Simultaneous Unilateral Abducens Nerve Palsy and Contralateral Anterior Ischaemic Optic Neuropathy as the Presenting Signs of Giant Cell Arteritis.

Ischaemic optic neuropathy is the most common, feared, and recognised ocular manifestation of giant cell arteritis (GCA), while extraocular muscle palsy rarely occurs in the disease. Overlooking the diagnosis of GCA in aged patients with acquired diplopia and strabismus is not only sight- but also life-threatening. Here, we present, for the first time, a case of unilateral abducens nerve palsy and contralateral anterior ischaemic optic neuropathy as the presenting signs of GCA in a 98-year-old woman. Prompt diagnosis and treatment prevented further visual loss and systemic complications and allowed for rapid resolution of the abducens nerve palsy. We also aim to discuss the possible pathophysiological mechanisms of diplopia in GCA and to emphasise that acquired cranial nerve palsy must raise suspicion of this severe disease in elderly patients, particularly in association with ischaemic optic neuropathy.

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

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.

Incidence and Prevalence of Polymyalgia Rheumatica and Giant Cell Arteritis in a Healthcare Management Organization in Buenos Aires, Argentina.

OBJECTIVE: To estimate incidence and prevalence of polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) in a university hospital-based health management organization (Hospital Italiano Medical Care Program) in Argentina. METHODS: Overall and sex-specific incidence rates (IRs) and prevalence were calculated (age ≥ 50 yrs). Incidence study followed members with continuous affiliation ≥ 1 year from January 2000 to December 2015. Diagnosis as per the 2012 European Alliance of Associations for Rheumatology/American College of Rheumatology (ACR) criteria for PMR or the ACR 1990 criteria for GCA. Prevalence was calculated on January 1, 2015. RESULTS: There were 176,558 persons who contributed a total of 1,046,620 person-years (PY). Of these, 825 developed PMR, with an IR (per 100,000 PY) of 78.8 (95% CI 73.4-84.2) overall, 90.1 (95% CI 82.9-97.2) for women, and 58.9 (95% CI 51.1-66.6) for men. Ninety persons developed GCA; the IR was 8.6 (95% CI 6.8-10.4) overall, 11.1 (95% CI 8.5-10.6) for women, and 4.2 (2.2-6.3) for men. There were 205 prevalent PMR cases and 23 prevalent GCA cases identified from a population of 80,335. Prevalence of PMR was 255 per 100,000 (95% CI 220-290) overall, 280 (95% CI 234-325) for women, and 209 (95% CI 150-262) for men; and the prevalence of GCA was 28.6 per 100,000 (95% CI 16.9-40.3) overall, 36.4 (95% CI 20.1-52.8) for women, and 14.2 (95% CI 0.3-28.1) for men. CONCLUSION: This is the first study of incidence and prevalence of PMR and GCA in Argentina. There were similarities and differences with cohorts from other parts of the world, but population-based epidemiologic studies in Latin America are needed.

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.

Choroidal ischemia as one cardinal sign in giant cell arteritis.

PURPOSE: To describe chorioretinal signs in a case series of Giant Cell Arteritis (GCA). METHODS: This is a multicenter retrospective observational case series with GCA that presented with a headache and an abrupt, unilateral loss in vision. Workup included temporal artery biopsies, intravenous fluorescein angiography, optical coherence tomography (OCT), optical coherence tomography angiography (OCTA), blood levels of erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP). RESULTS: There are a total of 8 GCA instances presented. Average age was 74.5. (Range 68-83 years). The patients reported that one eye's visual loss had suddenly started, along with a fresh headache and other systemic symptoms. Eight patients exhibited choroidal ischemia, five paracentral acute middle maculopathy (PAMM) lesions, five cotton wool spots, four anterior ischemic optic neuropathy, and one central retinal arterial occlusion at the time of presentation. The average ESR at presentation was 68 mm/hr (range 4-110), and 4/6 individuals had a significant increase. The mean CRP level was 6.2 mg/dL (range 2.0-15.4), and the level was always over the normal range. All patients' temporal artery biopsies were positive. CONCLUSION: Alongside PAMM lesions, cotton wool spots, anterior ischemic optic neuropathy, and central retinal artery occlusion, choroidal ischemia is a key angiographic indicator in the diagnosis of GCA. It may be crucial to recognize these typical ischemic chorioretinal signs while diagnosing GCA.

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.

Temporal superficial arteritis as differential diagnosis in patients with atherosclerotic changes due to advanced chronic renal disease: case report and review of the literature.

