Arteritis de células gigantes: ¿Cómo debutan pacientes chilenos? Análisis de casos y revisión de la literatura / Giant cell arteritis. Experience in 32 patients

BACKGROUND: Giant cell Arteritis (GCA) is the most common systemic vasculitis in patients over 50 years. Diagnosis is based on clinical, laboratory, imaging and biopsy. Temporal artery biopsy (TAB) may be inconclusive in up to 40% of patients. AIM: To describe disease features of patients diagnosed with GCA. MATERIAL AND METHODS: Review of pathology reports of giant cell arteritis and clinical records of patients seen with the diagnosis between 2000 and 2019. Demographic, clinical, laboratory, histopathology, imaging, treatment and follow-up variables were analyzed. RESULTS: We fetched 32 patients with a median age at diagnosis of 70.5 years (range 57-90), 81% women. Twenty eight percent had polymyalgia. 72% had only cranial symptoms, 12% had extracranial involvement and 13% exclusive extracranial involvement. The median time from onset of symptoms to diagnosis was two months (range 0.5-8). All had elevated erythrocyte sedimentation rate and c reactive protein. A TAB was performed in 27 patients and in 17 (65.4%) it confirmed the diagnosis. Transmural inflam- mation was the most frequent finding. All patients received steroids. Follow-up information was available from 25 patients and 92% received a steroid-spa- ring agent, usually methotrexate (74%). Ninety two percent achieved clinical remission in the first year and 59% had minor relapses during steroid tapering. CONCLUSIONS: Our patients showed frequent extracranial involvement and TAB was a useful diagnostic tool.

Necrosis lingual como forma de presentación de arteritis temporal / Lingual necrosis as a form of presentation of temporary arteritis

La arteritis de células gigantes es una vasculitis sistémica que compromete arterias de mediano y gran calibre, principalmente la arteria aorta y sus ramas. Su prevalencia es mayor en mujeres a partir de los 50 años, típicamente se manifiesta con fiebre, claudicación mandibular, cefalea, hiperestesia del cuero cabelludo y pérdida de la visión con neuropatía óptica isquémica anterior, en una minoría de casos aparecen síntomas menos frecuentes que dificultan y retrasan el diagnóstico. Se presenta el caso de una mujer de 76 años que consultó por dolor en la cavidad bucal con edema lingual y en cuello de 48 horas de evolución asociado a cefalea el mes previo. En el examen físico presentaba signos clínicos de isquemia lingual, por lo que se consideró como diagnóstico presuntivo compromiso isquémico por arteritis de células gigantes, e inició tratamiento con corticoides sistémicos realizándose una biopsia de arteria temporal que evidenció infiltrado linfocitario panparietal con engrosamiento de la túnica íntima y hallazgos compatibles con panarteritis. La arteritis de células gigantes debe ser sospechada en pacientes con manifestaciones de isquemia lingual, iniciándose en forma precoz el tratamiento para evitar complicaciones irreversibles.

Giant cell arteritis is a systemic vasculitis that affects arteries of medium and large caliber, mainly the aorta artery and its main branches. It is more frequent in women older than 50 years. The most common symptoms are fever, jaw claudication, headache, hyperesthesia of the scalp and loss of vision with anterior ischemic optic nerve disease. But, in a minority of cases, less frequent symptoms are observed that delay and make more difficult the diagnosis. Here, we present the case of a 76-year-old woman who came to our consultation having pain in the oral cavity and presenting tongue and neck edema for 48 hours. She had also suffered from headaches during the previous month. Because the physical examination showed clinical signs of lingual ischemia, a presumptive diagnosis of ischemic involvement due to giant cell arteritis was considered. She started a treatment with systemic corticosteroids and a temporal artery biopsy was performed. We conclude, that giant cell arteritis should be suspected in patients presenting lingual ischemia symptoms in order to start the specific treatment early enough to avoid irreversible complications.

