ABSTRACT
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.
Subject(s)
Humans , Male , Female , Middle Aged , Aged , Aged, 80 and over , Giant Cell Arteritis/diagnosis , Giant Cell Arteritis/pathology , Giant Cell Arteritis/drug therapy , Steroids/therapeutic use , Temporal Arteries , Biopsy , C-Reactive Protein , Methotrexate/therapeutic use , Retrospective StudiesABSTRACT
La calcifilaxis se caracteriza por una intensa deposición de calcio en pequeños vasos sanguíneos, piel y otros órganos, descripta principalmente en pacientes con insuficiencia renal crónica, trasplante renal o disfunción paratiroidea. Hasta la fecha, solo hay siete casos descriptos en la literatura de calcifilaxis que imita arteritis de células gigantes (ACG). En esta revisión presentamos el octavo caso documentado patológicamente.
Calciphylaxis is characterized by intense deposition of calcium in small blood vessels, skin, and other organs, described mainly in patients with chronic renal insufficiency, renal transplant of parathyroid dysfunction. To date, there are only seven cases described in literature of calciphylaxis mimicking giant cell arteritis (GCA). In this review, we present the eighth case pathologically documented.
Subject(s)
Humans , Male , Middle Aged , Temporal Arteries/pathology , Giant Cell Arteritis/diagnosis , Calciphylaxis/diagnosis , Calciphylaxis/pathology , Diagnosis, DifferentialABSTRACT
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.
Subject(s)
Aged , Humans , Polymyalgia Rheumatica , Giant Cell Arteritis , Giant Cell Arteritis/diagnosis , Giant Cell Arteritis/drug therapy , Angiography , Tomography, X-Ray Computed , Positron Emission Tomography Computed TomographyABSTRACT
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.
Subject(s)
Humans , Female , Aged, 80 and over , Giant Cell Arteritis/complications , Giant Cell Arteritis/diagnosis , Giant Cell Arteritis/mortality , Health of the Elderly , Frail Elderly , Myocardial Infarction/mortalityABSTRACT
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.
Subject(s)
Humans , Female , Aged , Giant Cell Arteritis/pathology , Tongue Diseases/pathology , Giant Cell Arteritis/diagnosis , Giant Cell Arteritis/drug therapy , Tongue Diseases/diagnosis , Tongue Diseases/drug therapy , Diagnosis, Differential , Glucocorticoids/therapeutic use , NecrosisABSTRACT
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.
Subject(s)
Humans , Female , Aged , Arteries/pathology , Giant Cell Arteritis/diagnosis , Aortitis , Giant Cell Arteritis/drug therapy , Biopsy , Prednisone/therapeutic use , Glucocorticoids/therapeutic useABSTRACT
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.
Subject(s)
Humans , Autoimmune Diseases/complications , Connective Tissue Diseases/complications , Vasculitis/complications , Arthritis, Rheumatoid/complications , Arthritis, Rheumatoid/diagnosis , Arthritis, Rheumatoid/physiopathology , Autoimmune Diseases/diagnosis , Autoimmune Diseases/physiopathology , Behcet Syndrome/complications , Behcet Syndrome/diagnosis , Behcet Syndrome/physiopathology , Churg-Strauss Syndrome/complications , Churg-Strauss Syndrome/diagnosis , Churg-Strauss Syndrome/physiopathology , Connective Tissue Diseases/diagnosis , Connective Tissue Diseases/physiopathology , Giant Cell Arteritis/complications , Giant Cell Arteritis/diagnosis , Giant Cell Arteritis/physiopathology , Granulomatosis with Polyangiitis/complications , Granulomatosis with Polyangiitis/diagnosis , Granulomatosis with Polyangiitis/physiopathology , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/diagnosis , Lupus Erythematosus, Systemic/physiopathology , Polyarteritis Nodosa/complications , Polyarteritis Nodosa/diagnosis , Polyarteritis Nodosa/physiopathology , Scleroderma, Systemic/complications , Scleroderma, Systemic/diagnosis , Scleroderma, Systemic/physiopathology , Sjogren's Syndrome/complications , Sjogren's Syndrome/diagnosis , Sjogren's Syndrome/physiopathology , Takayasu Arteritis/complications , Takayasu Arteritis/diagnosis , Takayasu Arteritis/physiopathology , Vasculitis, Central Nervous System/complications , Vasculitis, Central Nervous System/diagnosis , Vasculitis, Central Nervous System/physiopathology , Vasculitis/diagnosis , Vasculitis/physiopathologyABSTRACT
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...
Subject(s)
Humans , Male , Adult , Anti-Inflammatory Agents , Giant Cell Arteritis/diagnosis , Giant Cell Arteritis/drug therapy , Prednisone/therapeutic useABSTRACT
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.
Subject(s)
Humans , Female , Aged, 80 and over , Giant Cell Arteritis/complications , Giant Cell Arteritis/diagnosis , Vision Disorders/etiology , Giant Cell Arteritis/pathology , Biopsy , Blood Sedimentation , C-Reactive Protein , Optic Neuropathy, Ischemic/complications , Vision Disorders/diagnosisABSTRACT
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.
Subject(s)
Aged , Aged, 80 and over , Biopsy , Diagnosis, Differential , Female , Giant Cell Arteritis/diagnosis , Giant Cell Arteritis/pathology , Humans , Male , Middle AgedABSTRACT
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.
Subject(s)
Humans , Giant Cell Arteritis/immunology , Giant Cell Arteritis/diagnosis , Giant Cell Arteritis/metabolism , Giant Cell Arteritis/drug therapy , Interferon-alpha/metabolism , /immunology , /metabolism , Macrophages/immunology , Macrophages/metabolism , Risk Factors , Signs and SymptomsABSTRACT
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.
Subject(s)
Humans , Male , Middle Aged , Cough/diagnosis , Giant Cell Arteritis/diagnosis , Cough/pathology , Diagnosis, Differential , Ear Diseases/diagnosis , Giant Cell Arteritis/pathology , Pain/diagnosisSubject(s)
Aged , Female , Humans , Giant Cell Arteritis/complications , Tongue Diseases/etiology , Tongue/pathology , Azathioprine/therapeutic use , Giant Cell Arteritis/diagnosis , Giant Cell Arteritis/drug therapy , Immunosuppressive Agents/therapeutic use , Necrosis/drug therapy , Necrosis/etiology , Prednisone/therapeutic use , Tongue Diseases/drug therapy , Tongue Diseases/pathologyABSTRACT
Temporal or giant cell arteritis is the most common vasculitis affecting the temporal artery. We encountered an unusual case of involvement of the temporal artery, which showed marked proliferating capillaries admixed with a dense infiltrate of eosinophils affecting all layers of the vessel. It was concluded that these changes were those of an epithelioid hemangioma of the temporal artery.
Subject(s)
Angiolymphoid Hyperplasia with Eosinophilia/diagnosis , Epithelioid Cells/pathology , Giant Cell Arteritis/diagnosis , Hemangioma/diagnosis , Humans , Male , Middle Aged , Temporal Arteries/pathologyABSTRACT
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
Subject(s)
Humans , Male , Aged , Amyloidosis/diagnosis , Multiple Myeloma/diagnosis , Temporal Arteries/pathology , Giant Cell Arteritis/diagnosis , Amyloidosis/etiology , Amyloidosis/pathology , Biopsy , Bone Marrow/pathology , Multiple Myeloma/complications , Multiple Myeloma/pathology , Giant Cell Arteritis/etiology , Giant Cell Arteritis/pathologyABSTRACT
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.