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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.

Objective Vascular ultrasound (VUS) is a first-line test for giant cell arteritis (GCA) in Europe but has been of limited use in the United States. We report clinical experience with a multidisciplinary model of VUS for the evaluation of GCA at a large US medical center. Methods Patients who underwent VUS for evaluation of GCA between 2013 and 2017 were reviewed. Trained vascular technologists followed a standardized protocol to visualize bilateral temporal, carotid, subclavian, and axillary arteries. Vascular medicine physicians interpreted VUS as no arteritis, hyperechoic wall thickening, or acute arteritis. Characteristics of patients with versus without acute arteritis (no arteritis or hyperechoic wall thickening) were compared. Among patients with suspected new-onset GCA, the treating physician's pretest and posttest suspicion for GCA were compared. Results Of 530 patients, 10.6% had prior-onset GCA, 31.7% had polymyalgia rheumatica, and 57.6% were taking glucocorticoids. Most patients had no arteritis on VUS (84.3%); 10.6% had acute arteritis, and 5.1% had hyperechoic wall thickening. Typical GCA symptoms, such as jaw claudication and scalp tenderness, were significantly more frequent in patients with acute arteritis. For all 42 patients with suspected new-onset GCA and acute arteritis, posttest suspicion was unchanged or increased. Of 415 patients with suspected new-onset GCA and VUS without acute arteritis, suspicion decreased (76.4%) or was unchanged (20.2%). Conclusion We describe a multidisciplinary model for incorporating VUS into GCA care. When pretest suspicion was low and VUS did not reveal acute arteritis, posttest suspicion typically decreased, whereas when pretest suspicion was high and VUS revealed acute arteritis, posttest suspicion was reinforced.

Objectives GCA is a large vessel vasculitis (LVV) presenting with headache, jaw claudication, musculoskeletal and visual involvement. Current treatment is glucocorticoids and anti-IL-6 tocilizumab in refractory disease. The objective of this study was to explore the impact of GCA and its treatment on people’s health-related quality of life (HRQoL), to inform the development of a disease-specific patient-reported outcome measure (PROM) for use in clinical trials and practice. Methods Participants from the UK and Australia, with biopsy- or imaging-confirmed GCA, were interviewed to identify salient aspects of HRQoL in relation to GCA and its treatment. Purposive sampling included a range of demographic and disease features (cranial, LVV-GCA and visual involvement). Inductive analysis identified individual themes of importance, then domains. Candidate questionnaire items were developed from the individual themes, refined by piloting, cognitive interviews and a linguistic translatability assessment. Results Thirty-six interviews were conducted to saturation with participants with GCA from the UK (25) and Australia (11). Mean age was 74 years, 23 (63.9%) were female, 13 (36.1%) had visual loss and 5 (13.9%) had LVV-GCA. Thirty-nine individual themes within five domains were identified: physical symptoms; activity of daily living and function; participation; psychological impact; and impact on sense of self and perception of health. Sixty-nine candidate items were developed from individual themes; piloting and refinement resulted in a 40-item draft questionnaire. Conclusion This international qualitative study underpins the development of candidate items for a disease-specific PROM for GCA. The draft questionnaire is now ready for psychometric testing.

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 65-year-old man presented with a slight headache and transient visual disturbance. Magnetic resonance imaging (MRI) revealed occlusion of the left internal carotid artery (ICA) and acute brain infarctions in both hemispheres, and a blood examination indicated inflammation. Gadolinium enhancement was observed in the walls of the temporal arteries and ICAs. After we diagnosed giant cell arteritis (GCA) by a temporal artery biopsy, aspirin and corticosteroids were administered. The typical symptoms of GCA, such as jaw claudication and temporal artery tenderness, were absent during the entire clinical course, and the findings of contrast-enhanced MRI contributed to the diagnosis.

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.

Objective.To identify shared and distinct features of giant cell arteritis (GCA) and coronavirus disease 2019(COVID-19) to reduce diagnostic errors that could cause delays in correct treatment.Methods.Two systematic literature reviews determined the frequency of clinical features of GCA and COVID-19 in published reports. Frequencies in each disease were summarized using medians and ranges.Results.Headache was common in GCA but was also observed in COVID-19 (GCA 66%, COVID-19 10%). Jaw claudication or visual loss (43% and 26% in GCA, respectively) generally were not reported in COVID-19. Both diseases featured fatigue (GCA 38%, COVID-19 43%) and elevated inflammatory markers (C-reactive protein [CRP] elevated in 100% of GCA, 66% of COVID-19), but platelet count was elevated in 47% of GCA but only 4% of COVID-19 cases. Cough and fever were commonly reported in COVID-19 and less frequently in GCA (cough, 63% for COVID-19 vs 12% for GCA; fever, 83% for COVID-19 vs 27% for GCA). Gastrointestinal upset was occasionally reported in COVID-19 (8%), rarely in GCA (4%). Lymphopenia was more common in COVID-19 than GCA (53% in COVID-19, 2% in GCA). Alteration of smell and taste have been described in GCA but their frequency is unclear.Conclusion.Overlapping features of GCA and COVID-19 include headache, fever, elevated CRP and cough. Jaw claudication, visual loss, platelet count and lymphocyte count may be more discriminatory. Physicians should be aware of the possibility of diagnostic confusion. We have designed a simple checklist to aid evidence-based evaluation of patients with suspected GCA.

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.

Giant cell arteritis is the most common primary systemic vasculitis in adults aged ≥50 years and peaks in the eighth decade of life. Common symptoms include headache, scalp tenderness and jaw claudication. Elevated acute phase reactants (erythrocyte sedimentation rate and C-reactive protein) are present in >90% of patients. Visual loss is a well-recognised complication, but approximately 2–4% of giant cell arteritis patients experience stroke, most frequently in the vertebrobasilar territory. We describe a 72-year-old male who developed bilateral vertebral artery occlusion and middle cerebellar peduncle infarction secondary to giant cell arteritis in spite of high-dose steroids.

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 well-known cause of cranial vasculitis often presenting with headache and jaw claudication. Here we report the case of a woman suffering GCA who presented with critical lower limb ischemia. Despite best medical therapy, she developed progressive calf claudication and ulceration of the right foot. The findings on workup were highly suggestive of GCA involving the superficial femoral artery. The limb was successfully revascularized with angioplasty and placement of a drug-eluting stent. GCA is an important cause of lower limb ischemia and should be considered in patients without evidence of atherosclerosis. Endovascular intervention is a feasible treatment of critical limb ischemia due to GCA and has been shown to be safe in this case. Keywords: Endovascular procedures, Balloon angioplasty, Lower limb ischemia, Critical limb ischemia, Giant cell arteritis

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.