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.

Arteritis de células gigantes: actualización y proposición de un algoritmo de estudio

Giant cell arteritis (GCA) is a primary granulomatous systemic vasculitis involving the aorta and its main branches that affects people aged over 50 years with a genetic predisposition. Its main phenotypes are cranial and extracranial involvement, with or without symptoms of polymyalgia rheumatica. These phenotypes can overlap. The extracranial form can be oligosymptomatic and must be sought directly. The main complications of the disease are ischemia of essential territories such as the optic nerve or cerebral circulation, and aneurysmal dilations of the aorta and its large branches. Clinicians must be aware of all the presentation forms of the disease, to start a timely treatment and avoid potentially serious or fatal consequences. To date, the diagnosis of GCA is based on clinical and pathological criteria, with the temporal artery biopsy as the "gold standard" for diagnosis, although its sensitivity is variable. This can lead to an underdiagnosis in patients with negative biopsies or predominant extra-cranial symptoms. The emergence of new and valuable imaging tools substantially improved the timely diagnosis, mainly in subclinical and oligosymptomatic forms. Among them we highlight ultrasonography of the temporal and axillary arteries, Computed Tomography Angiography, Magnetic Resonance Angiography, and PET-CT. These imaging techniques are complementary, and their use is highly recommended. GCA treatment is based on steroidal therapy, often associated with a corticosteroid-sparing immunosuppressive agent. The follow-up is eminently clinical.

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.

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.

Arteritis de células gigantes relacionada a grandes vasos: a propósito de un caso / Large vessel giant cell arteritis: a case report

La arteritis de células gigantes corresponde a una vasculitis granulomatosa que afecta arterias de mediano y gran tamaño. El fenotipo clínico más conocido es la arteritis de la temporal, caracterizado por síntomas craneales clásicos como cefalea temporal, claudicación mandibular y síntomas visuales. Sin embargo, esta enfermedad puede comprometer otras grandes arterias como la aorta y sus ramas principales, denomi-nándose así, como arteritis de células gigantes de grandes vasos, la cual puede o no estar asociada a síntomas craneales. Presentamos el caso de una mujer de 74 años, con un cuadro de un mes de evolución, caracterizado por claudicación intermitente de extremidades inferiores, asociado a baja de peso de 3 kilos, sudoración nocturna. Al examen físico, fiebre y pulsos dismi-nuidos en extremidades inferiores.

Giant cell arteritis is a granulomatous vasculitis that affects arteries of medi-um and large size. The most well-known clinical phenotype is temporal arteri-tis, characterized by classic cranial symptoms such as temporal headache, man-dibular claudication and visual symptoms. However, this disease can involve other large arteries such as the aorta and its main branches, known as large ves-sel giant cell arteritis, which may or may not be associated with cranial symptoms.A 74-year-old woman is presented with claudication of lower extremities, associated with weight loss of 3 kilos, night sweats and fever over the past month. Physical ex-amination reveals decreased pulses in the lower extremities.

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.

Arteritis temporal en paciente varón de 38 años: reporte de caso y revisión de la literatura / Temporary arteritis in a 38-year-old patient male: report of case and review of the literature

Temporal arteritis, a large vassel vasculitis, particularly in its classical form, is extremely rare in individuals < 50 years. We report a 38 years old male patient that in the context of fever of unknown origin, and after clinical examination, laboratory and imagin analyses get the diagnosis in mention. Been treated with prednisone at 1 mg/kg/d, the therapeutic response was satisfactory. Today, the patient remains asymptomatic...

La arteritis temporal, una forma de vasculitis de vaso grande, particularmente en su forma clásica, es extremadamente rara en individuos < 50 años. Se reporta el caso de un paciente varón de 38 años que, en contexto de Fiebre de Origen Desconocido luego del estudio clínico, laboratorial y de imagenología, llevó al diagnóstico en mención. Al ser tratado con prednisona en dosis de 1 mg/kg/d, la respuesta terapéutica fue satisfactoria. Actualmente se encuentra asintomático...

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.

Inmunopatogenia de la arteritis de la temporal / Immunopathogenesis of temporal arteritis

La arteritis de la temporal, clasificada como una vasculitis que compromete vasos de gran y mediano calibre, debe ser considerada como una emergencia médica, dado el potencial de causar ceguera y accidentes vasculares. La lesión típica corresponde a granulomas en la pared vascular, los que están constituidos por macrófagos y célulasT CD4+. Éstos se activan en la adventicia, luego de interactuar con las células dendríticas nativas. La injuria tisular es mediada por diversos subtipos de macrófagos, los que ejercen las diferentes funciones efectoras. El daño que domina en la capa media resulta del estrés oxidativo y determina la apoptosis de las células musculares lisas y la nitración de las endoteliales. Por otro lado, factores de crecimiento derivados de macrófagos determinan la hiperplasia intimal y la consecuente oclusión luminal. Las manifestaciones clínicas se relacionan estrechamente con el sitio isquémico. El tratamiento de elección son los corticoides sistémicos, los cuales pueden asociarse a inmunosupresores como también con agentes biológicos.

