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A 74-year-old woman presented with fever, jaw claudication and bilateral temporal pain lasting for 1 week. On examination, the bilateral temporal arteries (TAs) were nodularly swollen, tender and hypopulsatile ([figure 1A][1]). A blood test showed elevated white cell count (9.33×109/L), high

A 75-year-old Caucasian woman presented with sudden-onset multifocal scotomas in her right eye's central vision for 1 day. There were subtle white intraretinal foveal lesions that correlated with patchy inner retinal hyperreflectivity on optical coherence tomography, suggestive of paracentral acute middle maculopathy. Initial cerebrovascular work-up was negative. Review of systems was positive for lethargy and jaw claudication. The sedimentation rate and c-reactive protein were elevated, but platelet count was normal. The patient was started on 60 mg oral prednisone daily and underwent bilateral temporal artery that confirmed the diagnosis of giant cell arteritis.

Clinicians usually easily recognize cranial manifestations of giant cell arteritis (GCA) such as new-onset headache, jaw claudication, scalp tenderness, and abrupt changes in visual acuity or blindness; however, when presented with an aberrant clinical course, the diagnosis becomes more elusive. In addition to temporal arteries and other extracranial branches of the carotid arteries, large vessel vasculitis (LVV) can also affect other blood vessels including coronary arteries, aorta with its major branches, intracranial blood vessels, and hepatic arteries.Over time, the scope of the symptoms typically associated with LVV has broadened and includes cases of fever of unknown origin accompanied with other constitutional symptoms that can mimic a range of neoplastic and infectious diseases. In up to half of patients with atypical LVV, liver enzyme level elevations with a cholestatic pattern have been observed. Alkaline phosphatase level and γ-glutamyl transferase level elevations tend to be more prevalent in those LVV patients with vigorous inflammatory responses, particularly in those with fever and other nonspecific constitutional symptoms. These patients also have more profound anemia and thrombocytosis. With the exception of rare instances of vasculitides and granulomas affecting the liver tissue, liver biopsy is generally of little help and primarily shows nonspecific changes of fatty liver.In this article, we review 3 patients who were eventually diagnosed with atypical LVV. The diagnosis was confirmed with temporal artery biopsy in 2 patients and with positron emission tomography/computed tomography in 1 patient. The common hepatic abnormality observed in all patients was the elevation of alkaline phosphatase level, which tended to respond rapidly to initiation of immunosuppressive treatment.

A 47-year-old man with a history of COVID-19 infection 2 months before presentation, presented with a scotoma of the paracentral visual field of the right eye. After thorough testing and evaluation, a diagnosis of paracentral acute middle maculopathy (PAMM) was established. Two months later, the patient developed temporal headache and jaw claudication. High-dose steroids were initiated, and workup for giant cell arteritis (GCA) was undertaken. The patient experienced resolution of the symptoms within 24 hours of steroid initiation. ESR, CRP, and temporal artery biopsy results were normal, although all were obtained more than 2 weeks after steroid initiation. To the best of our knowledge, our patient represents the first individual to date to potentially implicate COVID-19 in both small and large vessel vasculitis in the ophthalmic setting.

espanolLas vasculitis de gran vaso afectan predominantemente a la arteria aorta y sus ramas y se clasifican en dos entidades principales: la arteritis de celulas gigantes (ACG) y la arteritis de Takayasu (ATK). La ACG suele presentarse con cefalea temporal de reciente comienzo, claudicacion mandibular e hipersensibilidad del cuero cabelludo. Algunos pacientes presentaran tambien sintomas visuales (amaurosis fugaz y neuritis optica anterior) y manifestaciones sistemicas como la polimialgia reumatica, la fiebre y la perdida de peso. La biopsia de arteria temporal es una ayuda importante en su diagnostico. Otras herramientas son la ecografia de arterias temporales, la tomografia por emision de positrones combinada con tomografia computarizada (PET-TC) y la angiorresonancia magnetica (RM). Los glucocorticoides (GC) son el pilar del tratamiento de la ACG, reservando la asociacion de tocilizumab o metotrexato para pacientes con recurrencias o con toxicidad importante relacionada con los corticoides. La aparicion de sintomas en la ATK tiende a ser subaguda, lo que a menudo conduce a un retraso diagnostico. El diagnostico clinico de la ATK puede ser realizado en base a hallazgos clinicos sugestivos (sintomas constitucionales, carotidinia, hipertension, pulsos disminuidos o ausentes y/o soplos arteriales en pacientes de menos de 40 anos) y una prueba de imagen que muestre el estrechamiento vascular de la aorta y/o sus ramas primarias. La angio-TC ayuda a detectar complicaciones de la vasculitis y la PET/TC al evaluar la respuesta al tratamiento. El pilar del tratamiento de la ATK son los GC sistemicos, recomendando la asociacion precoz de un farmaco ahorrador de esteroides debido a la alta tasa de recaida. EnglishLarge-vessel vasculitis predominantly affects the aorta and its branches and is classified into two main entities: giant cell arteritis (GCA) and Takayasu arteritis (TKA). GCA usually presents with new-onset temporal headache, jaw claudication and scalp hypersensitivity. Some patients also have visual symptoms (amaurosis fugax and anterior optic neuritis) and systemic manifestations such as polymyalgia rheumatica, fever and weight loss. Temporal artery biopsy is an important diagnostic aid. Other tools include temporal artery ultrasound, positron emission tomography combined with computed tomography (PET-CT) and magnetic resonance angiography (MRA). Glucocorticoids (GC) are the mainstay of treatment for GCA, reserving combining with tocilizumab or methotrexate for patients with recurrences or significant corticosteroid-related toxicity. The onset of symptoms in TKA tends to be subacute, often resulting in diagnostic delay. A clinical diagnosis of TKA can be based on suggestive clinical findings (constitutional symptoms, carotid artery disease, hypertension, decreased or absent pulses and/or arterial murmurs in patients under 40 years of age) and an imaging test showing vascular narrowing of the aorta and/or its primary branches. Angio-CT helps to detect complications of vasculitis and PET/CT to assess response to treatment. Systemic GCs are the mainstay of treatment for TKA, and the early combination of a steroid-sparing drug due to the high rate of relapse.

A 79-year-old woman was admitted for suspected giant cell arteritis (GCA). She had suffered from dizziness, headache, jaw claudication and visual disturbance. Her medical history included bronchial asthma and parasinusitis. Her superficial temporal arteries were markedly enlarged with tenderness. Laboratory data showed eosinophilia (6968/µL) and a positive result of myeloperoxidase-ANCA. A histological examination of the biopsied artery revealed granulomatous inflammation consisting of lymphocytes and eosinophils with a multinucleated giant cell. Her conditions met both the criteria for GCA and eosinophilic granulomatosis with polyangiitis (EGPA). We finally considered that she had temporal arteritis as an initial manifestation of EGPA after a comprehensive literature review. To our knowledge, this is the first case in which temporal arteritis with a giant cell developed as an initial and sole manifestation of EGPA.

Objectives Imaging modalities have become common in evaluating patients for a possible diagnosis of GCA. This study seeks to contextualize how temporal arterial magnetic resonance angiography (TA-MRA) can be used in facilitating the diagnosis of GCA. Methods A retrospective cohort study was performed on patients who had been previously referred to a rheumatologist for evaluation of possible GCA in Hamilton, Ontario, Canada. Data including clinical features, inflammatory markers, imaging, and biopsy results were extracted. Multivariable logistic regression model to predict the diagnosis of GCA. Using these models, the utility of TA-MRA in series with or in parallel to clinical evaluation was demonstrated across the cohort as well as in subgroups defined by biopsy and imaging status. Results In total 268 patients had complete data. Those diagnosed with biopsy- and/or imaging-positive GCA were more likely to demonstrate classic features including jaw claudication and vision loss. Clinical multivariable modelling allowed for fair discriminability [receiver operating characteristic (ROC) 0.759, 95% CI: 0.703, 0.815] for diagnosing GCA; there was excellent discriminability in facilitating the diagnosis of biopsy-positive GCA (ROC 0.949, 0.898–1.000). When used in those with a pre-test probability of 50% or higher, TA-MRA had a positive predictive value of 93.0%; in those with a pre-test probability of 25% or less TA-MRA had a negative predictive value of 89.5%. Conclusion In those with high disease probability, TA-MRA can effectively rule in disease (and replace temporal artery biopsy). In those with low to medium probability, TA-MRA can help rule out the disease, but this continues to be a challenging diagnostic population.

Objective: This study aims to determine the clinical, laboratory, and ultrasonographic findings of giant cell arteritis in patients with Herpes Zoster. Methods: The study included 36 consecutive patients (median age 59.0 years; range 19 to 76 years) who were admitted to the Dermatology Outpatient Clinic with the diagnosis of Herpes Zoster. Demographic and clinical features of the patients were recorded. The presence of ultrasonographic characteristics of giant cell arteritis such as halo sign, compression sign, occlusion, and stenosis was also recorded using ultrasound. The patients were evaluated at baseline and 6 months. Results: A total of 36 patients were assessed. 4 patients had jaw claudication (11.1%), 5 patients had scalp tenderness (13.9%), 11 patients had a new-onset headache (30.6%) and, 23 patients had post-herpetic neuralgia (63.9%). No patients had elevated erythrocyte sedimentation rate and ultrasonographic findings of Giant cell arteritis. Conclusion: Our data show that a small proportion of patients with Herpes Zoster may have clinical findings suggesting Giant cell arteritis. However, they do not have elevated erythrocyte sedimentation rate and sonographic findings of Giant cell arteritis.

