic vasculitis that not only affect cranial vessels, but is increasingly associated with extracranial vessel involvement. This extracranial involvement can be asymptomatic, so it can go unnoticed after basic clinical and laboratory study and lead to serious complications. We report two cases of GCA, both presenting aortitis and one of them presenting also involvement of aortic arch and of neck large vessels. We discuss physiopathologic, clinic and therapeutic aspects, with the aims of developing management strategies for the future patients with similar manifestations.

Arteritic Anterior Ischemic Optic Neuropathy Associated with Giant Cell Arteritis in an Elderly Korean Man

No abstract available.

An update of neurological manifestations of vasculitides and connective tissue diseases: a literature review / Atualização das manifestações neurológicas das vasculitides e das doenças do tecido conjuntivo: revisão de literatura

Vasculitides comprise a heterogeneous group of autoimmune disorders, occurring as primary or secondary to a broad variety of systemic infectious, malignant or connective tissue diseases. The latter occur more often but their pathogenic mechanisms have not been fully established. Frequent and varied central and peripheral nervous system complications occur in vasculitides and connective tissue diseases. In many cases, the neurological disorders have an atypical clinical course or even an early onset, and the healthcare professionals should be aware of them. The purpose of this brief review was to give an update of the main neurological disorders of common vasculitis and connective tissue diseases, aiming at accurate diagnosis and management, with an emphasis on pathophysiologic mechanisms.

As vasculites são um grupo heterogêneo de doenças autoimunes primárias ou secundárias a uma grande variedade de doenças infecciosas sistêmicas, malignas ou do tecido conjuntivo. Estas últimas são as que ocorrem com mais frequência, porém os mecanismos patogênicos ainda não foram plenamente determinados. Diversas e frequentes complicações do sistema nervoso central e periférico ocorrem nas vasculites e doenças do tecido conjuntivo. Em muitos casos, os distúrbios neurológicos têm evolução clínica atípica ou mesmo início precoce, ao que todos os profissionais de saúde devem estar cientes. O objetivo desta breve revisão foi atualizar os principais distúrbios neurológicos da vasculite comum e das doenças do tecido conjuntivo, visando ao diagnóstico e ao tratamento corretos, com ênfase nos mecanismos fisiopatológicos.

Silent Giant Cell Arteritis in an Elderly Korean Woman

Giant cell arteritis (GCA) is a rare disease among Asians. Arteritic anterior ischemic optic neuropathy, which accompanies GCA, has not yet been reported in Koreans. Diagnosis of GCA is difficult if typical symptoms other than visual loss are absent. Here, we report a case of an 83-year-old Korean woman presenting with sudden visual loss in both eyes (oculus uterque, OU). Her visual acuities included perception of light in the right eye (oculus dexter, OD) and perception of hand motion in the left eye (oculus sinister, OS). The results of the Hardy-Rand-Rittler test and Ishihara test showed total dyschromatopsia OU. The Goldmann perimetry test revealed a total field defect OD and paracentral island OS. Fundus examination revealed chalky-white disc swelling OU. Other systemic symptoms and signs were unremarkable. The erythrocyte sedimentation rate, C-reactive protein and platelet count were highly elevated. Temporal artery biopsy revealed multiple lymphocytes and multinucleated giant cells in the arterial media layer. To our knowledge, this is the first report of GCA in a Korean that has been confirmed with temporal artery biopsy. In conclusion, silent GCA can occur in Koreans, and hence, elderly patients presenting with chalky-white disc swelling, and corresponding laboratory findings must be evaluated for GCA.

Management of ischemic optic neuropathies.

