Accuracy of Doppler ultrasound in the diagnosis of giant cell arteritis: a systematic review and meta-analysis

Abstract Background Giant cell arteritis (GCA) is the most common primary systemic vasculitis in people 50 years of age and over, and it is considered a medical emergency due to the potential risk of permanent visual loss. Color Doppler ultrasound (CDU) of the temporal arteries is a rapid, noninvasive method to diagnose GCA. This study aims to determine the diagnostic accuracy of the halo sign in temporal arteries by CDU in people with suspected GCA. Methods The systematic literature review included the search for publications in the following electronic databases: PubMed, Embase, CENTRAL, LILACS, WHO ICTRP, ClinicalTrials.gov, gray literature up to December 2022, and no date or language restrictions were applied. We analyzed studies including patients over 50 years of age with suspected GCA evaluating CDU of temporal arteries as a diagnostic tool against clinical diagnosis as a standard reference. Paper titles and abstracts were selected by two investigators independently for all available records. The quality of the studies was assessed using the Quality of Diagnostic Accuracy Studies tool (QUADAS-2) and the R software (version 4.2.1) was used for data analysis. The protocol of this review is registered with PROSPERO (CRD42016033079). Results Twenty-two studies including 2893 participants with suspected GCA who underwent temporal artery CDU were evaluated. The primary analysis results showed a sensitivity of 0.76 [95% confidence interval (95 CI) 0.69-0.81] and specificity of 0.93 (95 CI 0.89-0.95) when the halo sign was compared to clinical diagnosis. The sensitivity value of 0.84 (95 CI 0.72-0.92) and specificity of 0.95 (95 CI 0.88-0.98) were found in five studies involving 1037 participants that analyzed the halo sign and temporal artery compression sign. A sensitivity of 0.86 (95 CI 0.78-0.91) and specificity of 0.95 (95 CI 0.89-0.98) were found in four studies with 603 participants where the halo sign was evaluated CDU on temporal and axillary arteries. Conclusion The detection of the halo sign by CDU of temporal arteries has good accuracy for the diagnosis of cranial GCA. The compression sign in temporal arteries and the addition of axillary arteries assessment improves the diagnostic performance of CDU for GCA. Trial registration PROSPERO CRD42016046860.

Recommendations of diagnosis and treatment of giant cell arteritis and polymyalgia rheumatic in China / 中华内科杂志

Polymyalgia rheumatica (PMR) is a syndrome characterized by pain and morning stiffness in the neck and shoulder and pelvic girdles, as well as raised acute-phase reactants, with or without systemic symptoms, such as fever. Giant cell arteritis (GCA) is a systemic vasculitis of unclear etiology that involves systemic arteries, principally affecting medium- and large-sized arteries with skipped, segmental alterations and granulomatous vasculitis seen on histopathology. In China, epidemiological data describing GCA are still limited; thus, the prevalence might be underestimated. The involvement of vessels in GCA can cause irreversible visual impairment or loss and stroke, which are serious complications. PMR is three times more prevalent than GCA, and other specific diseases should be excluded before the diagnosis is established. PMR symptoms can be present in 40%-60% of patients with GCA. Conversely, GCA can develop in 15% of patients with PMR. Chinese Rheumatology Association, based on the clinical diagnosis and treatment guidelines in 2005, utilizing the experience and guidelines of diagnosis and treatment at home and abroad, formulated this specification to standardize the diagnosis and treatment of GCA and PMR and improve the patient′s prognosis.

Tongue Necrosis due to Giant Cell Arteritis / Necrose da língua por arterite de células gigantes

Giant Cell Arteritis is a vasculitis that mainly affects women over 50. The most common manifestations are headache, jaw claudication, and amaurosis. If not diagnosed early, it can lead to rare irreversible ischemic consequences, with tongue necrosis being one of these. We report a case of a previously undiagnosed patient with lateral tongue necrosis who responded well to oral corticosteroid treatment. The diagnosis is clinical, laboratory and histological and may be aided by imaging exams. Initial treatment is with oral corticosteroids, with methotrexate and tocilizumab as alternatives. Diagnostic suspicion and quick start of treatment favorably influence the prognosis of the disease (AU)