A 67-year-old Caucasian female presented in August 2019 to our rheumatology service, with 3 days history of severe neck pain and right-sided headache with aches in both shoulders and arms and mild stiffness. Other symptoms included mild jaw claudication. She had recently returned from Majorca after an uneventful two-week trip. She had a background of severe allergic asthma and allergic rhinitis, well controlled with omalizumab which was started in 2016, based on persistently high IgE. Her sister suffers from a type of vasculitis and is currently on steroids. The patient is an ex-smoker and drinks two bottles of wine a week. She had high inflammatory markers with raised eosinophilic count and was admitted for further work up to rule out infection and to commence steroid after for a likely diagnosis of eosinophilic granulomatosis with polyangiitis. Shortly after admission to the acute assessment unit, she became confused and febrile. An extensive work up ruled out infection, and she was started on steroids and treated for acute hyponatremia. Omalizumab was stopped. She improved and was discharged on a tapering dose of steroids and was weaned off completely within 4 months. Her inflammatory markers returned to normal as well as her eosinophilic count, with complete resolution of her presenting symptoms.

Background: Giant cell arteritis is an immune-mediated, medium to large vessel vasculopathy affecting individuals over 50 years of age. It can cause sudden, severe and irreversible loss of vision, most commonly from an arteritic posterior ciliary artery occlusion causing anterior ischaemic optic neuropathy. The optic nerve appearance would typically be swollen and chalky white. Visual reduction secondary to choroidal ischaemia is a much less frequent presentation, the signs of which can be more subtle in appearance, making its early recognition potentially more challenging. Case Presentation: A 51-year-old male presented to eye casualty complaining of a one-week history of neck pain, intermittent headaches and jaw claudication, associated with reduced vision in his right eye. Presenting visual acuity was hand movements and 6/5 in the right and left eyes respectively. On examination, he was noted to have a right relative afferent pupillary defect, a pale macular area in the right eye with a possible cherry red spot. There was no optic disc swelling. ESR was 34 and CRP was 46 and he was wrongly diagnosed with a non-arteritic central retinal artery occlusion. He subsequently re-presented 5 weeks later with vision loss in his left eye. Best corrected visual acuity was now 6/60 and 6/12 in the right and left eyes respectively. Dilated fundoscopy showed multiple yellow-white lesions in the posterior pole of the left eye and a retinal cotton wool spot. The right optic nerve was pale, and left was normal. A fundus fluorescein angiogram showed delayed choroidal filling and the temporal artery biopsy was suggestive of GCA. He was started on 110mgs of oral prednisolone. After 4 weeks of steroids his BCVA was 6/36 and 6/6 in the right and left eyes respectively. His neck pain, headaches and jaw claudication had all resolved and his ESR and CRP had returned to normal levels. Conclusion: Our case highlights the need for increased awareness of this uncommon presentation of this potentially blinding disease, to allow prompt and appropriate treatment. Our case is unusual in that despite a delayed diagnosis of 5 weeks, visual acuity initially improved with treatment.

Background: Giant Cell Arteritis (GCA), is the most common primary vasculitis. It affects large vessels such as the aorta and its branches. According to Chapel Hill Consensus, GCA is one of the larger vessel vasculitis. The underlying mechanism involves inflammation of the large arteries. The most frequent presentation consists of headache, polymyalgia, and jaw claudication. GCA can put the visual prognosis at risk, and rapid diagnosis is compulsory. Cotton wool spots, due to focal inner retinal ischemia, are an early diagnostic ophthalmological sign. The most frequent presentation is a rapid, partial or complete blindness. However, atypical presentations, such as uveitis, especially in the anterior chamber, can delay diagnosis. Case Report: We report a 75-year-old woman with GCA who initially presented with anterior uveitis and without any other clinical sign. At the beginning, there was the only ophthalmic sign and systemic inflammation, the all exhaustive work-up including positron emission tomography (PET) scan was negative. The biology was fully normal without auto-immune profile (Angiotensin converting enzyme level, Interferon Gamma Release Assay, Syphilis serology, antinuclear antibody titer, Rheumatoid factor, CCP antibodies, and chest x-ray were normal. HLA B27 was negative). In the following weeks, she subsequently developed large vessel vasculitis with headache and more typical sign. She developed cotton wool spots linked to retinal arteriolar hypoperfusion. Anterior uveitis has been reported rarely in GCA and moreover, it is very uncommon at the early stages of GCA. Our case stresses that uveitis onset can precede large vessels vasculitis and typical symptoms of GCA. PET-scan is a useful tool for atypical GCA, but its sensitivity is not perfect, and its repetition can be helpful in selected cases such as that of this patient.

Giant cell arteritis (GCA) is a granulomatosis accompanied by angiitis that affects medium- and large-sized arteries. We present a case of GCA in a patient who presented with jaw claudication and occlusal pain. A 78-year-old man visited our department with left-sided facial pain associated with mastication. He also reported spontaneous left-sided facial pain and diminished vision. Based on his age, his new-onset left-sided headache, and a high erythrocyte sedimentation rate, he was diagnosed with GCA with immediate initiation of prednisolone (PSL) therapy. His fever subsided the following day. A temporal artery biopsy (TAB) was performed 7 days after hospitalization and showed a false-negative result. His left-sided facial pain and headache subsided 29 days after therapy initiation. He was diagnosed with bladder cancer 3 months later, and we considered a probable association between his left-sided facial pain and paraneoplastic syndrome. GCA was diagnosed clinically based on the American College of Rheumatology criteria. Prompt PSL treatment is warranted without waiting for TAB results to avoid visual loss or other complications in this patient population.

Was ist neu? EULAR-Empfehlungen zur Anwendung von Bildgebung Sofern mit hoher Expertise und rasch verfügbar, wird in erster Linie eine frühzeitige Bildgebung zur Ergänzung der klinischen Kriterien für die Diagnose der RZA empfohlen. Aktualisierte EULAR-Empfehlungen zum Management Für Patienten mit RZA-Verdacht wird eine Überweisung an ein spezialisiertes Team/Zentrum empfohlen; die RZA-Verdachtsdiagnose sollte dann weiter mittels Bildgebung oder Temporalarterienbiopsie bestätigt werden. Glukokortikoide (GC) sind weiterhin Medikamente der ersten Wahl. Für ausgewählte Patienten wird eine additive Therapie mit Tocilizumab empfohlen; alternativ zu Tocilizumab kann Methotrexat eingesetzt werden. Zum routinemäßigen Einsatz von Thrombozytenaggregationshemmern oder Antikoagulanzien wird nicht mehr geraten. Update Tocilizumab bei Riesenzellarteriitis Der Interleukin-6-Rezeptor-Antagonist kann in initialer Kombination mit GC effektiv eine anhaltende Remission bei der RZA induzieren. Bei Absetzen einer Tocilizumab-Therapie nach einem Behandlungszeitraum von 1 Jahr besteht bei ca. der Hälfte der Patienten die Remission fort.

Background Giant cell arteritis has a wide variety of clinical symptoms, one of them being cervical radiculopathy, which mainly involves the C5 nerve root. If the patient does not develop typical clinical symptoms of giant cell arteritis but has C5 radiculopathy, it may be misdiagnosed as polymyalgia rheumatica or elderly-onset rheumatoid arthritis due to old age, high serum inflammatory markers, and difficulty in raising both upper limbs. Case presentation A 72-year-old Japanese man with a month-long history of dyspnea on exertion and with difficulty in raising both upper limbs was referred to our hospital because of elevated serum C-reactive protein (12.62 mg/dL). He had no typical symptoms of giant cell arteritis such as headache, jaw claudication, visual loss, and fever. The patient tested negative for rheumatoid factor and anti-cyclic citrullinated peptide antibody, and matrix metalloproteinase-3 was within the normal range (54.3 ng/mL). Musculoskeletal ultrasound examination showed absence of tenosynovitis, bursitis, and synovitis, and the patient did not meet the classification criteria of polymyalgia rheumatica or rheumatoid arthritis; hence, those two diseases were unlikely. A precise neurological examination suggested bilateral C5 and C6 anterior radiculopathy and left C4 radiculopathy. Since cervical magnetic resonance imaging showed no mechanical causality, cervical radiculopathy of unknown origin was suggested. Fluorodeoxyglucose positron emission tomography/computed tomography revealed increased fluorodeoxyglucose lineal uptake along the vessel walls, including temporal arteries, vertebral arteries, and axillary arteries. Results of the biopsy of the left superficial temporal artery were compatible with giant cell arteritis. He was successfully treated with 30 mg of prednisolone, and both upper limbs could be elevated. Conclusions If the patient was misdiagnosed with polymyalgia rheumatica or elderly-onset rheumatoid arthritis based on only clinical symptoms and laboratory data, his symptoms might not improve due to insufficient steroid dose and vascular complications may occur later. Although rare, peripheral neuropathy in giant cell arteritis may include cervical radiculopathy. The musculoskeletal ultrasound and precise neurological examination were the turning points for the diagnosis of this case, and making a careful diagnosis using these methods was important.