Ischemic optic neuropathies (IONs) consist primarily of two types: anterior ischemic optic neuropathy (AION) and posterior ischemic optic neuropathy (PION). AION comprises arteritic AION (A-AION: due to giant cell arteritis) and non-arteritic AION (NA-AION: due to other causes). PION consists of arteritic PION (A-PION: due to giant cell arteritis), non-arteritic PION (NA-PION: due to other causes), and surgical PION (a complication of several systemic surgical procedures). These five types of ION are distinct clinical entities etiologically, pathogenetically, clinically and from the management point of view. In the management of AION, the first crucial step with patients aged 50 and over is to identify immediately whether it is arteritic or not because A-AION is an ophthalmic emergency and requires urgent treatment with high-dose steroid therapy to prevent any further visual loss in one or both eyes. Patients with NA-AION, when treated with systemic corticosteroid therapy within first 2 weeks of onset, had significantly better visual outcome than untreated ones. Systemic risk factors, particularly nocturnal arterial hypotension, play major roles in the development of NA-AION; management of them is essential in its prevention and management. NA-PION patients, when treated with high-dose systemic steroid therapy during the very early stages of the disease, showed significant improvement in visual acuity and visual fields, compared to untreated eyes. A-PION, like A-AION, requires urgent treatment with high-dose steroid therapy to prevent any further visual loss in one or both eyes. There is no satisfactory treatment for surgical PION, except to take prophylactic measures to prevent its development.

Arteritis de la temporal con VHS y PCR normales / Temporal arteritis with normal VHS and PCR

Se presenta un caso de arteritis de células gigantes con escasos criterios clínicos, VHS y PCR normal con neuropatía óptica isquémica posterior. Se hace una pequeña revisión del tema discutiéndose la importancia de la sospecha diagnóstica a pesar de los criterios clínicos y de laboratorios clásicamente descritos, destacando la importancia de la biopsia para el diagnóstico definitivo para el comienzo de la terapia específica, pilar fundamental para el pronóstico ocular y sistémico.

A case of giant cell arteritis with low clinical criteria, VHS and normal PCR with posterior ischemic optic neuropathy. A small review of the topic discussed the importance of the suspected diagnosis despite the clinical and laboratory criteria traditionally described, highlighting the importance of biopsy for definitive diagnosis for the commencement of specific therapy, a fundamental pillar for the ocular prognosis and systemic.

Aspectos clínicos da arterite temporal: [revisão] / Clinical aspects of the temporal arteritis: [review]

Introdução: A doença de Horton, ou arterite temporal, é a vasculite sistêmica auto-imune mais comum em adultos, especialmente nos idosos. Objetivo: Realizar uma revisão de literatura sobre os diversos aspectos da doença de Horton, evidenciando as manifestações clínicas otorrinolaringológicas. Método: Foram consultadas as bases de dados on-line EMedicine, Encyclopedia of Medicine, FindArticles, LILACS, MEDLINE, Merck Manuals On-Line Medical Library e SciELO, aplicando à pesquisa os termos: Doença de Horton, Arterite Temporal e Arterite de Células Gigantes, para artigos publicados entre 1996 e 2008. Revisão de Literatura: As manifestações clínicas da doença têm início após os 50 anos de idade, sendo mais freqüentes próximo dos 72 anos. A cefaléia intensa, a tumefação temporal, a claudicação mandibular e o déficit visual são os principais sinais e sintomas. Pode haver ainda disacusia, necrose lingual e odinofagia. Considerações Finais: Múltiplos aspectos da Doença de Horton não têm esclarecimentos devidos e, mesmo sendo uma doença que acomete muitos adultos, não possui investigações profundas em grande quantidade. A melhor compreensão desta levará ao incremento das possibilidades de cura dos pacientes e redução da morbidade, especialmente nas áreas oftalmo e otorrinolaringológica.

Introduction: The Horton's disease, or temporal arteritis, is the most common autoimmune systemic vasculitis in adults, especially the old-aged. Objective: To review the literature about the several aspects of the Horton's disease, and confirm the otorhinolaryngologic clinical manifestations. Method: The study searched online databases such as EMedicine, Encyclopedia of Medicine, FindArticles, LILACS, MEDLINE, Merck Manuals On-Line Medical Library and Scielo, and applied to the search the terms: Horton's disease, Temporal Arteritis and Giant Cells Arteritis, for articles published between 1996 and 2008. Literature's Review: The disease's clinical manifestations start after 50 years old, and it's more frequent near 72 years old. The intense headache, temporal tumefaction, mandibular claudication and visual loss are the main signals and symptoms. There may occur dysacusis, necrosis of the tongue and odynophagia. Considerations: Lots of aspects of the Horton's disease do not have their due elucidation and, even being a disease that affects many adults, it doesn't have deep investigations in great quantity. A better understanding of this disease will lead to the increment of cure possibilities and reduction of morbidity in the patients, especially in ophthalmologic and otorhinolaryngologic areas.