Utilidad de la ecotomografía doppler en el diagnóstico de la arteritis de células gigantes / Utility of the doppler ultrasound in the diagnostic approach of giant cell arteritis

Resumen: La arteritis de células gigantes es una vasculitis sistémica frecuente, especialmente en mayores de 50 años. Ante la sospecha clínica el diagnóstico debe ser confirmado con histología o estudios de imágenes. La biopsia de arteria temporal se considera el gold standard, sin embargo, tiene sus limitaciones, una de ellas es la alta tasa de falsos negativos. El estudio por ecotomografía Doppler permite la visualización de las arterias temporales y en manos experimentadas puede constituir un procedimiento diagnóstico alternativo a la biopsia en el estudio inicial de esta entidad. Esta técnica es de fácil acceso, bajo costo, sin riesgos y no expone al paciente a radiación ionizante. En este trabajo revisamos la utilidad clínica de la ecotomografía Doppler en el estudio de la arteria temporal y sus hallazgos imagenológicos en la aproximación diagnóstica a la arteritis de células gigantes, siendo las características de compromiso el engrosamiento parietal arterial hipoecogénico, habitualmente concéntrico, no compresible.

Abstract: Giant cell arteritis is a frequent systemic vasculitis, especially in patients older than 50 years old. When clinically suspected, the diagnosis should be made on the basis of histology or imaging methods. Temporal artery biopsy is considered the gold standard for the diagnosis but it has some caveats, especially the existence of false negatives. Doppler ultrasound study is a low cost, accessible tool that allows visualization of temporal arteries and, in experienced hands, can replace the biopsy in the initial evaluation of the disease, without the risks of ionizing radiation. In this article we review the clinical utility of the temporal artery Doppler ultrasound and its findings in the diagnostic approach of giant cell arteritis, being characteristic a non-compressible, hypoechoic, most commonly concentric arterial wall thickening.

Arteritis de células gigantes en el sistema vertebrobasilar, isquemias cerebelo-occipitales y parálisis III par: caso clínico / Giant cell arteritis of the vertebrobasilar system, posterior ischemia and palsy of the third nerve: report of one case

Giant cell arteritis is the most common vasculitis in patients aged over 50 years. We report an 89-year-old woman with significant weight loss and persistent frontal-occipital headaches lasting two months. The neurological examination at admission identified a decrease in visual acuity of the left eye, paralysis of the third cranial nerve of the right eye and alterations of body motility without objective signs of damage of the motor or sensitive pathways. Magnetic resonance imaging showed changes of the temporal artery wall and in both vertebral arteries, as well as bilateral cerebellar and occipital ischemic lesions. The Doppler ultrasound of the temporal arteries was compatible with Giant cell arteritis. Treatment with steroids was started. While receiving oral prednisone, the patient suffered new infarcts of the posterior territory, documented with a CAT scan.

Clinical correlation of biopsy results in patients with temporal arteritis

Summary Objective: Temporal arteritis is systemic vasculitis of medium and large sized vessels. The lowest incidence rates were reported in Turkey, Japan and Israel. We aimed to investigate the results of patients with biopsy-proven temporal arteritis and those classified according to the American College of Rheumatology criteria from a low-incidence region for temporal arteritis. The results of our study are noteworthy, since there is limited data on pathologic diagnosis of temporal arteritis in Turkey. Method: We studied the medical records, laboratory findings such as erythrocyte sedimentation rate and C-reactive protein levels, biopsy results, and postoperative complications of all the patients operated for temporal artery biopsy at our clinic. We used the computerized laboratory registry that keeps all records of 42 consecutive temporal artery biopsy results from January 2011 to December 2016. Results: The mean age was 66±12.5 years. The most common manifestations on admission were temporal headache, optic neuritis and jaw claudication, respectively. Temporal artery biopsy results confirmed tempoal arteritis in eight out of 42 (19%) patients. There was no statistically significant difference between biopsy-positive and biopsy-negative groups in terms of sex, age, erythrocyte sedimentation rate, C-reactive protein and biopsy length. Conclusion: We were not able to find a correlation between the analysis of biopsy results and clinical evaluation of patients with temporal arteritis. We suggest that diagnosis of temporal arteritis depends on clinical suspicion. Laboratory examination results may not be helpful in accurate diagnosis of tempoal arteritis.