Apresentamos um caso de arterite de células gigantes com amaurose, seguida de necrose da língua, diagnosticado clinicamente com boa resposta terapêutica ao corticoide oral. Salientamos que os casos de necrose da língua são extremamente raros. A suspeita diagnóstica de arterite de células gigantes é de fundamental importância e mesmo na impossibilidade de realizar a biópsia de artéria temporal, não se deve retardar o início do tratamento sob pena de acarretar sequelas definitivas ou manifestações mais graves da doença (AU)

Recomendaciones de la Sociedad Argentina de Reumatología en el manejo de la arteritis de células gigantes / Recommendations of the Argentine Society of Rheumatology in the management of giant cell arteritis

La arteritis de células gigantes (ACG) es una vasculitis sistémica que afecta a personas adultas; compromete vasos arteriales de mediano y gran calibre, con potenciales complicaciones de gravedad, como la ceguera, y es considerada una emergencia médica. El objetivo de estas guías fue desarrollar las primeras recomendaciones argentinas para su tratamiento, basadas en la revisión de la literatura mediante metodología GRADE. Un panel de expertos en vasculitis elaboró las preguntas en formato PICO (población, intervención, comparador y outcomes), y luego un panel de expertos en metodología efectuó la revisión de la bibliografía con la extracción de la evidencia para cada una de las preguntas. Se realizó un focus group de pacientes para conocer sus preferencias y experiencias. Finalmente, con la información recabada, el panel de expertos en vasculitis procedió a la votación de las recomendaciones que a continuación se presentan.

Giant cell arteritis (GCA) is a systemic vasculitis affecting adult patients and involving large and medium vessels. Potential serious complications as blindness may occur and it is considered a medical emergency. The objective of elaborating this guideline was to develop first Argentinian GCA treatment recommendations using GRADE methodology. An expert panel generated clinically meaningful questions addressing aspects of the treatment of GCA in the Population, Intervention, Comparator and Outcome (PICO) format and then a group of methodology experts reviewed and extracted data from literature summarizing available evidence. A patient's focus group discussion took place gathering information on their preferences and experiences. Finally, the vasculitis expert panel, with all the information obtained, voted recommendations here presented.

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.

Simultaneous Appearance of Arteritic Anterior Ischemic Optic Neuropathy and Central Retinal Artery Occlusion in Giant Cell Arteritis

@#Giant cell arteritis (GCA) is uncommon among Asian population. It is frequently associated with sight threatening complications. Simultaneous bilateral ocular involvement with different pathology is uncommon. We would like to highlight a rare case of GCA that was presented with transient visual loss over the right eye with simultaneous onset of central retinal artery occlusion as well as arteritic anterior ischemic optic neuropathy in both eyes. High dose intravenous methylprednisolone then subsequently maintenance dose of oral steroid and oral aspirin were given. His visual acuity remained the same after treatment. Early diagnosis and treatment of GCA is crucial. Visual outcome can be devastating if treatment is delayed.

Analysis of clinical characteristics and adverse events related factors in patients with giant cell arteritis / 中华风湿病学杂志

Objective:To identify the clinical characteristics and adverse events of patients with giant cell arteritis (GCA).Methods:Patients who were hospitalized and diagnosed with GCA in Zhongshan Hospital, Fudan University, from December 2009 to November 2020 were enrolled into a retrospective study analysis. Baseline data and follow-up data were collected. Adverse events were defined as one of the following events: relapses, ischemic complications and death. Patients with adverse events were analyzed in clinical features and risk factors by univariate and multivariate analysis. Associations with adverse events were assessed using ROC curve and survival curves.Results:A total of 69 patients with GCA were included in this study, with the male: female ratio of 1∶1.03. Fatigue and headache were common symptoms. Finally, 61 patients were followed up at the end of May in 2021. Over the mean follow-up time of (35±20) months, adverse events occurred in 16 cases (26.2%). Patients with adverse events had significantly lower levels of platelet and globulin at baseline than those without adverse events [(325±142)×10 9/L vs (238±112)×10 9/L, t=2.22, P=0.030]; [(31±6) g/L vs (26±6) g/L, t=2.74, P=0.008]. Red cell volume distribution width-coefficient of variation (RDW-CV) was considered an independent risk factor for adverse events [ OR (95% CI)=0.32 (0.14,0.74), P=0.008]. Further, patients especially with RDW-CV<14.75% were prone to have adverse events, which occurred in 2.6%, 20.5%, 25.6%, 33.3%, 41.00% in 1, 2, 3, 5, 10 years. Its risk increased significantly after 2 years ( P=0.042, P=0.021, P=0.002, P=0.001). The incidence of adverse events was much higher in patients with RDW-CV<14.75%(95% CI=0.002). Conclusion:Adverse events are common in patients with GCA. RDW-CV is an independent risk factor for having adverse events. Low level of RDW-CV predicts an increased risk of adverse events by the following years.