Temporal arthritis, which is classified as a large-and medium-caliber vessel vasculitis, should be considered as a medical emergency, given its potential to cause blindness and strokes. The injury typically corresponds to granulomas in the vascular wall, which are composed of macrophages and CD4+ T cells. They are activated in the adventitia, after interacting with native dendritic cells. Immunopathological mechanisms involve different subtypes of macrophaesges, which exert different effector functions. Damage that prevails within the median layer is secondary to oxidative stress and triggers apoptosis of smooth muscle cells and nitration of endothelial cells. On the other hand, growth factors derived from macrophages determine intimal hyperplasia and subsequent luminal occlusion. Clinical manifestations are closely related to the ischemic site. The treatment of choice is systemic corticosteroids, which can be associated with immunosuppressive drugs as well as biological agents.

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.

Isquemia crítica de miembros inferiores por arteritis de células gigantes / Lower limb critical ischemia secondary to giant cell arteritis

Presentamos el caso de una mujer de 72 años de edad con isquemia crítica de los miembros inferiores por arteritis de células gigantes, documentado clínica, arteriográfica y patológicamente (biopsia arteria temporal), con excelente respuesta al tratamiento con prednisona y ciclofosfamida.

Arteritis de células gigantes con compromiso de grandes vasos y arteria temporal / Large vessel and temporal artery involvement in temporal arteritis

El motivo de presentación de este cuadro clínico es la manifestación inicial atípica de una enfermedad arterial inflamatoria. Lo que generó la consulta de la paciente fue la sintomatología isquémica que padecía, con el desarrollo de claudicación de ambos miembros superiores, acompañado de un fenómeno de Raynaud y úlceras en la mano izquierda. Se describe la historia clínica de la paciente, cómo se llegó al diagnóstico de arteritis de la arteria temporal y los tratamientos realizados.

Arteritis temporal: Enfermedad de Horton / Temporal arteritis: Horton disease

La arteritis de células gigantes (ACG), es una enfermedad vasculítica común en la población anciana. Muchas manifestaciones son frecuentemente desconocidas y su diagnóstico y apropiado tratamiento se postergan. En base a cinco observaciones personales, exponemos una revisión sobre etiopatogenia, clínica, paraclínica y tratamiento de diferentes formas de la enfermedad

Arteritis temporal de células gigantes (ATCG): estudio de 7 pacientes / Temporal giant cell arteritis (TGCA): study of 7 patients

Se estudian 7 pacientes con diagnóstico de arteritis de células gigantes en el Hospital Clinicoquirúrgico "Saturnino Lora" de Santiago de Cuba; para lo cual se tuvo en cuenta exclusivamente su comprobación histológica. Se analizaron: la edad, el sexo y comienzo de los síntomas, y se revisaron las características clínicas presentadas por estos pacientes. Se exponen los hallazgos biológicos y se subraya la elevación de la velocidad de sedimentación globular (VSG) y el aumento de la globulina alfa-2 en el estudio de electroforesis de proteínas. Finalmente se discuten aspectos morfológicos y patogénicos de la enfermedad, así como su tratamiento mediante el empleo de los corticosteroides

Arteritis de células gigantes y polimialgia reumática / Giant cell arteritis and rheumatic polymialgia

Se analizan las principales características tanto epidemiológicas como clínicas de los cuadros aparentemente infrecuentes pero no excepcionales en nuestro medio . Ellos son la arteritis de células gigantes o arteritis temporal y la polimialgia reumática. Ambos afectan a mayores de 50 ó 60 años y suelen presentarse en forma concomitante, lo que ha hecho precisar que podrían ser manifestaciones clínicas de una sóla entidad clínica. Se mencionan los principales elementos diagnósticos de laboratorio, destacando la importancia práctica de la velocidad de sedimentación en el control de la evolución y de las alternativas histológicas en la biopsia de arteria temporal. Se señala la utilidad en ambos cuadros del tratamiento esteroidal, el que en caso de la arteritis temporal debe instituirse lo más precozmente posible para evitar el compromiso ocular que es grave. Se enfatizan los inconvenientes que reviste, en los pacientes de edad, la administración de corticoides por largos períodos o en forma indefinida