Arteritis de células gigantes: compromiso extenso de la aorta y grandes ramas demostrado por tomografía por emisión de positrones. Caso clínico / Extensive arterial involvement in giant cell arteritis: report of one case

Large vessel vasculitis and particularly Temporal Arteritis are systemic diseases that may affect the aorta and its major branches, mainly involving extra cranial branches of the carotid artery. We report a 72-year-old man presenting with weight loss, fever and malaise. Positron emission computed tomography (PET CT) showed an extensive inflammation of the aorta and its major branches. Temporal artery biopsy confirmed the presence of vasculitis with granulomas. Treatment with a high dose of steroids had an excellent clinical response. This case underscores the utility of PET CT in the assessment of this disease.

Giant cell arteritis: a multicenter observational study in Brazil

OBJECTIVE: To describe demographic features, disease manifestations and therapy in patients with giant cell arteritis from referral centers in Brazil. METHODS: A retrospective cohort study was performed on 45 giant cell arteritis patients from three university hospitals in Brazil. Diagnoses were based on the American College of Rheumatology classification criteria for giant cell arteritis or temporal artery biopsy findings. RESULTS: Most patients were Caucasian, and females were slightly more predominant. The frequencies of disease manifestations were as follows: temporal headache in 82.2%, neuro-ophthalmologic manifestations in 68.9%, jaw claudication in 48.9%, systemic symptoms in 44.4%, polymyalgia rheumatica in 35.6% and extra-cranial vessel involvement in 17.8% of cases. Aortic aneurysms were observed in 6.6% of patients. A comparison between patients with biopsy-proven giant cell arteritis and those without temporal artery biopsies did not yield significant differences in disease manifestations. All patients were treated with oral prednisone, and intravenous methylprednisolone was administered to nearly half of the patients. Methotrexate was the most commonly used immunosuppressive agent, and low-dose aspirin was prescribed to the majority of patients. Relapses occurred in 28.9% of patients, and aspirin had a protective effect against relapses. Females had higher prevalences of polymyalgia rheumatica, systemic manifestations and jaw claudication, while permanent visual loss was more prevalent in men. CONCLUSIONS: Most of the clinical features of Brazilian giant cell arteritis patients were similar to those found in other studies, except for the high prevalence of neuro-ophthalmic manifestations and permanent blindness in the Brazilian patients. Aspirin had a protective effect on relapses.

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.

Giant cell arteritis: A clinical and pathological study.

Background. Giant cell arteritis is a vasculitis affecting large- and medium-calibre vessels. It is not uncommon in the West and there are many large series in the literature. However, there are very few reports of giant cell arteritis among Indian patients. Methods. We did a retrospective study of 9 Indian patients (5 men and 4 women; age range 59–81 years [mean and median 70 years]) who had had a temporal artery biopsy for suspected giant cell arteritis at a tertiary care hospital. Results. Eight patients had biopsy-proven giant cell arteritis. The common presenting features were pyrexia of unknown origin (4), headache (6) and blurring of vision (2). The erythrocyte sedimentation rate was elevated and ranged from 25 to 120 mm at the end of the first hour (mean 96, median 105). The C-reactive protein level, which was available in 5 cases, was raised. Giant cells and inflammatory cells were seen in 7 of 8 temporal artery biopsies; a transmural lymphocytic and neutrophil infiltrate without giant cells was present in 1 case. All patients were treated with steroids and they responded well. Conclusion. Temporal arteritis is probably under-recognized in India. Pyrexia is a common presenting feature of the disease; temporal arteritis should be considered in the differential diagnosis of elderly patients with pyrexia of unknown origin.

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.

Tos prolongada como manifestación inicial de la arteritis de la temporal / Prolonged cough as initial symptom of temporal arteritis

La tos prolongada puede ser un desafío diagnóstico y terapéutico. Presentamos el caso de un enfermo de 62 años con tos seca y quebrantamiento del estado general durante cuatro semanas, seguido de un síndrome febril prolongado cuyo diagnóstico final fue arteritis de la temporal, con confirmación por anatomía patológica. El dolor en los pabellones auriculares al adoptar el decúbito lateral fue otro síntoma interesante. La tos prolongada puede ser una manifestación inicial de la arteritis temporal en adultos mayores.