Case report - Introduction Giant cell arteritis (GCA) is a systemic vasculitis primarily affecting large- and medium-sized arteries. Classic symptoms include headache, scalp tenderness, jaw claudication and visual disturbances. Ophthalmic artery involvement commonly causes anterior ischaemic optic neuropathy. Uncommon ocular features include anterior segment ischaemia, hypotony, tonic pupil or rarely choroidal ischaemia. Heterogenicity of presentation can make diagnosis difficult leading irreversible visual loss. We report a case of bilateral macular choroidal ischaemia with atypical symptoms of GCA. It emphasises the need of complete evaluation in elderly patients with GCA and visual symptoms and the need to start aggressive treatment to prevent visual loss. Case report - Case description 73-year-old caucasian lady presented to the eye emergency department with diplopia. Medical history includes systemic hypertension, hypothyroidism and hyperlipidaemia, no past ocular history. Eye examination was normal except decompensated fourth nerve paresis. Thyroid function was normal. Diplopia resolved spontaneously. Patient re-presented with a floater in the right eye and left-sided atypical headache without jaw claudication. Investigations: normal FBC, CRP 126, ESR 100, PV 1.67. Following rheumatologist review she was commenced on oral prednisolone 60 mg with clinical suspicion of GCA. Temporal artery biopsy confirmed GCA. She had TIA subsequently. MRI revealed small area of acute infarct in left ganglio-capsular region. Clopidogrel was started for secondary prevention. In the ophthalmology clinic she saw a lacy pattern. Her Log MAR VA in right and left eye was 0.64 and 0.76, respectively. Fundoscopy revealed retinal pigment epithelial (RPE) mottling at the maculae, right more than left eye. Optical Coherence Tomography (OCT) macula revealed bilateral RPE elevations and serous pigment epithelial detachment bilaterally, patchy central RPE atrophy with external limiting membrane disruption, more pronounced in the right eye. Fundus fluorescein angiogram and indocyanine green angiography confirmed bilateral choroidal ischaemia (triangular shaped with the base at the equator) at the macula worse in right than left eye. Oral prednisolone was continued with gradual tapering. VA improved to Log MAR 0.5 and Log MAR 0.2 in right and left eye at six weeks. OCT showed signs of RPE re-modelling with resolution of sub retinal fluid (resolution of inflammation). At recent follow up Log MAR VA is 0.26 and 0.06 in right and left eye respectively. She is on oral prednisolone 20 mg once a day tapering 2.5 mg every 2 weeks. OCT shows further re-modelling of the ellipsoid zone in the left eye, but her right eye shows more RPE atrophy and thinning with RPE degeneration. Case report - Discussion We report an unusual case of GCA with atypical symptoms and bilateral choroidal ischaemia. Patients with GCA usually present with systemic symptoms and signs like headache, scalp tenderness, fever, and jaw claudication. Variable presentation can often lead to misdiagnosis and consequent irreversible loss of vision. Visual symptoms as the first and only sign of GCA was first reported in 1952. Posterior ciliary arteries in the eye can be affected leading to optic nerve infarction and subsequent anterior ischaemic optic neuropathy (AION). AION and visual field loss accounts for 80—90% of cases with ocular signs of GCA. Posterior ciliary artery occlusion can rarely cause patches of choroidal infarcts which appear as chorio-retinal degeneration in a couple of weeks. These patches are usually in the mid-peripheral fundus, usually triangular shaped with the base towards equator and apex toward posterior pole. In our case the presentation was very atypical in the sequence of symptoms. Her raised inflammatory markers raised the suspicion of GCA and prompt referral to rheumatology was done. Aggressive treatment with oral steroids was started with stomach and bone protection. Temporal artery biopsy confirmed the diagnosis. The bilateral triangular ischaemic areas found on FFA and ICG confirmed the macular choroidal ischemia. Her OCT also showed bilateral RPE mottling showing degenerative changes due to choroidal infarct from posterior ciliary artery occlusion. We managed to preserve the vision in our case by starting the timely aggressive steroid treatment. In summary, we report an unusual case of GCA with atypical symptoms and bilateral choroidal ischaemia where further visual loss was avoided due to timely intervention. GCA has variety of presentations; a combined team approach of ophthalmologists and rheumatologists can prevent irreversible visual loss in such cases. Case report - Key learning points GCA is a chronic idiopathic inflammation more commonly seen in the large- and medium-sized vessels. Posterior ciliary arteries in the eye can be affected in GCA leading to optic nerve infarction and subsequent anterior ischaemic optic neuropathy (AION). AION and visual field loss accounts for 80—90% of cases with ocular signs of GCA. Posterior ciliary artery occlusion can rarely cause patches of choroidal infarcts which appear as chorio-retinal degeneration in a couple of weeks. These patches are usually in the mid-peripheral fundus, usually triangular shaped with the base towards equator and apex toward posterior pole. Prompt diagnosis and aggressive treatment with corticosteroids can prevent visual loss in one or both eyes. Any patient over 50 years of age presenting with visual symptoms of amaurosis fugax, diplopia, or visual loss with ocular signs of anterior or posterior ischaemic optic neuropathy, central retinal artery occlusion or cilioretinal artery occlusion should create a high suspicion for GCA. This group of patients should have urgent ESR, CRP and PV evaluation. If suspected, high-dose corticosteroids must be started followed by temporal artery biopsy for confirmation. It is imperative to diagnose GCA early and start treatment urgently to prevent visual loss. A multidisciplinary team approach in patients with GCA can prevent sight loss and life too.

To report a case of simultaneous bilateral ophthalmic artery occlusion in diagnosed giant cell arteritis (GCA).A 77-year-old male patient presented to the emergency department with simultaneous vision loss in both eyes for 3 hours. Headache at both temples and jaw claudication had been present for 3 weeks. Laboratory values demonstrated an initially increased C-reactive protein (CRP) of 202.0 mg/L and an erythrocyte sedimentation rate (ESR) of 100 mm within the first 20 minutes. Duplex sonography of the right and left temporal arteries revealed a "halo sign." A case of GCA was suspected, and intravenous high-dose methylprednisolone therapy was immediately administered. The clinical examination revealed a bilateral central retinal artery occlusion and fluorescein angiography showed a hot optic disc in the right eye and patchy choroidal hypoperfusion in both eyes. Biopsy of the left temporal artery was performed, which confirmed a florid temporal arteritis with complete thrombotic occlusion of the vascular lumen. Despite a good response to the administered therapy (CRP 17.0 mg/L 1 week after initiation), the visual prognosis was significantly limited through retinal and optic nerve involvement. By the follow-up examination 8 weeks later, the near visual acuity was 20/400 in the right and left eye at a distance of 16 inches.We hereby present a simultaneous bilateral ophthalmic artery occlusion as a rare complication of GCA. The combination of central retinal artery occlusion, arteritic anterior ischemic optic neuropathy, and choroidal hypoperfusion suggests an acute inflammatory involvement of the ophthalmic artery. In cases of the slightest suspicion of giant cell arteritis, an immediate high-dose steroid therapy initiation is of utmost importance.Wir berichten über einen Patienten mit gleichzeitig aufgetretenem Verschluss beider Aa. ophthalmicae bei diagnostizierter Riesenzellarteriitis.Ein 77-jähriger männlicher Patient stellte sich in der Notaufnahme mit gleichzeitigem Sehverlust auf beiden Augen vor, der seit 3 Stunden bestehe. Kopf- und Kauschmerzen waren seit 3 Wochen vorhanden. Die Laborwerte zeigten ein initial erhöhtes C-reaktives Protein (CRP) von 202,0 mg/l und eine Erythrozytensedimentationsrate (ESR) von 100 mm innerhalb der ersten 20 Minuten. Die Duplexsonografie der rechten und linken A. temporalis zeigte ein „Halo-Zeichen“ und bei Verdacht einer Riesenzellarteriitis wurde eine sofortige intravenöse hochdosierte Methylprednisolon-Therapie eingeleitet. Die klinische Untersuchung ergab einen beidseitigen Zentralarterienverschluss und die Fluoreszenzangiografie zeigte eine Leckage der Papille des rechten Auges sowie eine choroidale Hypoperfusion an beiden Augen. Es wurde eine Biopsie der linken Temporalarterie durchgeführt, die eine floride Arteriitis temporalis mit vollständigem thrombotischem Verschluss des Gefäßlumens bestätigte. Trotz eines guten Ansprechens auf die verabreichte Therapie (CRP 17,0 mg/l 1 Woche nach Beginn) war die Sehprognose durch Netzhaut- und Sehnervenbeteiligung deutlich eingeschränkt. Bei der Nachuntersuchung 8 Wochen später lag der Nahvisus bei 1/20 bei der Lesetafel am rechten und linken Auge.Wir stellen hier einen gleichzeitigen bilateralen Verschluss der A. ophthalmica als seltene Komplikation der Riesenzellarteriitis vor. Die Kombination aus Zentralarterienverschluss, arteriitischer anteriorer ischämischer Optikusneuropathie und choroidaler Hypoperfusion lässt auf eine akute entzündliche Beteiligung der A. ophthalmica schließen. Bei dem geringsten Verdacht auf eine Riesenzellarteriitis ist die sofortige Einleitung einer hochdosierten Steroidtherapie von größter Bedeutung.