Ultrasonido Doppler de arterias temporales en pacientes con arteritis de células gigantes: estado del arte y revisión sistemática de la literatura / Temporal artery Doppler ultrasound in patients with giant cell arteritis: State of the art and systematic review of the literature

RESUMEN La arteritis de células gigantes es una vasculitis que afecta de manera predominante a vasos de gran calibre y aparece en personas mayores de 50 arios. Su presentación clínica incluye cefalea, alteraciones auditivas o síntomas similares a polimialgia reumática. En su forma más grave puede causar pérdida de visión uni- o bilateral, secundaria a neuropatía óptica isquémica de tipo arterítico. En la actualidad, el estándar de referencia para su diagnóstico es la biopsia de arterias temporales, procedimiento que no es inocuo y que puede tener como complicaciones infección, lesión nerviosa o sangrado, entre otras. Entre las técnicas no invasivas de diagnóstico, el ultrasonido y el Doppler de arterias temporales han tomado un rol cada vez más importante en el diagnóstico de esta entidad, dado que son pruebas benignas, con nulos efectos adversos y, a través de estrategias como las clínicas fast-track apoyadas en este método diagnóstico, se ha logrado reducir las complicaciones isquémicas de la enfermedad.

ABSTRACT Giant cell arteritis is a vasculitis that predominantly affects large calibre vessels, and usually appears in people over 50 years-old. Its clinical presentation includes headache, hearing impairment, or polymyalgia rheumatica-like symptoms. In its most severe form, it can cause uni- or bilateral vision loss secondary to arteritic ischaemic optic neuropathy. Currently, the gold standard for its diagnosis is the temporal artery biopsy, a procedure that is not harmless and may have complications such as infection, nerve injury, bleeding, among others. Among non-invasive diagnostic methods, the ultrasound and temporal artery Doppler have gained a predominant role in the diagnosis of giant cell arteritis, as it is a benign test with no adverse effects. Through strategies such as «fast-track¼ clinics, supported by this diagnostic method, a reduction has been achieved in ischaemic complications of the disease.

Calcifilaxis de la arteria temporal simulando arteritis temporal / Calciphylaxis of the temporal artery simulating temporal arteritis

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.

PET-CT FDG en el diagnóstico y seguimiento de vasculitis de grandes vasos / Positron emission tomography-CT scan (PET-CT) in the diagnosis of large vessel vasculitis

Vasculitides are a broad group of diseases that can involve any kind of vessel in any organ. These can be classified according to the size of the affected vessels. The most used classification categorizes them in small, medium, and large vessel vasculitis. Large vessel vasculitis can be further divided in Takayasu arteritis and giant cell arteritis which can sometimes be indistinguishable, even with biopsy. Radiology plays an important role identifying distribution patterns and disease extension18. Fluorine-Fluorodeoxyglucose (FDG) PET-CT shows increased vessel wall FDG uptake in patients with active large vessel vasculitis. Multiple studies show that FDG PET-CT helps to identify the anatomic structures with the disease, as well as evaluate its progression with a high sensibility and specificity in non-treated patients with large vessel vasculitis.

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.

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.

