Arteritis de Células Gigantes

Descripción General

Epidemiología

• Vasculitis Vasculitis Inflammation of any one of the blood vessels, including the arteries; veins; and rest of the vasculature system in the body. Systemic Lupus Erythematosus sistémica idiopática más común
• La edad es el mayor factor de riesgo:
  • Generalmente afecta a personas mayores de 50 años
  • Mayor incidencia en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum la 8va y 9na década de la vida
• 3 veces más frecuente en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum las mujeres
• Ascendencia del norte de Europa y escandinava
• Un índice de masa corporal (IMC) más bajo se asocia a un mayor riesgo.

Etiología

• Se desconoce la causa exacta.
• Los LOS Neisseria factores genéticos pueden aumentar la susceptibilidad:
  • Variantes genéticas del complejo mayor de histocompatibilidad (CMH)
  • Subtipos de antígenos leucocitarios humanos (HLA, por sus siglas en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum inglés)
  • Genes Genes A category of nucleic acid sequences that function as units of heredity and which code for the basic instructions for the development, reproduction, and maintenance of organisms. DNA Types and Structure implicados en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum las células T
• El 45%–50% tienen polimialgia reumática concurrente.

Fisiopatología

La arteritis de células gigantes está causada por una complicada cascada de inflamación vascular, daño y reparación disfuncional:

• No se ha HA Hemolytic anemia (HA) is the term given to a large group of anemias that are caused by the premature destruction/hemolysis of circulating red blood cells (RBCs). Hemolysis can occur within (intravascular hemolysis) or outside the blood vessels (extravascular hemolysis). Hemolytic Anemia identificado el evento desencadenante.
• Afecta a las arterias grandes y medianas:
  • Suele afectar a la aorta Aorta The main trunk of the systemic arteries. Mediastinum and Great Vessels: Anatomy y a sus ramas principales
  • Afectación común de las ramas de las arterias carótidas y vertebrales
• Afecta sobre todo a la arteria temporal superficial (rama de la arteria carótida externa)
• Mecanismo:
  1. Un antígeno es detectado por las células dendríticas en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum la adventicia → recluta más células dendríticas, linfocitos y macrófagos.
  2. Las células dendríticas procesan y presentan los LOS Neisseria antígenos → activación de células T del antígeno de diferenciación 4 (cluster of differentiation 4, CD4+).
  3. Las células T CD4+ proliferan → inflamación vascular a través de la activación de los LOS Neisseria macrófagos.
  4. La capa medial es invadida por células inflamatorias → los LOS Neisseria macrófagos sufren una organización granulomatosa (células gigantes) → se producen factores de crecimiento y metaloproteinasas → pérdida de células musculares lisas vasculares y destrucción de la lámina elástica interna.
  5. Las células musculares lisas lesionadas responden a este daño → los LOS Neisseria miofibroblastos se diferencian, migran hacia la capa íntima y producen proteínas de la matriz extracelular → hiperplasia intimal y oclusión arterial → complicaciones isquémicas de la enfermedad.

Presentación Clínica

Manifestaciones clínicas

• Constitucionales:
  • Fiebre
  • Fatiga
  • Malestar
  • Pérdida de peso
  • Anorexia Anorexia The lack or loss of appetite accompanied by an aversion to food and the inability to eat. It is the defining characteristic of the disorder anorexia nervosa. Anorexia Nervosa
• Cabeza y cuello:
  • Cefalea:
    • Ocurre en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum ⅔ de los LOS Neisseria pacientes
    • Gravedad y calidad variables
    • Se localiza en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum las sienes y puede ser sensible al AL Amyloidosis tacto
    • Sensibilidad del cuero cabelludo asociada
  • Claudicación de la mandíbula:
    • Ocurre en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum aproximadamente la mitad de los LOS Neisseria pacientes
    • Dolor Dolor Inflammation o fatiga mandibular durante la masticación que se alivia con el descanso
• Oftálmicas:
  • Pérdida transitoria de la visión (amaurosis fugaz):
    • Es el resultado de la pérdida de flujo sanguíneo en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum el ojo
    • Típicamente monocular
    • Indolora, transitoria e intermitente
    • Visión borrosa, diplopía o pérdida de visión
  • Pérdida permanente de la visión:
    • Complicación más temida
    • Debido a la neuropatía óptica isquémica anterior o posterior, la oclusión de la arteria central de la retina Retina The ten-layered nervous tissue membrane of the eye. It is continuous with the optic nerve and receives images of external objects and transmits visual impulses to the brain. Its outer surface is in contact with the choroid and the inner surface with the vitreous body. The outermost layer is pigmented, whereas the inner nine layers are transparent. Eye: Anatomy o a isquemia cerebral
    • Pérdida parcial o total de la visión, indolora y súbita
    • Puede afectar a 1 o ambos ojos
    • Rara vez es reversible