Giant cell arteritis is an autoimmune disease that affects medium and large caliber vessels, creating deposits of inflammatory clusters on the arterial wall. It is the most common form of large vessel vasculitis, but given the variability of biopsy efficiency and of other diagnostic strategies employed, the diagnosis of this disease is challenging. We report the case of a 69-year-old female patient who presented with neurological deficit and increased bilateral sensation in the temporal region associated with excruciating headache. Workup revealed calcification of the superficial temporal, vertebral and ophthalmic arteries, as well as suggestive findings on Doppler ultrasound such as the halo sign, pointing to superficial temporal arteritis though not excluding the possibility of those calcifications being consistent with atherosclerosis in a patient with advanced chronic renal disease, which has been reported as giving rise to false-positive results. Knowledge of the main differences between the 2 diagnoses is important, given the wide range of diagnostic imaging possibilities which can avoid the need for biopsy.

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.

Polymyalgia rheumatica: A case series from Colombia and analysis of Latin America.

OBJECTIVE: Polymyalgia rheumatica (PMR) is the most common inflammatory disease in patients over 50 years. Information about the disease in Latin America (LATAM) is scarce. We aimed to evaluate a group of Colombian patients with PMR and to conduct a systematic review of PMR in LATAM. METHODS: A multicentric retrospective study was performed. Medical records of 256 PMR patients were evaluated. Patients were divided into two groups, those fulfilling the 2012 European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria for PMR and those who did not (i.e., clinical diagnosis). A systematic literature review and meta regression was performed comparing Colombian vs LATAM patients. RESULTS: From 256 patients, 145 (56.6%) fulfilled the 2012 EULAR/ACR criteria, and 111 (43.3%) were classified by clinical diagnosis. Inflammatory bilateral shoulder pain, pelvic girdle aching, morning stiffness >45 min, elevated erythrocyte sedimentation rate (ESR), and C-reactive protein (CPR), and Methotrexate (MTX) prescription were more common in the 2012 EULAR/ACR group. None of the included patients presented overt polyautoimmunity (PolyA), whereas up to 24% exhibited latent PolyA. In addition, these patients showed high frequency of malignancy (7.59%). In the meta regression analysis, Colombian patients exhibited lower ESR levels, and were less likely to develop giant cell arteritis (GCA) as compared to the rest of LATAM data. CONCLUSION: Patients with PMR in LATAM exhibit similar phenotypes from other cohorts worldwide. Malignancy, GCA and latent PolyA should be considered in the routine clinical follow-up of patients with PMR.

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.

Is the intima-media thickness of temporal and axillary arteries influenced by cardiovascular risk?

OBJECTIVES: To measure with US the intima-media thickness (IMT) of temporal (superficial, parietal and frontal branches) and axillary arteries in subjects without a diagnosis of GCA and/or PMR with different cardiovascular (CV) risk; and to assess the performance of previously proposed cut-off values for normal IMT. METHODS: Subjects ≥ 50 years without a diagnosis of GCA or PMR were included. Bilateral US of the temporal arteries, including the frontal and parietal branches, and axillary arteries was performed by two sonographers using a 10-22 MHz and 6-18 MHz probe. The following previously proposed cut-offs were considered: superficial temporal artery: 0.42 mm; frontal branch: 0.34 mm; parietal branch: 0.29 mm; axillary artery: 1.0 mm. RESULTS: A total of 808 arteries in 101 subjects were evaluated; of these, 31 (30.7%) were classified as very high CV risk, seven (6.9%) as high, 34 (33.7%) as moderate and 29 (28.7%) as low risk. Subjects with very high or high risk showed higher IMT than those with moderate or low risk in the superficial temporal arteries [0.23 (s.d. 0.07) vs 0.20 (s.d. 0.04), P < 0.01] and in the axillary arteries [0.54 (s.d. 0.17) vs 0.48 (s.d. 0.10), P = 0.002] . The IMT was higher than the reference cut-off in 13/808 (1.6%) arteries, in ≥1 artery in 10/101 subjects (10.1%). Of these 10 subjects, 8 (80%) were classified as having very high or high risk. CONCLUSION: Our results suggest that CV risk might influence the US-determined IMT of the temporal and axillary arteries in subjects without GCA. Therefore, in patients with suspected GCA, particular attention should be paid when measuring the IMT in those patients with very high/high CV risk.

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.

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.

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.

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.

Temporal arteritis, Sjögren's syndrome and mixed pain components as manifestations in a diabetic older adult: systematic review based on a case report.

Temporal arteritis (TA) is an inflammatory vascular disease common in the European population. It is mainly characterized by sudden onset headache. TA is rarely associated with other autoimmune diseases, such as Sjögren's syndrome (SS). We present the case of a Peruvian 71 year-old man with SS history, who was admitted to the emergency department due to severe headache evolved in 4 days, periocular pain and right ptosis. The authors also performed a systematic review of case reports or case series of patients diagnosed with both TA and SS. This temporal arteritis case is an atypical presentation because headache was characterized by mixed nociceptive and neuropathic pain components. Despite the infrequency, new studies should be carried out to identify comorbidities in TA patients.

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.