Arterite de células gigantes coronariana e infarto agudo do miocárdio / Coronary giant cell arteritis and acute myocardial infarction

Arterite de Células Gigantes (ACG) é uma vasculite sistêmica, granulomatosa, mediada por fatores imunitários, envolvendo artérias de grande e médio calibre e afetando preferencialmente idosos. A morte decorrente da ACG é rara e resulta principalmente da ruptura da aorta. Neste trabalho é relatado o caso de paciente de 83 anos, que faleceu inesperadamente, durante tratamento de ACG. A necropsia revelou envolvimento inflamatório das artérias coronárias, com trombose da artéria descendente anterior esquerda, infarto do miocárdio, ruptura da parede anterior do ventrículo esquerdo, hemopericárdio e tamponamento cardíaco. Infarto do miocárdio determinando morte súbita é uma complicação excepcional da ACG.

Giant cell arteritis (GCA) is a systemic immune-mediated granulomatous vasculitis of large- and medium-sized arteries mainly affecting elderly people. Death from GCA alone is rare and usually results of ruptured aorta. In this paper is reported a case of a 83-year-old woman who unexpectedly died during treatment of GCA. Necropsy revealed inflammatory involvement of the coronary arteries with left descendent anterior artery thrombosis, myocardial infarct and rupture of the anterior wall of the left ventricle, as well as hemopericardium and cardiac tamponade. Myocardial infarction leading to sudden death is an exceptional complication of GCA.

Anterior ischemic optic neuropathy: a comparison of the optic disc area of patients with the arteritic and non-arteritic forms of the disease and that of normal controls

PURPOSE: To evaluate the optic disc area of patients with non-arteritic anterior ischemic optic neuropathy (NA-AION) and arteritic anterior ischemic optic neuropathy (A-AION) and compare the results between each other and with those from controls in order to verify the existence and the magnitude of anatomical factors predisposing to the development of anterior ischemic optic neuropathy. METHODS: This is a case-controlled study of the optic disc area of 24 consecutive patients affected with NA-AION, 13 patients with A-AION and 24 consecutive healthy normals, sex- and age-matched with the patients with the NA-AION group. Measurements of optic disc area were performed for each studied group using disc photographs projected, measured and corrected for the refractive error and the keratometric readings, according to Littmann's method in each studied group. The results were compared using variance analysis. RESULTS: The mean areas and standard deviations of the optic discs from patients with NA-AION, A-AION and normals were respectively 1.99 ± 0.35 mm², 2.29 ± 0.39 mm² and 2.49 ± 0.53 mm². The statistical analysis revealed that the mean areas of the optic disc of patients with NA-AION were significantly different from those of normal controls. No statistical difference was found between A-AION and normal controls. CONCLUSION: NA-AION occurs predominantly in small discs while the arteritic form of the disease shows no such preference. Factors related to optic disc structure play a role in the pathophysiology of NA-AION. The occurrence of AION in large optic optic discs should raise the suspicion of temporal arteritis. On the other hand, small optic disc areas do not rule out that vasculitis.