• Hallazgos musculoesqueléticos:
  • Polimialgia reumática:
    • Dolor Dolor Inflammation y rigidez matutina en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum los LOS Neisseria músculos y articulaciones proximales
    • Hombros, pelvis Pelvis The pelvis consists of the bony pelvic girdle, the muscular and ligamentous pelvic floor, and the pelvic cavity, which contains viscera, vessels, and multiple nerves and muscles. The pelvic girdle, composed of 2 “hip” bones and the sacrum, is a ring-like bony structure of the axial skeleton that links the vertebral column with the lower extremities. Pelvis: Anatomy ósea, cuello y torso afectados

Examen físico

• Cardiovascular:
  • Pulsaciones disminuidas
  • Sensibilidad y agrandamiento de la arteria temporal
  • Presión arterial discordante en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum los LOS Neisseria brazos → afectación de la arteria subclavia
  • Soplos
  • Soplo cardíaco de insuficiencia aórtica → sospecha de aneurisma de aorta Aorta The main trunk of the systemic arteries. Mediastinum and Great Vessels: Anatomy ascendente
• Fundoscópico:
  • Puede ser totalmente normal con síntomas transitorios
  • Isquemia retiniana → manchas de algodón en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum la retina Retina The ten-layered nervous tissue membrane of the eye. It is continuous with the optic nerve and receives images of external objects and transmits visual impulses to the brain. Its outer surface is in contact with the choroid and the inner surface with the vitreous body. The outermost layer is pigmented, whereas the inner nine layers are transparent. Eye: Anatomy
  • Neuropatía óptica isquémica anterior (pérdida de flujo sanguíneo en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum la parte anterior del nervio óptico) → disco hinchado y pálido y márgenes de la retina Retina The ten-layered nervous tissue membrane of the eye. It is continuous with the optic nerve and receives images of external objects and transmits visual impulses to the brain. Its outer surface is in contact with the choroid and the inner surface with the vitreous body. The outermost layer is pigmented, whereas the inner nine layers are transparent. Eye: Anatomy borrosos
• Hallazgos musculoesqueléticos:
  • Rango de movimiento limitado en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum hombros, cuello y cadera
  • Sinovitis de muñeca y metacarpofalángica