Ataque isquémico transitorio como manifestación inicial de arteritis de células gigantes: reporte de caso / Transient ischemic attacks initial manifestation of giant cell arteritis: case report

RESUMEN La arteritis de células gigantes es la vasculitis sistémica de medianos y grandes vasos más frecuente en adultos mayores de 50 años. Afecta de manera preferencial a las ramas de las arterias carótidas y vertebrales, pero también se han visto involucradas las arterias axilares, las femorales y las iliacas. Se reporta el caso de un paciente adulto que debutó con clínica de accidente isquémico transitorio asociado con cefalea frontotemporal de moderada intensidad persistente. A la exploración física se encontraba con arterias temporales prominentes y dolorosas, pero no refería síntomas o signos de claudicación mandibular ni de polimialgia reumática. Se confirmó el diagnóstico de arteritis de células gigantes mediante biopsia de arteria temporal y se instauró corticoterapia de forma temprana con buena respuesta al tratamiento. Los eventos isquémicos cerebrovasculares son una presentación relativamente poco frecuente y se han descrito clásicamente cuando ya se han iniciado síntomas de vasculitis. En este caso se presentó como manifestación inicial del periodo activo de la enfermedad, lo cual conlleva un reto diagnóstico que es importante tener en cuenta para iniciar las medidas terapéuticas de manera precoz y disminuir las complicaciones potencialmente graves asociadas. Dentro de tales medidas es preciso destacar el uso de agentes biológicos como el tocilizumab que como terapia adyuvante reduce el riesgo de recaída y la exposición acumulativa de corticoides, en comparación con la monoterapia con corticoides en ciertos casos de arteritis de células gigantes con complicaciones.

SUMMARY Giant cell arteritis is the most common systemic vasculitis of medium and great vessels in adults over 50 years of age. This involvement preferentially the branches of the carotid and vertebral arteries, but the axillary, femoral and iliac arteries have also been involved. The case of an adult patient who debuted with a transient ischemic accident clinic who associated manifestation of moderate persistent frontotemporal headache and the physical examination with prominent and painful temporal arteries is reported. No symptoms or signs of mandibular claudication or polymyalgia rheumatica are reported. The diagnosis of giant cell arteritis was confirmed by temporal artery biopsy and corticosteroid therapy was instituted early with a good response to treatment. Cerebrovascular ischemic events are a relatively rare presentation and have been classically described when symptoms of vasculitis have started, in this case they presented as the initial manifestation of the active period of the disease, which denotes a diagnostic challenge that is important to consider. to initiate therapeutic measures early and reduce associated potentially serious complications. Among these therapeutic measures, the use of biological agents such as Tocilizumab, which as adjuvant therapy reduces the risk of relapse and cumulative corticosteroid exposure compared to corticosteroid monotherapy in cases of giant cell arteritis with complications, should be highlighted.

Edema facial como manifestación inicial atípica de arteritis de células gigantes: a propósito de un caso y revisión de la literatura / Facial edema as an atypical initial manifestation of giant cell arteritis: a case report and review of the literature

Introducción: la arteritis de células gigantes es la vasculitis de vaso grande más frecuente y se ve predominantemente en adultos ma-yores de 50 años. El diagnóstico es en base a la clínica que se compone de cefalea, polimialgia reumática, sensibilidad en relación a la arteria temporal, compromiso del estado general, entre otras cosas, más laboratorio que se evidencia reactantes de fase aguda elevados y anemia y se confirma con biopsia de arteria temporal. Métodos: reporte de un caso de una paciente con debut con cefalea y aumento de volumen facial, lo que conllevó un diagnóstico erróneo de celulitis facial. Debido a esta presentación atípica, se retrasó el diagnóstico de arteritis de la temporal y tratamiento oportuno. Resultados: la arteritis de células gigantes es una patología que posee un gran rango de presentaciones atípicas, lo que ocurre en hasta un 38% de los pacientes que poseen la enfermedad, manifestaciones que incluyen neuralgia del trigémino, infartos linguales, aneurismas aórticos, edema facial, entre otros. Conclusión: es muy relevante conocer las presentaciones atípicas de esta patología que son muy frecuentes de encontrar en los pacientes y conocerlas nos permite aumentar nuestra sospecha clínica permitiendo un diagnóstico y tratamiento oportuno, evitando consecuencias irreversibles por el retraso diagnóstico.