OBJETIVO: Calcular a área do disco óptico de pacientes com neuropatia óptica isquêmica anterior não arterítica (NOIA-NA) e com neuropatia óptica isquêmica anterior por arterite temporal (NOIA-A) comparando os resultados entre si e com o grupos controle para verificar a existência e a magnitude de fatores anatômicos predisponentes ao desenvolvimento da neuropatia óptica isquêmica anterior. MÉTODOS: Estudo prospectivo, caso-controle das áreas dos discos ópticos de 24 pacientes acometidos por NOIA-NA, 13 com NOIA-A e 24 indivíduos controles normais. As medidas da área do disco óptico foram realizadas a partir de retinografias projetadas, sendo as medidas corrigidas levando em conta o erro refracional e a ceratometria de acordo com o método de Littmann em cada um dos grupos estudados. Os resultados foram comparados usando-se análise de variância. RESULTADOS: Os valores médios e desvios-padrão para as áreas do disco óptico dos olhos com NOIA-NA, NOIA-A e normais foram respectivamente de 1,99 ± 0,35 mm²; 2,29 ± 0,39 mm² e 2,49 ± 0,53 mm². A análise estatística revelou diferença significativa entre o grupo de olhos de pacientes com NOIA-NA e os controles normais. Não houve diferença significativa entre os olhos com NOIA-A e os controles. CONCLUSÕES: A forma não arterítica da NOIA ocorre em olhos com discos ópticos pequenos, ao passo que a forma arterítica da doença não mostra esta preferência. Fatores anatômicos estruturais do disco óptico têm um papel importante na fisiopatogenia da NOIA-NA. A ocorrência de NOIA em disco óptico de grandes dimensões deve reforçar a suspeita de arterite temporal. Discos ópticos pequenos, por outro lado não permitem excluir aquela vasculite.

Necrosis de la lengua en arteritis temporal / Tongue necrosis in temporal arteritis

La arteritis temporal es la vasculitis sistémica más frecuente en adultos. Las complicaciones menos frecuentes comprenden disfagia, vértigo, tos seca, infarto cerebral y necrosis de cuero cabelludo, labios y lengua. Una mujer de 76 años se presenta a la consulta con intensa cefalea y ardor en la lengua, con posterior necrosis de la hemilengua izquierda

Temporal arteritis is the most commom systemic vasculitis in adults. The less frecuent complications include dysphagia, vertigo, dry cough, stroke and necrosis of the scalp, lips and tongue. A 76 years old woman presented with strong headache and tongue tenderness, developing later on necrosis of the left half of her tongue.

Is Visual Loss Due to Giant Cell Arteritis Reversible?

Giant cell arteritis (GCA) is a common systemic vasculitis with an unknown etiology. It mainly affects people older than 50 years of age and often presents with symptoms such as headache, jaw claudication, visual loss, polymyalgia rheumatica and increased erythrocyte sedimentation rate (ESR). Established blindness is irreversible if the steroid treatment is not administered within a few days. Here, we report a case of GCA in a patient with a normal ESR whose left eye perceived just light at the initiation of treatment. Immediately prior to the combined treatment with high dose oral steroids and cyclophosphamide, the ESR level had increased to 80 mm/h and the vision improved after the combined treatment four months later.

Giant cell arteritis--a rare cause of fever of unknown origin in India.

Giant cell arteritis (GCA) is a systemic large vessel vasculitis. Awareness of various manifestations of GCA is essential for early recognition and prompt treatment so as to prevent complications like blindness. GCA is one of the relatively common causes of fever of unknown origin (FUO) in the elderly in USA and Europe. However, no such cases have been reported from India. A case of GCA presenting as FUO is reported and the literature reviewed.

Doença encéfalo-vascular como primeira manifestaçäo de arterite temporal: relato de dois casos / Brain vascular disease presenting as first manifestation of temporal arteritis: report of two cases

É raro doença encéfalo-vascular como primeira manifestaçäo de arterite temporal. Relatamos dois casos, nos quais o diagnóstico emergiu da anamnese. Mulher de 55 anos havia apresentado enfarto em hemisfério cerebral esquerdo há 3 meses. Vinha com melhora progressiva quando teve perda visual completa e a família nos procurou para uma segunda opiniäo. A paciente havia tido hemiplegia direita, afasia e distúrbio visual à esquerda no primeiro episódio; o quadro atual era de perda visual completa bilateral. Com suspeita de arterite temporal a paciente foi internada; a VHS era de 97 mm e a fundoscopia mostrava intensa neurite óptica isquêmica. Homem de 75 anos apresentou síndrome de Wallemberg. Na revisäo da anamnese destacou-se cefaléia temporal e no exame, processo inflamatório na regiäo da artéria temporal; a VHS era de 70 mm e a biópsia confirmou diagnóstico. A "tecnolatria" tem contaminado o exercício da medicina; convém que relembremos a soberania dos dados clínicos.