Prolonged cough could be a diagnostic and therapeutic dilemma. We present here the case of an elder man who suffered from a dry cough and malaise during four weeks, followed by a fever of unknown origin. After many studies and review of symptoms, a biopsy of the temporal artery confirmed a giant cell arteritis. Cough and malaise could be an early symptom of temporal arteritis in elderly patients. Pain in the outer ear on the lateral decubitus could be another interesting symptom in this disease.

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.

Mieloma múltiple y arteritis de células gigantes sin amiloidosis / Simultaneous multiple myeloma and giant cell arteritis without systemic amyloidosis

La amiloidosis sistémica primaria y el mieloma múltiple con amiloidosis primaria se han presentado con características clínicas e histopatológicas que simulan una arteritis de células gigantes. Hasta el momento la asociación se basaba en el rol antigénico del depósito de amiloide sobre las arterias, desencadenando la respuesta inmune que finaliza con una arteritis. Presentamos el primer caso en la literatura de un paciente con mieloma múltiple y arteritis de células gigantes sin amiloidosis sistémica, sugiriendo una relación patogénica entre estas dos entidades. En el caso de nuestro paciente se descartó la presencia de amiloide en la pared arterial, por lo que proponemos que el estímulo para el desarrollo de la arteritis podría ser una excesiva producción de interleuquina 6 fabricada por las células mielomatosas

Primary systemic amyloidosis with clinical and histopathologic features of giant cell arteritis has already been described. The association of multiple myeloma (with primary amyloidosis) and giant cell arteritis is also known. We present the first case in the literature of a patient with multiple myeloma and giant cell arteritis without systemic amyloidosis, suggesting a pathogenic relationship between the two diseases

Arteritis de la temporal: manifestada por compromiso del estado general: caso clínico y revisión de la literatura / Temporal arteritis demonstrated by commitment of the general state: case report and review of the literature

La arteritis de la temporal corresponde a la vasculitis primaria más frecuente, cuyo principal factor de riesgo es la edad. Clínicamente se caracteriza por cefalea asociado a claudicación de la articulación temporomandibular y síntomas de compromiso del estado general, junto con signos inflamatorios en el área afectada. En un 40 por ciento de los casos se asocia a Polimialgia reumática. El tratamiento es con corticoides, cuyo principal beneficio es prevenir la perdida visual asociada a esta patología. Presentamos el caso de un paciente de sexo masculino de 77 años, cuyo diagnóstico final se llego luego de un largo estudio por compromiso del estado general, este caso pone de manifiesto la necesidad de considerar esta entidad, aun en pacientes sin los signos clásicos asociados a esta vasculitis.

A forma arterítica da neuropatia óptica isquêmica anterior: estudo de 25 casos / The arteritic type of anterior ischemic optic neuropathy: study of 25 cases

O quadro clínico, laboratorial, campimétrico e angiofluoresceinográfico de 25 pacientes (40 olhos) com a forma arterítica da neuropatia óptica isquêmica anterior (NOIA-A) foi estudada com a finalidade de definir o perfil da doença possibilitando seu diagnóstico diferencial com a forma näo arterítica (NOIA-A) e com outras doenças do nervo óptico. A NOIA-A ocorreu em pacientes entre 60 e 88 anos (média 74 anos), predominando no sexo feminino (64 por cento). Em 15 pacientes o envolvimento foi bilateral, ocorrendo simultaneamente ou nas primeiras semanas após o acometimento incial. Cefaléia e dor ocular foram os pródromos mais frequentes, enquanto todos os pacientes apresentavam, ao momento da perda visual, sintomas e sinais sistêmicos da arterite de células gigantes (ACG). Elevaçäo da proteina C reativa, do fibrinogênico e da velocidade de hemossedimentaçäo foram as alteraçöes laboratoriais mais frequentemente encontradas. A acuidade, em geral, foi severamente acometida. As principais anormalidades fundoscópicas foram edema do disco óptico, usualmente pálido, com ou sem hemorragias, e alteraçöes retinianas. Exame do campo visual demonstrou uma variedade de defeitos, principalmente altitudinais inferiores enquanto a principal característica angiofluoresceinográfica observada foi o atraso setorial ou difuso do enchimento da coróide