Introduction: giant cell arteritis is the most frequent large vessel vasculitis and is seen predominantly in adults over 50 years. The diag-nosis is based on the clinic that is composed of headache, polymyalgia rheumatic, sensitivity near the temporal artery, compromise of the general condition, among other things, added to a laboratory that is evidenced like severe acute phase reactants and anemia and finally, is confirmed with temporal artery biopsy. Methods: a case report of a patient who debuted with headache and increased facial volume that led to a wrong diagnosis of facial cellulite. Because of this atypical presentation of the disease, the diagnostic took more time than usual and delayed the accurate diagnosis and timely treatment; this could have caused irreversible consequences. Results:giant cell arteritis has a wide range of atypical presentations; this may occur even up to 38% of patients that have this disease; manifes-tations include: trigeminal neuralgia, lingual infarct, aortic aneurysm, facial edema, and other symptoms. Conclusion: it is important to study the atypical presentations of this pathology because they are usually founded in patients. If we are informed about the atypical presentations, we can increase our clinical suspicion, and that allows us to get the right diagnosis and opportune treatment, avoiding irreversible consequences because of a late diagnosis.

Aortitis: Hallazgos radiológicos en relación a sus diversas etiologías / Aortitis: Radiological Findings in Relation to its Various Etiologies

Resumen La inflamación de la aorta (aortitis) es una patología poco frecuente, con etiología infecciosa (pseudoaneurisma micótico, sífilis) y no infecciosa (arteritis, aortitis idiopática, espondilitis anquilosante, entre otras) de difícil diagnóstico clínico y variable pronóstico. Por esa razón, la utilización de diversos métodos por imágenes, tales como la tomografía computada multidetector (TCMD), la tomografía computada por emisión de positrones (PET-TC), la resonancia magnética (RM) y ultrasonido (US) facilitan la identificación, seguimiento y tratamiento de esa entidad. El siguiente trabajo tiene como objetivo realizar una revisión y actualización bibliográfica acerca de la aortitis y sus diversas etiologías, ejemplificando con casos de nuestra institución.

Abstract Aortic inflammation (aortitis) is a rare pathology, with infectious (fungal pseudoaneurysm, syphilis) and noninfectious etiology (arteritis, idiopathic aortitis, ankylosing spondylitis, among others), it has a difficult clinical diagnosis and a variable prognosis. The use of various imaging methods such as multidetector computed tomography (MDCT), magnetic resonance imaging (MRI), positron emission tomography-computed tomography (PET-CT) and ultrasound (US) facilitate the identification, monitoring and treatment of this entity. The following paper aims to perform a literature review and update about aortitis and its various etiologies, exemplifying cases of our institution.

Giant cell arteritis secondary to combined nivolumab and ipilimumab in metastatic pleural mesothelioma

@#We report a rare case of isolated giant cell arteritis caused by combined immunotherapy with nivolumab and ipilimumab in metastatic pleural mesothelioma. Whilst combination immunotherapy is thought to provide synergistic anti-tumor effects in metastatic malignancies, it has also been associated with an increased frequency of severe immune-related adverse effects. To our knowledge, giant cell arteritis has been described in only four previous cases in relation to single agent immunotherapy, and our patient is the first reported case of isolated giant cell arteritis from combination immunotherapy. This report adds to the literature a rare case of an important adverse effect that clinicians should be aware of, especially with increasing use of combination immunotherapy

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.

Vasculitis del tronco celíaco como presentación de arteritis de Takayasu / Vasculitis of the celiac artery as a presentation of Takayasu's arteritis

La arteritis de Takayasu es una vasculitis idiopática y granulomatosa crónica que se manifiesta en forma de panaortitis, de etiología desconocida, aunque se postula un origen autoinmune. Es progresiva y genera, tanto en adultos como en niños, estenosis segmentaria, oclusión, dilatación y/o aneurismas. La vasculitis aislada gastrointestinal sin afectación sistémica es rara. Este caso lleva a tener en cuenta la manifestación abdominal atípica de la arteritis de Takayasu en el diagnóstico diferencial de un síntoma frecuente, como la epigastralgia, y a destacar el rol que ocupan en la actualidad los métodos de imágenes no invasivos para su diagnóstico.

Takayasu arteritis is an idiopathic and chronic granulomatous vasculitis manifested in the form of panaortitis, of unknown etiology, even though an autoimmune origin is postulated. It is progressive and generates, in adults and children, segmental stenosis, occlusion, dilation and / or aneurysms. Isolated gastrointestinal vasculitis without systemic involvement is rare. This case leads us to take into account the atypical abdominal manifestation of Takayasu arteritis in the differential diagnosis of a frequent symptom, such as epigastralgia, and to highlight the role currently played by non-invasive imaging methods for its diagnosis.

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.