Giant cell arteritis (GCA), also known as temporal arteritis, is the most common systemic inflammatory vasculitis occurring in the elderly. Patients usually present with vision loss, headache, jaw claudication, diplopia, myalgia and constitutional symptoms. The most common ocular manifestations are anterior ischemic optic neuropathy (AION), choroidal ischemia and central retinal artery occlusion. Here we describe a case of GCA presenting with vision changes secondary to retinal detachment and vitreous hemorrhage. Upon temporal artery biopsy, GCA was confirmed and it was found to be accompanied by temporal artery dissection as well. Patient was treated with pulse dose intravenous steroids after which her symptoms improved. Based on our literature review, this is the first reported case of GCA presenting in this manner.

Case Presentation A 56-year-old man presented to the pulmonary clinic with dyspnea and hypoxemia on exertion. He was an avid biker and skier who had noticed a significant decrease in high-level physical activity over the past 3 years. He reported dyspnea, desaturations at altitudes higher than 9,000 feet, dry cough, tachycardia, and palpitations with exercise. Review of systems was also notable for gluten-intolerance, Raynaud’s phenomenon, recurrent skin lesions and joint swelling, pain, and stiffness in the areas overlying the jaw, wrists, knees, and ankles (after capsaicin exposure). He denied fever, chills, anorexia, weight loss, hair loss, ocular symptoms, jaw claudication, chest pain, or lower extremity swelling. He had a five pack-year smoking history, no history of prematurity, childhood asthma, recurrent infections, or environmental and occupational exposure. Based on pulmonary function tests from an outside provider, he had received a diagnosis of exercise-induced asthma and had been prescribed an albuterol inhaler to use on an as-needed basis, which failed to improve his symptoms. He was later prescribed a mometasone-formoterol inhaler, still with no symptomatic improvement.

Background/Aims Giant cell arteritis (GCA) is a large vessel vasculitis mainly affecting the arteries of the head and neck which, if untreated, may lead to permanent vision loss. Glucocorticoids are highly effective at turning off inflammation but come with toxic side effects, making prompt diagnosis essential. There is no gold-standard investigation for GCA. Although specific, temporal artery biopsy (TAB) is only positive in approximately 25% of cases making it problematic as a rule-in diagnostic test. Vascular ultrasound may aid diagnosis but there is a rapid reduction in sensitivity with glucocorticoid use and it is not yet universally available. Diagnosis therefore requires the integration of clinical judgment with blood tests measuring inflammation, imaging, and biopsy. Aims:Identify which components of the history, examination and laboratory findings are most predictive of a positive diagnosis of GCA in the local region of Yeovil District Hospital, and to investigate the usefulness of alternative blood biomarkers. Methods Data was collected from GCA clinic attendances between August 2018 and February 2020 using electronic notes, clinic letters and the pathology system. Predictive values, sensitivity, specificity, and receiver operating characteristic (ROC) curves were calculated for each individual parameter and for groups of parameters. Results Ninety-one patients presented to GCA clinic in the 18 months studied. Median age was 71 and 73% were female. 56 patients with suspected disease went on to have TAB, of which 38/56 (68%) were of adequate length ( > =10mm), and of those, 12/38 (32%) confirmed a diagnosis of GCA. 43/91 (47%) patients were ultimately diagnosed biopsy proven or suspected GCA. Headache was the most common presenting feature (88%) followed by raised ESR (55%), raised CRP (53%), visual disturbance (44%), scalp tenderness (33%), jaw claudication (31%), PMR symptoms (27%) and temporal artery abnormalities (20%). Headache and raised CRP+/-ESR were the most sensitive markers (91% and 100%, respectively). They were, however, the least specific (4% and 36%). Temporal artery abnormality was the most specific finding (81%). ROC analysis revealed that the best-performing biomarkers were monocytes (area under the ROC curve (AUC) of 0.81) and platelets (AUC 0.80), which were superior to jaw claudication, the best-performing classical biomarker (AUC 0.68). Platelets above 450 x 109/L had a specificity of 96% with a likelihood ratio of 10.9. Monocytes above 0.45 x 106/L had sensitivity and specificity of 100% and 67%, respectively. Conclusion GCA cannot be accurately predicted by any single feature. In this cohort, absence of headache with a normal CRP+/-ESR ruled out GCA. Platelets and monocytes performed better than all the classical parameters associated with GCA. Validation of these biomarkers in a larger cohort is now needed to ascertain cut-off points which may help to develop a more accurate method to predict cases of GCA. Disclosure T. Somoskeoy: None. A. Bourn: None. S. Knights: None. B. Mulhearn: None.

Giant cell arteritis (GCA) is a medium-to-large vessel vasculitis of the elderly. Common constitutional clinical features include headache, scalp tenderness, and jaw claudication. Severe unilateral or bilateral visual loss is the most feared ophthalmic complication of GCA. Scalp necrosis is a known ischemic complication of GCA with approximately 100 cases reported in the literature to date. We report a case of scalp pain and an erythematous cutaneous lesion in the distribution of ophthalmic division of the trigeminal nerve that mimicked herpes zoster ophthalmicus. A temporal artery biopsy was positive for GCA, and small vessel arteritis was seen at the time of simultaneous skin biopsy. To the best of our knowledge, this is the first such report in the English language ophthalmic literature.

A 55-year-old obese woman with a history of migraine and tension-type headaches presented with headache for 5 days with acute worsening for 1 day. The headache was throbbing, holocephalic, and maximal at the left temple, and worsened with both standing and lying down and with exertion. The headache was initially mild for 4 days before acutely worsening in severity and waking her from sleep with associated nausea and photophobia. Unlike her typical headaches, it did not improve with over-the-counter analgesics and was more prolonged. There were no associated visual complaints, hearing changes or phonophobia, jaw claudication, weakness, numbness, or paresthesias.

A 78-year-old woman with fever of unknown origin that had persisted for 3 months, systemic edema, and cervical lymphadenopathy was admitted to our hospital. Skin purpura and jaw claudication were subsequently observed. Histopathological examinations of the lymph nodes, skin, and temporal artery revealed findings characteristic of eosinophilic granulomatosis with polyangiitis (EGPA). However, she had no past medical history of asthma with modest eosinophilia. Although EGPA is a systemic vasculitis characterized by asthma and eosinophilia, various limited forms have been described. This was therefore considered to be an atypical form of non-asthmatic EGPA complicating with temporal arteritis (TA) diagnosed by tissue biopsy.

Giant Cell Arteritis (GCA) is a common systemic vasculitis that involves medium and large arteries, most frequently extracranial branches of the carotid artery. Prompt diagnosis and treatment is essential to prevent devastating complications, such as blindness, stroke and aortic aneurysm or dissection.Typical symptoms include headache, temporal artery tenderness and jaw claudication. On rare occasions patients present with complaints related to pulmonary involvement. These include interstitial infiltration, pulmonary nodules, pleural effusion and pulmonary haemorrhage.The authors report the case of a 74-year-old man who presented with symptoms compatible with GCA confirmed by temporal biopsy, and pulmonary nodules. These lesions were biopsied and histological exam confirmed features compatible with systemic vasculitis. The lesions resolved after two months of steroid treatment.This case shows that we should be aware of atypical GCA manifestations. Keywords: Giant Cell Arteritis, Temporal Arteritis, Horton's disease, Systemic vasculitis, Large-vessel vasculitis, Pulmonary nodules

Purpose To develop and validate neural network (NN) vs logistic regression (LR) diagnostic prediction models in patients with suspected giant cell arteritis (GCA). Design: Multicenter retrospective chart review. Methods An audit of consecutive patients undergoing temporal artery biopsy (TABx) for suspected GCA was conducted at 14 international medical centers. The outcome variable was biopsy-proven GCA. The predictor variables were age, gender, headache, clinical temporal artery abnormality, jaw claudication, vision loss, diplopia, erythrocyte sedimentation rate, C-reactive protein, and platelet level. The data were divided into three groups to train, validate, and test the models. The NN model with the lowest false-negative rate was chosen. Internal and external validations were performed. Results Of 1,833 patients who underwent TABx, there was complete information on 1,201 patients, 300 (25%) of whom had a positive TABx. On multivariable LR age, platelets, jaw claudication, vision loss, log C-reactive protein, log erythrocyte sedimentation rate, headache, and clinical temporal artery abnormality were statistically significant predictors of a positive TABx (P≤0.05). The area under the receiver operating characteristic curve/Hosmer-Lemeshow P for LR was 0.867 (95% CI, 0.794, 0.917)/0.119 vs NN 0.860 (95% CI, 0.786, 0.911)/0.805, with no statistically significant difference of the area under the curves (P=0.316). The misclassification rate/false-negative rate of LR was 20.6%/47.5% vs 18.1%/30.5% for NN. Missing data analysis did not change the results. Conclusion Statistical models can aid in the triage of patients with suspected GCA. Misclassification remains a concern, but cutoff values for 95% and 99% sensitivities are provided (https://goo.gl/THCnuU).