Diagnóstico

Algoritmo diagnóstico

Modalidades de diagnóstico

• En EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum 1er lugar, evaluar los LOS Neisseria estudios de laboratorio:
  • VES y PCR PCR Polymerase chain reaction (PCR) is a technique that amplifies DNA fragments exponentially for analysis. The process is highly specific, allowing for the targeting of specific genomic sequences, even with minuscule sample amounts. The PCR cycles multiple times through 3 phases: denaturation of the template DNA, annealing of a specific primer to the individual DNA strands, and synthesis/elongation of new DNA molecules. Polymerase Chain Reaction (PCR):
    • Casi siempre elevados
    • Inespecífico
  • Hemograma:
    • Anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview and Types normocrómica
    • Trombocitosis reactiva
    • Leucocitos normales o mínimamente elevados
  • Panel de función hepática:
    • Fosfatasa alcalina elevada
    • Disminución de la albúmina
• Pasos a seguir:
  • Biopsia de la arteria temporal (estándar de oro):
    • Debe realizarse en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum todos los LOS Neisseria pacientes con sospecha de arteritis de células gigantes
    • Las lesiones son segmentarias, por lo que el diagnóstico requiere una biopsia de un segmento largo del vaso.
    • Hallazgos histopatológicos:
      • Panarteritis Panarteritis Giant Cell Arteritis
      • Células gigantes
      • Lámina elástica interna fragmentada
    • Si es negativa, debe considerarse una biopsia de la arteria temporal contralateral.
  • Ultrasonido Doppler Doppler Ultrasonography applying the doppler effect, with frequency-shifted ultrasound reflections produced by moving targets (usually red blood cells) in the bloodstream along the ultrasound axis in direct proportion to the velocity of movement of the targets, to determine both direction and velocity of blood flow. Ultrasound (Sonography) color:
    • Posible alternativa no invasiva para la biopsia de la arteria temporal, pero es operador dependiente
    • Debe evaluar los LOS Neisseria vasos de la cabeza, el cuello y las extremidades superiores
    • El “signo del halo” (área oscurecida que rodea el lumen vascular por el edema Edema Edema is a condition in which excess serous fluid accumulates in the body cavity or interstitial space of connective tissues. Edema is a symptom observed in several medical conditions. It can be categorized into 2 types, namely, peripheral (in the extremities) and internal (in an organ or body cavity). Edema mural) es altamente específico.
• Si la prueba anterior no es diagnóstica, considerar la posibilidad de evaluar la afectación de los LOS Neisseria grandes vasos:
  • Ultrasonido Doppler Doppler Ultrasonography applying the doppler effect, with frequency-shifted ultrasound reflections produced by moving targets (usually red blood cells) in the bloodstream along the ultrasound axis in direct proportion to the velocity of movement of the targets, to determine both direction and velocity of blood flow. Ultrasound (Sonography) color de las arterias axilares y subclavias
  • Tomografía computarizada con angiografía
  • Resonancia magnética (RM) o angiografía por resonancia magnética (RM)
  • Tomografía por emisión de positrones ( PET PET An imaging technique that combines a positron-emission tomography (PET) scanner and a ct X ray scanner. This establishes a precise anatomic localization in the same session. Nuclear Imaging)

Tratamiento

Opciones de tratamiento

• Dosis altas de glucocorticoides sistémicos:
  • Es el pilar del tratamiento y debe iniciarse rápidamente si hay una fuerte sospecha de arteritis de células gigantes
  • Sin pérdida de visión: prednisona
  • Con pérdida de visión: metilprednisolona
  • Disminución lenta en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum un periodo de 6–12 meses
• Tratamiento coadyuvante:
  • Tocilizumab Tocilizumab Immunosuppressants: se utiliza como agente ahorrador de esteroides en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum combinación con glucocorticoides.
  • Metotrexato

Medidas generales

• Consulta con reumatología
• Seguimiento rutinario mensual:
  • Monitorizar los LOS Neisseria síntomas y realizar examen físico.
  • Tendencia de la VES y PCR PCR Polymerase chain reaction (PCR) is a technique that amplifies DNA fragments exponentially for analysis. The process is highly specific, allowing for the targeting of specific genomic sequences, even with minuscule sample amounts. The PCR cycles multiple times through 3 phases: denaturation of the template DNA, annealing of a specific primer to the individual DNA strands, and synthesis/elongation of new DNA molecules. Polymerase Chain Reaction (PCR)
• Tamizaje de tuberculosis Tuberculosis Tuberculosis (TB) is an infectious disease caused by Mycobacterium tuberculosis complex bacteria. The bacteria usually attack the lungs but can also damage other parts of the body. Approximately 30% of people around the world are infected with this pathogen, with the majority harboring a latent infection. Tuberculosis spreads through the air when a person with active pulmonary infection coughs or sneezes. Tuberculosis
• Vacunación contra la influenza Influenza Influenza viruses are members of the Orthomyxoviridae family and the causative organisms of influenza, a highly contagious febrile respiratory disease. There are 3 primary influenza viruses (A, B, and C) and various subtypes, which are classified based on their virulent surface antigens, hemagglutinin (HA) and neuraminidase (NA). Influenza typically presents with a fever, myalgia, headache, and symptoms of an upper respiratory infection. Influenza Viruses/Influenza y neumonía por neumococo
• Terapia antiplaquetaria en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum pacientes con aterosclerosis: Debe considerarse un inhibidor de la bomba de protones porque el uso de aspirina y glucocorticoides, y la edad aumentarán el riesgo de hemorragia gastrointestinal.
• Prevención de la osteoporosis Osteoporosis Osteoporosis refers to a decrease in bone mass and density leading to an increased number of fractures. There are 2 forms of osteoporosis: primary, which is commonly postmenopausal or senile; and secondary, which is a manifestation of immobilization, underlying medical disorders, or long-term use of certain medications. Osteoporosis con calcio y vitamina D
• Monitorizar la densidad ósea.