An 80-year-old man with medical history of hypertension developed a sudden loss of vision in the left eye without any associated pain, flashes, or floaters. The review of systems was negative for any headaches, jaw claudication, scalp or temporal tenderness, or other symptoms suggestive of polymyalgia rheumatica (PMR). The patient's medications were benazepril and amlodipine for the treatment of hypertension. Clinical examination showed normal vital signs. Visual acuity testing revealed 20/20 vision on the right and hand movement perception only in the temporal field of the left eye, with no light perception on the nasal field of the same eye. Pupils were 3 mm bilaterally, round, and reactive with a relative afferent pupillary defect on the left. Dilated funduscopic examination was unremarkable on the right side and revealed macular whitening and retinal blanching with cherry-red spot on the left. Temporal artery pulses were present. The remainder of the neurologic examination was unremarkable. Laboratory investigations including erythrocyte sedimentation rate, C-reactive protein, lipid profile, and glycosylated hemoglobin were normal.

Background Giant cell arteritis (GCA) is the most common form of systemic vasculitis in persons aged 50 years and older. Medium and large vessels, like the temporal and axillary arteries, are commonly affected. Typical symptoms are headache, scalp tenderness, jaw claudication and ophthalmological symptoms as loss of visual field, diplopia or amaurosis due to optic nerve ischemia. Tongue pain due to vasculitic affection of the deep lingual artery can occur and has so far not been visualized and followed up by modern ultrasound. Case presentation We report the case of a 78-year-old woman with typical symptoms of GCA, such as scalp tenderness, jaw claudication and loss of visual field, as well as severe tongue pain. Broad vasculitic affection of the extracranial arteries, vasculitis of the central retinal artery and the deep lingual artery could be visualized by ultrasound. Further did we observe a relevant decrease of intima-media thickness (IMT) values of all arteries assessed by ultrasound during follow-up. Especially the left common superficial temporal artery showed a relevant decrease of IMT from 0.49 mm at time of diagnosis to 0.23 mm on 6-months follow-up. This is the first GCA case described in literature, in which vasculitis of the central retinal artery and the lingual artery could be visualized at diagnosis and during follow-up using high-resolution ultrasound. Conclusion High-resolution ultrasound can be a useful diagnostic imaging modality in diagnosis and follow-up of GCA, even in small arteries like the lingual artery or central retinal artery. Ultrasound of the central retinal artery could be an important imaging tool in identifying suspected vasculitic affection of the central retinal artery.

The coronavirus disease 2019 has displayed multi-system manifestations since its first presentation. This article highlights an unusual presentation of COVID-19 that was reviewed by our instituition's otolaryngology and ophthalmology team.We present 2 cases of COVID-19 which presented with unilateral otalgia and ipsilateral pulsatile headaches involving the temporal area. They were referred to the otolaryngology team for assessment of otalgia and subsequently referred to the ophthalmology team for possible giant cell arteritis (GCA). Both patients had no jaw claudication, scalp pain, or tenderness.Serology testing showed raised C-reactive protein (CRP) but normal platelets and erythrocyte sedimentation rate. Case 1 was tested for COVID-19 as part of a preoperative workup which returned positive. With a marked similarity in presentation, Case 2 was tested for COVID-19 which also returned positive.These 2 cases highlight another set of symptoms that COVID-19 patients may present with. In the context of a COVID-19 pandemic, if a patient presents symptoms similar to GCA but with isolated CRP, it should prompt consideration for COVID testing.

Giant cell arteritis is a large-vessel vasculitis. It is exceptional before 50 years of age, while its incidence increases with advancing age. There is a female predominance, with females being two to three times more frequently affected than males. The hallmark clinical feature of giant cell arteritis is headache. Visual loss, related to vasculitis of the posterior ciliary or, less commonly, the retinal arteries occurs in about one-sixth of patients with giant cell arteritis, usually before treatment with glucocorticoids is started, while jaw claudication is described by half of patients. In some patients, systemic symptoms predominate and may be the only manifestation.

Giant cell arteritis (GCA), or Horton’s arteritis, presenting solely as fever is very rare. Usually, it manifests with typical features such as visual problems, headache and jaw claudication, or it can be associated with polymyalgia rheumatica. We describe the case of a patient with GCA who presented only with prolonged fever, the cause of which was not determined by diagnostic tests. LEARNING POINTS: Fever may be the only symptom of giant cell arteritis (GCA). It is important to consider GCA in the differential diagnosis of fever of unknown origin as early diagnosis is crucial for prompt treatment and to prevent catastrophic complications such as vision loss or stroke. Temporal artery biopsy remains the gold standard for diagnosing GCA.

To provide an overview of mimickers of large vessel vasculitis (LVV), by the main presenting manifestation, i.e., systemic, vascular, and cranial manifestations. The main differential diagnoses in patients with giant cell arteritis (GCA) and Takayasu arteritis (TAK) presenting with systemic manifestations (i.e., fever, anorexia, weight loss, night sweats, arthralgia/myalgia, and/or increased inflammatory indexes) are neoplastic, infectious, or other inflammatory conditions. In patients with vascular manifestations (such as peripheral ischemia, vascular stenoses, or aneurysms), atherosclerosis and non-inflammatory vascular diseases should be excluded. In those presenting with predominant cranial symptoms (i.e., temporal headache, jaw claudication, scalp tenderness, transient or permanent vision loss), other causes of headache, cerebrovascular accidents, optic neuropathy, and neuromuscular syndromes need to be considered. The diagnosis of LVV maybe challenging, especially when patients present with atypical or incomplete clinical forms. In these cases, a multidisciplinary approach is strongly recommended.

Background:EULAR recommends doppler ultrasound (US) as the first line imaging in patients with Giant Cell Arteritis (GCA) suspect. Traditionally, US non-compressive halo sign has been used for diagnosis but prospective studies on response and disease monitoring are lackingObjectives:The HAS GCA study has the objective of prospectively assessing role of US in diagnosis, prognosis and monitoring in newly diagnosed GCA. We report early baseline and up to month 3 data on our current recruitment in a study that has suffered disruption from the pandemicMethods:HAS GCA (IRAS#264294) is an ongoing, prospective, multicentre study recruiting from referrals of suspected GCA to fast track clinics. The objective is to recruit 270 patients, including 68 GCA patients. Based on the Southend GCA clinical pre-test probability score (SPTPS)1, patients were stratified in to low, intermediate and high risk categories2. Temporal and axillary US Halo Scores were calculated from the halo thickness and extent in bilateral temporal arteries, parietal and frontal branches and axillary arteries. These individual scores were summed (TA Halo Score x1 plus; AA Halo Score x3) to generate a Total Halo Score (THS)3.Mann Whitney U test and Fisher’s exact test were used to compare baseline features between GCA and controls. Wilcoxon signed rank test was used to evaluate disease features at baseline and at 3 months in GCA patients. Sensitivity (Sn) and Specificity (Sp) were calculated, where applicable. P value Results:Ninety-three patients (29 GCA, 64 controls) have been recruited thus far: 18 completed 3-month follow up assessment; 4 were lost to follow up (2 died, 2 withdrew consent due to pandemic). Demographics, clinical features, and US results are shown (Table 1).Table 1.Baseline features of GCA patients and controls.GCA (n=29)Controls (n=64)P-valueAge, median (IQR)75 (71-80)67 (61.25 – 75.0)0.001Female, n (%)15 (42)50 (78)0.01SPTPS category, n (%) Low risk0 (0)31 (48) Intermediate risk7 (24)25 (39)0.24 High risk22 (76)8 (13)Halo score (HS), median (range) Temporal artery HS10 (1-21)1 (0-9) Axillary artery HS12 (0-18)6 (0-18) Total HS21 (2-38)6 (0-19)Clinical features, n (%) Temporal headache21 (72)40 (63)0.48 Scalp tenderness17 (59)31 (48)0.38 Jaw claudication19 (66)4 (6) PMR symptoms16 (55)6 (9) Constitutional symptoms17 (59)18 (28)0.006 Visual disturbance18 (62)38 (59)1 Vision loss7 (24)4 (6)0.03Among GCA patients, 23 had cranial, 2 large-vessel and 4 mixed phenotypes (cranial plus large vessel) disease.Jaw claudication (66%) and polymyalgic symptoms (55%) were the dominant features in GCA patients. Median age 75 years in GCA (42% females) and 67 years in controls (78% females). GCA and controls were stratified by SPTPS to Low risk (0% vs 48%; Sn-undefined, Sp-97), Intermediate risk (24% vs 39%; Sn-100, Sp-100) and High risk (76% vs 13%; Sn-95, Sp-88). Optimal SPTPS cut-off point was ≥12 (Sn-93, Sp-86); ≥10 (Sn-100 & Sp-69).Median THS was 21 in GCA and 6 in controls. Optimal cut-off Halo Score in diagnosis was TAHS ≥5 (Sn-90, Sp-98), AAHS ≥11 (Sn-55, Sp-80), THS ≥18 (Sn-72%, Sp-98%). Among the 18 patients who completed 3-months follow up, median TAHS, AAHS and THS reduced from 10 to 2.5, 12 to 6 and 21 to 10, respectively (Figure 1).Conclusion:Along with SPTPS, Halo Score successfully discriminates GCA from non GCA mimics. HS is effective in showing 3-month response and may be a useful marker to monitor GCA disease activity.References:[1]Laskou F et al. A probability score to aid the diagnosis of suspected giant cell arteritis. Clin Exp Rheumatol. 2019[2]Sebastian A et al. Probability-based algorithm using ultrasound and additional tests for suspected GCA in a fast-track clinic. RMD Open. 2020[3]Sebastian A et al. Halo score (temporal artery, its branches and axillary artery) as a diagnostic, prognostic and disease monitoring tool for Giant Cell Arteritis (GCA). BMC Rheumatol. 2020Disclosure of Interests:Alwin Sebastian: None declared, Alessandro Tomelleri: None declared, Abdul Kayani: None declared, Mohammad Tariq: None declared, Diana Prieto-Peña: None declared, Sue Inness: None declared, Jo Jackson: None declared, Kornelis van der Geest Speakers bureau: Roche, Bhaskar Dasgupta Speakers bureau: Roche, GSK, BMS, Sanofi, Abbie, Grant/research support from: Roche

Background:Giant cell arteritis (GCA) is a systemic large vessel vasculitis that results in symptoms (e.g., headaches, jaw claudication, fatigue, pain, etc.) that can have multiple negative impacts on an individual’s health-related quality of life.Objectives:To identify important symptoms and impacts experienced by individuals with GCA, develop a conceptual model, and evaluate available patient reported outcome (PRO) measures for appropriateness for inclusion in GCA clinical studies.Methods:Published literature on disease experience from databases, conference abstracts, and Guidance/OMERACT documents were reviewed. A GCA conceptual model was developed based on the symptoms and impacts identified from this literature and supplemented with clinical input. A detailed review of selected PROs was then conducted to assess concept coverage and gaps to evaluate appropriateness for a GCA population.Results:Of 245 identified citations, 24 abstracts and 18 articles were reviewed. Key symptoms affecting GCA patients (e.g., ischemic and constitutional symptoms) and impacts to the lives of GCA patients (e.g., reduced physical, social and emotional functioning) formed the basis for a GCA conceptual model (see Figure 1).No GCA-specific PROs were identified. Therefore, the PROs that were used most frequently in GCA clinical studies were reviewed and evaluated. These included the SF-36, FACIT-Fatigue, Patient Global Assessment (PtGA), Multidimensional Fatigue Inventory (MFI), EQ-5D, Health Assessment Questionnaire (HAQ), Brief Illness Perception Questionnaire (BIPQ), and Visual Functioning Questionnaire-25 (VFQ-25). These PROs included some key impacts but did not assess most GCA-specific symptoms (e.g., headaches, fever, etc.). No measures were developed with input from GCA patients and there has been limited evaluation of the measurement properties of these PROs in GCA patients.The FACIT-Fatigue, SF-36, and the PtGA, which were frequently administered in published GCA studies, measure key GCA concepts such as fatigue, functioning, impact on daily activities and disease status. The measurement properties (reliability, validity, sensitivity to change) have been documented in several other rheumatologic diseases (Table 1).Conclusion:In the absence of a GCA-specific PRO, the SF-36, FACIT- Fatigue, and PtGA are recommended for use in GCA clinical studies for the assessment of key concepts identified in the literature. Qualitative and quantitative research should be conducted with GCA patients to assess the content validity and measurement properties of these PROs in a GCA population.Table 1.Summary of Recommended MeasuresDescriptionSF-36FACIT-FatiguePtGATypeGeneric, health status measureFatigue and impact on function/daily activitiesMeasure of disease activityMethod of AdministrationSelf/InterviewerSelf/InterviewerSelf# of Items36131Recall periodPast 4 weeks/past weekPast 7 daysVariesScoring8 domain scores; 2 component summary scoresTotal scoreTotal scoreMeasurement propertiesMeasurement properties demonstrated in other rheumatologic diseases (SLE, RA, SpA, and chronic knee pain); construct validity demonstrated in a single study with GCA patientsMeasurement properties demonstrated in rheumatologic populations including SLE, PsA, and RASelect measurement properties have been demonstrated in GCA and other populations (RA, TAK, RP)Concept coverageGCA impacts (emotional, physical, and social functioning, and daily activities), bodily pain, fatigueFatigueOverall assessment of disease activityAdministered in recent GCA studiesYESYESYESSLE= systemic lupus erythematosus, SpA = spondyloarthritis, PsA=psoriatic arthritis, RA=rheumatoid arthritis, GCA= giant cell arteritis, TAK=Takayasu’s arteritis, RP=relapsing polychondritisDisclosure of Interests:Hilary H Colwell Consultant of: Janssen Pharmaceutical Companies of Johnson & Johnson, Susan Mathias Consultant of: Janssen Pharmaceutical Companies of Johnson & Johnson, Kelly Kato Employee of: Janssen Pharmaceutical Companies of Johnson & Johnson, Catherine E Najem Shareholder of: Johnson & Johnson, Employee of: Janssen Pharmaceutical Companies of Johnson & Johnson, Amy M Archer Employee of: Janssen Pharmaceutical Companies of Johnson & Johnson

Background Giant cell arteritis (GCA) is the most common systemic vasculitis in the American population older than 50 years and is a sight-threatening and life-threatening disease. It is definitively diagnosed with a temporal artery biopsy. Although there are many studies focusing on the clinical presentation and laboratory values in diagnosing GCA in the general population, studies focusing on the veteran population are lacking. This is the first study describing the diagnostic features of GCA in the US military veterans. Methods We performed a retrospective chart review in the Veterans Information Systems and Technology Architecture Computerized Patient Record System (CPRS 1.0, Department of Veterans Affairs Health Data Systems). Anatomic pathology reports from temporal artery biopsies (TABs) were collected, as well as the clinical presentation and laboratory values for each case. Frequency, sensitivity, and specificity were calculated for clinical variables, such as new-onset headache and vision changes, including diplopia, ischemic vision loss/optic disc disease, and amaurosis fugax. A logistic regression (LR) prediction model was then developed to compare veteran risk factors with those of the general population. Results Of 292 patients, 40 had positive TABs (13.7%). The average age of subjects with positive TABs was 73 ± 8.8 years (mean ± SD). The average erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) in patients with positive TABs (69.1 mm/hr and 56.6 mg/L, respectively) were significantly higher than ESR and CRP in patients with negative TABs (50.5 mm/hr, P = 0.0016 and 32.2 mg/L, P = 0.0394, respectively). Mean platelet levels were much higher (317.6 × 10/L) in patients with positive TABs than platelet levels in those with negative TABs (260.6 × 10/L, P = 0.0005). CRP was the most sensitive variable at 83.3%, followed by ESR with a sensitivity of 80% and new-onset headache with a sensitivity of 62.5%. Jaw claudication and polymyalgia rheumatica (PMR) were most specific (81.3% and 89.3%, respectively). Headache was the most common presenting symptom overall (71.6%), followed by vision changes (50.3%), scalp tenderness (25.7%), jaw claudication (20.9%), and PMR-related symptoms (12.7%). The LR prediction model included scalp tenderness, log (CRP), log (platelets), vision changes, and age, with 50% sensitivity and 88.36% specificity. Platelets (odds ratio [OR] = 4.309, P = 0.049), CRP (OR = 1.504, P = 0.022) and scalp tenderness (OR = 3.860, P = 0.016) were statistically significant predictors of a positive TAB in this population. Conclusions Veterans Administration (VA) patients present with symptomatology similar to that of the general population. A positive biopsy was found in female veterans more frequently than in their male counterparts. Platelet count and scalp tenderness were most predictive. Our LR model provided a highly specific method for detecting GCA in the veteran population at this institution, but further studies are needed to determine the generalizability of the model. This retrospective study serves as a basis for future multicenter VA-wide studies to characterize the unique features in this population.

A 72-year-old man presented with a short history of headache, jaw claudication, double vision, amaurosis fugax and distended temporal arteries. A diagnosis of giant cell arteritis (GCA) was confirmed on temporal artery ultrasound and temporal artery biopsy. Despite treatment with high-dose oral glucocorticoid (GC) and multiple pulses of intravenous methylprednisolone, his vision deteriorated to hand movements in one eye. 8 mg/kg intravenous tocilizumab, a humanised, recombinant anti-IL-6 receptor antibody, was administered within 48 hours of vision loss and continued monthly, resulting in marked visual improvement within days, as well as sustained remission of GCA. This case suggests a possible role for tocilizumab as a rescue therapy to prevent or recover visual loss in patients with GCA resistant to GC treatment, termed refractory GCA. Further research is required to elucidate the role of intravenous administration of tocilizumab in this setting.

Polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) are related inflammatory diseases of adults aged 50 years or older. The diagnosis of PMR is based on morning stiffness, proximal shoulder and pelvic girdle pain, and functional impairment. GCA is characterized by headache, jaw claudication, and visual disturbances. Constitutional symptoms and elevated inflammatory markers are common to both conditions. Temporal artery biopsy remains the gold standard for diagnosis of GCA. Glucocorticoids are the cornerstone of therapy, with tapering regimens individualized to the patient. Prompt diagnosis and treatment are essential to avert vision loss in GCA. Tocilizumab increases remission rates in GCA.

Objectives To determine the role of the ocular pulse amplitude (OPA) from Pascal dynamic contour tonometry in predicting the temporal artery biopsy (TABx) result in patients with suspected giant cell arteritis (GCA). Design Prospective validation study. Participants Adults aged 50 years or older who underwent TABx from March 2015 to April 2017. Methods Subjects on high-dose glucocorticoids more than 14 days or without serology before glucocorticoid initiation were excluded. The OPA from both eyes was obtained and averaged just before TABx of the predominantly symptomatic side. The variables chosen for the a priori prediction model were age, average OPA, and C-reactive protein (CRP). Erythrocyte sedimentation rate (ESR), platelets, jaw claudication, and eye findings were also recorded. In this study, subjects with a negative biopsy were considered not to have GCA, and contralateral biopsy was performed if the clinical suspicion for GCA remained high. An external validation set (XVAL) was obtained. Results Of 109 TABx, 19 were positive and 90 were negative. On univariate logistic regression, the average OPA had 0.60 odds for positive TABx (p = 0.03), with no statistically significant difference in age, sex, CRP, ESR, or jaw claudication. In suspected GCA, an OPA of 1 mm Hg had positive likelihood ratio 4.74 and negative likelihood ratio 0.87 for positive TABx. Multivariate regression of the prediction model using optimal mathematical transforms (inverse OPA, log CRP, age >65 years) had area under the receiver operating characteristic curve (AUROC) = 0.85 and AUROCXVAL = 0.81. Conclusions OPA is lower in subjects with biopsy-proven GCA and is a statistically significant predictor of GCA.

Carotid atherosclerosis and giant cell arteritis (GCA) are two distinct medical conditions with an overlapping clinical spectrum of vascular symptoms such as vision loss and ischemic stroke. This is because both diseases cause arterial ischemia with a predilection for carotid vasculature. In addition, high-vascular risk individuals who are diagnosed with GCA are usually elderly with age >55 years with high-vascular risk and thus can have underlying atherosclerosis. All these factors can pose a diagnostic dilemma for the physicians as GCA is a medical emergency which if left untreated can result in significant morbidity and mortality. Thus, it is important to avoid attributing occlusive arterial disease in elderly patients to atherosclerosis alone because some may have GCA. We present a case report in which presence of diffuse atherosclerosis was a major pitfall while making a timely diagnosis of GCA.

Objective Colour Doppler Sonography (CDS) in giant cell arteritis (GCA) allows the study of involvement of cranial arteries other than the temporal arteries, which are inconvenient to biopsy, such as the facial (FaA), and occipital (OcA) arteries. We aimed to estimate the frequency of the FaA, and OcA involvement in GCA; and to explore the clinical characteristics of these subgroups of patients. Methods From 1 January 2014 to 31 December 2016 we prospectively performed a CDS of the FaA, and OcA in addition to the temporal (TA), and the extracranial supra-aortic arteries in all newly diagnosed patients suspected of having GCA. All the arteries were evaluated in two planes for the highly specific halo sign. Results During the 36-month observation period we performed a CDS of the cranial and extra-cranial arteries in 93 GCA patients. We observed the halo sign on the FaA, and OcA in 38 (40.9%), and 29 (31.2%) cases, respectively. The FaA, or OcA were affected in 4/22 (18.2%) patients with a negative TA CDS. FaA involvement significantly correlated with jaw claudication and with severe visual manifestations, including permanent visual loss. Conclusions A fifth of patients with a negative CDS of the TAs had signs of vasculitis on the CDS of the FaA, or OcA. The addition of FaA and OcA CDS to the routine CDS of the TAs could identify 4.3% more patients and thus further improve the sensitivity of the CDS in the suspected GCA.

Rhinocerebral mucormycosis (RCM) can lead to internal carotid artery thrombosis. Here, we report the first case of RCM with temporal artery thrombosis following HLA-haploidentical stem cell transplantation in an adolescent presenting with low-grade fever, right mandibular pain, and right jaw claudication. This case suggests that RCM can cause temporal artery thrombosis and should be considered as a differential diagnosis in severely immunocompromised patients with maxillary sinusitis presenting with jaw claudication.

Giant cell arteritis (GCA) affects large extracranial supraaortic arteries. Ischemic stroke is a presenting feature in up to 10% and carries a mortality rate of 75%1. A 72-year-old woman was admitted with acute dizziness and jaw claudication. Clinical examination showed swollen temporal arteries, dysarthria, ataxia of the lower limbs, and left hemianopia. Erythrocyte sedimentation rate was elevated …

A 74-year-old man presented with acute onset right superonasal visual field loss and blurred vision. He had no other focal neurological symptoms, headache, jaw claudication or scalp tenderness. Medical history included coronary artery bypass grafting, hypertension and hypercholesterolaemia.

Background:Giant cell arteritis (GCA) is the most common medium- and large vessel vasculitis in the elderly population. The diagnostic algorithm has changed in recent years in many institutions from temporal artery biopsy (TAB) to imaging based diagnosis (1). However, classification criteria that relied on TAB remained unchanged since 1990 (2). Reflecting the recent change in practice the Diagnostic and Classification Criteria for Vasculitis Study (DCVAS) group presented a draft classification criteria set for GCA. Endorsement by major scientific societies is currently pending (3).Objectives:To test the accordance of the GCA cohort of a single center in southern Norway between 2006 and 2018 to the draft DCVAS classification criteria in GCA.Methods:All patients diagnosed with GCA between 2006 and 2018 in our clinic were identified by international classification of disease (ICD) code for GCA (m31.5/m31.6) in the local electronic database. The draft DCVAS classification criteria were applied to all patients at the time of diagnosis, irrespective of the algorithm used to diagnose GCA. In the draft DCVAS classification criteria a score of 6 would be necessary to classify a patient as having GCA.Results:A total of 77 patients (55 female) diagnosed with GCA in the defined timespan were identified. Mean age was 69.2 years (57 – 83 years). As all patients were diagnosed with vasculitis and were older than 40 years of age, all patients met the compulsory criteria. The table 1 below shows the absolute number of patients that fulfilled the single criteria.The number of patients fulfilling the draft DCVAS classification criteria was 75 (97.4%). In the female cohort 96.4% patients finally diagnosed with GCA would be classified as having GCA as would 100% in the male cohort.Mean score was 12.4 (range 3-25). In 33.3 % of patients imaging was critical for classification. Laboratory findings were critical for classification in 9.3 %, head ache and scalp tenderness in 5.3%, temporal artery biopsy, morning stiffness and jaw claudication in 2.7%. The differentiation in possible and definite vasculitis in TAB did not change the result in any patient. Neither did sight-loss contribute to classify any patient. Five patients (6.5%) were diagnosed on clinical features and laboratory findings alone. Thirteen patients diagnosed with GCA and fulfilling DCVAS criteria had negative biopsies, three patients had an inconclusive TAB. Mean arterial biopsy length was 9 mm.Table 1.Draft DCVAS classification criteriaAboslute numberAge>40 years and vasculitis diagnosis (compulsatory)77New temporal headache+248Scalp tenderness+221Sudden visual loss+22Morning stiffness shoulder/neck+234Jaw claudication+222ESR > 50 mm/h or CRP > 10 mg/l+372TAB showing possible vasculitis+38TAB showing definite vasculitis+516Halo sign+561Bilateral axillary involvement+325PET showing aortitis+34Conclusion:A high percentage (97.4 %) of the patients diagnosed with GCA in our cohort fulfilled the draft DCVAS classification criteria. The high mean score (12.4) showed that DCVAS criteria did not depend heavily on single criteria. As diagnosis in our cohort was mainly based on ultrasound, imaging was the most critical item. This might be desirable as clinical features and laboratory markers should in general be confirmed by either imaging or TAB to ascertain diagnosis. However, five patients were solely diagnosed on clinical features and laboratory markers. Sight-loss did not contribute to classification in our cohort as most patients were seen in a fast-track clinic (FTC), but might be valuable in cohorts without FTC.References:[1]Dejaco C et al, Ann Rheum Dis. 2018;77(5):636-643[2]Hunder GG et al, Arthritis Rheum. 1990 Aug;33(8):1122-8[3]Merkel PA: oral presentation, American College of Rheumatology Annual Meeting 2018: October 19-24; 2018Disclosure of Interests:None declared

A 60-year-old woman with decreased visual acuity in her right eye and right-sided jaw claudication was found to have ocular ischemic syndrome secondary to complete occlusion of the brachiocephalic artery. Although jaw claudication is often considered to be pathognomonic for giant cell arteritis, it has a broad differential diagnosis including both vascular and nonvascular conditions.