Diagnóstico Diferencial

• Accidente cerebrovascular: enfermedad en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum la que un flujo sanguíneo deficiente en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum el cerebro provoca la muerte de las células. Hay 2 tipos: accidente cerebrovascular isquémico, debido a la falta de irrigación sanguínea, y el accidente cerebrovascular hemorrágico, debido a una hemorragia. Los LOS Neisseria síntomas incluyen debilidad focal, entumecimiento, pérdida de la visión, disartria y cefalea. Para el diagnóstico se utilizan la tomografía computarizada y la RM del cerebro, que permiten diferenciar el accidente cerebrovascular de la arteritis de células gigantes.
• Migraña: se caracteriza por cefaleas recurrentes que van de moderados a graves, que suelen afectar a la mitad de la cabeza y que son de naturaleza pulsátil. Los LOS Neisseria síntomas asociados incluyen náuseas, vómitos y sensibilidad a la luz, sonidos u olores. El diagnóstico es clínico, basado en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum la historia y el examen físico. Los LOS Neisseria estudios de laboratorio y de imagenología no serán relevantes. Los LOS Neisseria antecedentes, el examen físico y los LOS Neisseria marcadores inflamatorios bajos ayudarán a diferenciar la migraña de la arteritis de células gigantes.
• Glaucoma Glaucoma Glaucoma is an optic neuropathy characterized by typical visual field defects and optic nerve atrophy seen as optic disc cupping on examination. The acute form of glaucoma is a medical emergency. Glaucoma is often, but not always, caused by increased intraocular pressure (IOP). Glaucoma: un aumento de la presión intraocular daña el nervio óptico y causa ceguera. Los LOS Neisseria síntomas del glaucoma Glaucoma Glaucoma is an optic neuropathy characterized by typical visual field defects and optic nerve atrophy seen as optic disc cupping on examination. The acute form of glaucoma is a medical emergency. Glaucoma is often, but not always, caused by increased intraocular pressure (IOP). Glaucoma de ángulo cerrado incluyen visión borrosa, halos alrededor de la luz, dolor Dolor Inflammation ocular intenso, cefalea, náuseas y vómitos. El diagnóstico se realiza mediante un examen oftalmológico y mediciones de la presión intraocular, que diferenciarán el glaucoma Glaucoma Glaucoma is an optic neuropathy characterized by typical visual field defects and optic nerve atrophy seen as optic disc cupping on examination. The acute form of glaucoma is a medical emergency. Glaucoma is often, but not always, caused by increased intraocular pressure (IOP). Glaucoma de la arteritis de células gigantes.
• Arteritis de Takayasu: vasculitis Vasculitis Inflammation of any one of the blood vessels, including the arteries; veins; and rest of the vasculature system in the body. Systemic Lupus Erythematosus granulomatosa de grandes vasos que afecta principalmente a la aorta Aorta The main trunk of the systemic arteries. Mediastinum and Great Vessels: Anatomy y sus ramas primarias. Los LOS Neisseria pacientes suelen ser mujeres jóvenes o de mediana edad. Los LOS Neisseria signos y síntomas incluyen sensibilidad de la arteria carótida, claudicación de las extremidades y ausencia o debilidad de los LOS Neisseria pulsos. Los LOS Neisseria marcadores inflamatorios elevados y la TC con angiografía de la aorta Aorta The main trunk of the systemic arteries. Mediastinum and Great Vessels: Anatomy pueden ayudar al AL Amyloidosis diagnóstico. El tratamiento incluye glucocorticoides y agentes ahorradores de glucocorticoides. Los LOS Neisseria datos demográficos del paciente, los LOS Neisseria antecedentes y los LOS Neisseria hallazgos del examen diferenciarán la arteritis de Takayasu de la arteritis de células gigantes.
• Neuropatía óptica isquémica anterior no arterítica: una lesión isquémica idiopática del nervio óptico. Los LOS Neisseria pacientes presentan una pérdida de visión repentina, indolora y monocular. Un examen fundoscópico mostrará una inflamación del disco óptico. La arteritis de células gigantes debe considerarse en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum estos individuos, y puede descartarse con marcadores inflamatorios normales. No se conoce ningún tratamiento eficaz, pero los LOS Neisseria pacientes suelen recibir aspirina.