Summary Background We hypothesize that stenting of the internal carotid artery can immediately impede blood flow to the external carotid artery by either plaque shift or stent coverage of the ostium, and thereby cause ischemic symptoms like ipsilateral jaw claudication. Methods Thirty-three patients with high-grade asymptomatic stenosis of the internal carotid artery who underwent endovascular treatment were examined by ultrasound of the external carotid artery and performed an exercise test by chewing chewing gum synchronously to an electronic metronome for 3 min. Tests were performed before, the day after, and 1 week after the stenting procedure. Claudication time was defined as the timespan until occurrence of pain of the masseter muscle and/or chewing dyssynchrony to the metronome for more than 15 s. Ten patients with an isolated, atherosclerotic stenosis of the external carotid artery served as controls. Results A significantly reduced claudication time (in seconds) was recorded in patients who underwent carotid artery stenting compared to baseline values; median 89 (interquartile range, IQR, 57 to 124) vs. median 180 (IQR 153 to 180; p < 0.001). By categorization of the flow velocity at the external carotid artery into faster or slower as 200 cm/sec, the effect was even accentuated. Stenting values showed improvement 1 week after but did not return to baseline levels. No respective changes were found in controls. Conclusion Stenting of the internal carotid artery lead to ipsilateral flow deterioration at the external carotid artery resulting in temporary jaw claudication. This impairment attenuated over the time and was significantly reduced after 1 week.

Giant-cell arteritis (GCA), commonly known as temporal arteritis, is a chronic granulomatous vasculitis that affects predominantly the extracranial branches of the carotid artery. Although an algorithm for diagnosing GCA that includes both biopsy and imaging examinations has been recently proposed, it harbors the possibility of false negatives. Hence, jaw claudication caused by ischemia of the masticatory muscles is one of the important clinical predictors for implementation of temporal artery biopsy (TAB). We describe a case of GCA in which jaw claudication was the only clinical predictor for implementation of TAB. A 78-year-old man was referred to our department with facial pain associated with mastication. He had been admitted 3 weeks previously to another department to investigate an unidentified fever. A blood test revealed an elevated C-reactive protein level and a high erythrocyte sedimentation rate (70 mm/h). Although ultrasonography and computed tomography angiography of the temporal region showed no findings specific for GCA, based on our examinations we determined that his facial pain arose from jaw claudication rather than temporomandibular disorder. Histopathological examination by temporal artery biopsy showed intima thickening with disruption of elastic lamina and inflammatory cell infiltration, and we consequently diagnosed GCA. Clinical symptoms immediately resolved after prescription of prednisolone 40 mg/day for 2 days. In this case of GCA the imaging examinations of the superficial temporal artery were false-negative, and jaw claudication was the most important predictor of GCA.

Anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis and giant cell arteritis (GCA), also known as small-vessel vasculitis and large-vessel vasculitis, respectively, are sometimes difficult to distinguish by only the size and distribution of the vessels involved in daily clinical practice. We describe two cases of GCA with typical symptoms of GCA, i.e. a patient with jaw claudication complicated with microscopic polyangiitis (MPA), who had pleuritis, mononeuritis multiplex, interstitial pneumonia and scleritis. We treated the two cases as MPA and both patients achieved remission. When patients have atypical symptoms of GCA, not only GCA but also ANCA-associated vasculitis (AAV) should be investigated.

Background:Temporal artery biopsy (TAB) is the gold standard for diagnosing giant cell arteritis (GCA). However, previous studies have reported that the discordance rate of TAB is 3-45%,i.e., in unliteral TAB, GCA may be overlooked in one in five patients, approximately. Evidence as to whether bilateral TAB should be performed initially or one-sided TAB is sufficient for diagnosing GCA is lacking.Objectives:To investigate the predictors of patients with GCA in whom one-sided TAB is sufficient.Methods:The present study was a cross-sectional, single center study conducted from April 1, 2011 to July 31, 2019 at Tokyo Metropolitan Tama Medical Center. Of all consecutive GCA cases for which bilateral TAB was performed, bilaterally positive cases and unilaterally positive cases were extracted as bilateral positive group (BPG) and unilateral positive group (UPG), respectively. GCA was defined in accordance with the classification criteria of the 1990 American College of Rheumatology, and GCA was diagnosed if no other etiology was found within six months after beginning of high-dose glucocorticoid treatment. Demographic, clinical and laboratory data were obtained from the medical records, and the BPG and the UPG were compared statistically in each variable. Statistical significance was defined asp< 0.05.Results:During study, 264 biopsies were performed for 145 cases, who suspected GCA and underwent TAB. The pathological positivity rate was 26.1% (68 / 264 biopsies). Of these, 53 cases had final diagnosis of GCA, in which 43 cases were biopsy proven GCA. Thirty-seven biopsy proven GCA with bilateral TAB were enrolled; 64.9% women; mean (SD) age 75 (8.9) years; median [IQR] TAB length 17.5 [13.0,20.0] mm; headache 54.1%; jaw claudication 45.9%; scalp tenderness 16.2%; temporal artery (TA) tenderness 32.4%; TA engorgement 32.4%; TA pulse abnormality 5.4%; visual symptoms 2.7%; a fever of 38.5°C or higher 40.5%; shoulder girdle pain 48.6%; imaging of aortitis or arteritis 40.5%; median [IQR] white blood cell 9,100 [7200, 12050] /μl; median [IQR] platelet cell 37.5 [27.0, 46.3] ×104/μl; median [IQR] C-reactive protein (CRP) 10.1 [3.9, 16.5] mg/dL; erythrocyte sedimentation rate [IQR] 105 [66, 129] mm/h. Thirty-one in 37 cases were positive bilaterally while 6 in 37 cases were positive unilaterally; and the discordance rate was 16.2%. The median sample length after formalin fixation was 19.0 mm for the BPG and 14.5 mm for the UPG (p= 0.171). The parameters above were compared between UPG and BPG. Of these, only the serum CRP value (mg/dL) differed statistically between groups, and the median value of the two groups was 10.6 and 6.5, respectively (median test:p= 0.031). To predict BPG, in whom unilateral TAB is sufficient for diagnosing GCA, the cut-off value of serum CRP with a specificity of 100% and a sensitivity of 61.3% was set at 9.3 mg/dL (ROC analysis: AUC 0.726).Conclusion:When the serum CRP level is 10 mg/dL or higher in GCA suspected patients, an unilateral TAB alone was sufficient for an accurate diagnosis.References:[1]Hellmich, B, et al.Ann Rheum Dis2020;79(1):19-30.[2]Breuer, GS, et al.J Rheumatol. 2009;36(4):794-796.[3]Czyz CN, et al.Vascular2019;27(4):347-351.[4]Durling B, et al.Can J Ophthalmol2014;49(2):157-161.Figure.Comparison of median CRP levels between unilaterally positive group and bilaterally positive group.Disclosure of Interests:None declared

Objective We investigated the ability of known clinical signs and symptoms, as well as common laboratory tests, to correctly predict a positive temporal artery biopsy. Design A prospective cohort study. Participants Consecutive patients in a tertiary referral centre undergoing temporal artery biopsy. Methods Clinical information was collected using a predesigned questionnaire. Pathology results and laboratory information were collected from digital patient records. Main Outcome measure The predictive value of clinical signs, symptoms, and laboratory values of a positive temporal artery biopsy. Results Over a 3-year period, 259 patients were enrolled and 251 patients were analyzed. Sixty-one patients had a positive biopsy. Clinical features most predictive of a positive biopsy were jaw claudication (positive likelihood ratio [LR+] 2.31) and abnormal temporal artery pulse (LR+ 2.62). Receiver operating characteristic curves generated for erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and platelets values showed an area under curve (AUC) value of 0.71, 0.75, and 0.76, respectively. The initiation of steroids decreased the diagnostic utility of the ESR, CRP, and platelets values (AUC = 0.58, 0.61, and 0.63, respectively). Conclusions A variety of clinical signs and symptoms were observed in patients referred for a temporal artery biopsy. Clinical signs and symptoms were less accurate in predicting a positive biopsy than laboratory tests. No combination of clinical signs and symptoms tested was able to predict giant cell arteritis with the certainty necessary to justify or withhold long-term steroid therapy.

Objectives Vision complications and a stroke represent severe cranial ischaemic complications (sCIC) associated with increased morbidity and mortality in GCA. We aimed to determine the risk factors for sCIC in GCA. Methods We analysed the medical records of prospectively enrolled GCA patients diagnosed between September 2011 and August 2019, and compared the clinical and laboratory characteristics of patients with and without sCIC defined as either severe vision complications (diplopia, transient vision loss, permanent partial vision field/acuity defect and permanent visual loss) or stroke. Results During the 96-month observation period, we identified 295 new GCA patients [65.4% female, median (interquartile range) age 74.7 (67.3–80.0) years]. Sixty-one (20.7%) patients developed sCIC (52 isolated severe vision complications, 5 isolated ischaemic strokes and 4 patients with both complications). In a multivariable logistic regression model jaw claudication [odds ratio (OR) 3.43 (95% CI: 1.84, 6.42), P Conclusion Increasing age, jaw claudication and smoking predicted sCIC, while higher CRP decreased the risk of sCIC in our GCA cohort.