Scalp Tenderness Research Articles

Teaching Video NeuroImage: Bilateral Hemifacial Spasm in Giant Cell Arteritis.

An 80-year-old man developed bitemporal headache and scalp tenderness. Both temporal arteries were prominent (Figure, A), with halo sign on ultrasonography.1 Temporal arteritis was diagnosed and oral prednisone (50 mg/d) initiated. Three days later, he developed spasms of the orbicularis oculi and frontalis muscles with eyebrow elevation and eye twitching (other Babinski sign),2 consistent with bilateral hemifacial spasm (Video 1). Brain MRI and time-of-flight angiography revealed exclusively supratentorial acute infarcts (Figure, B and C) without intracranial neurovascular conflicts. Intravenous methylprednisolone (1 g/d for 5 days) was started with resolution of spasms within 24 hours and clinical stabilization. Temporal artery inflammation may cause facial nerve irritation and hemifacial spasm (Figure, D).

Giant cell arteritis-related stroke in a large inception cohort: A comparative study

ObjectiveStroke caused by giant cell arteritis (GCA) is a rare but devastating condition and early recognition is of critical importance. The features of GCA-related stroke were compared with those of GCA without stroke and atherosclerosis-related or embolic stroke with the aim of more readily diagnosing GCA. MethodsThe study group consisted of 19 patients who experienced GCA-related strokes within an inception cohort (1982-2021) of GCA from the internal medicine department, and the control groups each consisted of 541 GCA patients without a stroke and 40 consecutive patients > 50 years of age with usual first ever stroke from the neurology department of a French university hospital. Clinical, laboratory, and imaging findings associated with GCA related-stroke were determined using logistic regression analyses. Early survival curves were estimated using the Kaplan-Meier method and compared using the log rank test. ResultsAmongst 560 patients included in the inception cohort, 19 (3.4%) developed GCA-related stroke. GCA-related stroke patients had more comorbid conditions (p = 0.03) and aortitis on imaging (p = 0.02), but less headache (p < 0.01) and scalp tenderness (p = 0.01). Multivariate logistic regression analysis showed that absence of involvement of the anterior circulation (OR = 0.1 – CI: 0.01-0.5), external carotid ultrasound (ECU) abnormalities (OR = 8.1 – CI: 1.3-73.9), and C-reactive protein (CRP) levels > 3 mg/dL (OR = 15.4 – CI: 1.9-197.1) were independently associated with GCA-related stroke. Early survival of GCA-related stroke patients was significantly decreased compared with control stroke patients (p = 0.02) and GCA patients without stroke (p < 0.001). ConclusionsThe location of stroke and assessment of ECU results and CRP level could help improve the prognosis of GCA-related stroke by bringing this condition to the clinician's attention more quickly, thus shortening diagnostic delay.

P298 Ocular manifestations in a giant cell arteritis cohort

Abstract Background/Aims The ophthalmic features of giant cell arteritis involve a spectrum of severity, from transient symptoms to bilateral visual loss due to anterior ischaemic optic neuropathy or, less commonly, central retinal or cilioretinal artery occlusion. Data of reported ocular involvement are varied, with a range of 10-70%. The variation may be related to the specialty of the unit reporting the data. We run an interdisciplinary service in a tertiary academic unit and report a complete picture of the frequency and nature of ocular involvement in GCA. Methods The records of 350 consecutive patients, diagnosed objectively on the basis of biopsy or imaging, were reviewed. In our centre, the hospital pathway mandates all individuals with suspected GCA to have a formal ophthalmology assessment including visual acuity, pupil exam and full dilated fundus assessment. We recorded the common signs and symptoms using a structured form which included ophthalmologic symptoms and examination findings, along with symptoms commonly associated with GCA: scalp tenderness, headache, jaw claudication, shoulder girdle stiffness, anorexia, night sweats, weight loss, and fever. Results From January 2012 to September 2021, 350 individuals were diagnosed with GCA by biopsy, ultrasonography or positron emission tomography. The mean age was 74 ± 7.7 years. 237 (68%) of patients were females. 99 (28%) presenting with GCA had visual symptoms and or signs. 34 had mono-ocular and two had binocular loss of vision. A summary of the key visual symptoms and signs are shown in Table 1. Only five patients with visual symptoms did not have any symptoms commonly associated with GCA. Conclusion We report the frequency of visual involvement in one of the largest cohorts of individuals with GCA. 28% have ocular symptoms. Partial or total monocular field loss occurred in 12% of cases. 1.4% of patients may be presenting with visual symptoms as the only sign of GCA. Rarely, permanent visual loss may occur without any other manifestation of GCA. Disclosure S. Mansfield Smith: None. M. Al-Hashimi: None. G. Ducker: None. C. Jones: None. C. Mukhtyar: None.

P044 Giant cell arteritis in an immunocompromised patient with rheumatoid arthritis

Abstract Background/Aims Giant cell arteritis (GCA) is a common medium-large vessel vasculitis with an approximate incidence of 10 cases per 100,000 people over 50 years old. First line management of GCA is with high dose glucocorticoids followed by steroid sparing agents, namely methotrexate with tocilizumab for resistant cases. Here we describe a patient established on methotrexate for rheumatoid arthritis, receiving rituximab with intravenous methylprednisolone (IVMP) who went on to develop GCA. Methods A 65-year-old Caucasian female with seropositive rheumatoid arthritis (RA) established on 20mg weekly subcutaneous methotrexate and low dose 5mg prednisolone was escalated to her tenth cycle of rituximab with 100mg IVMP for an arthritic flare. The day after her second infusion, two weeks apart, the patient presented with headaches. These were severe temporal pains with scalp tenderness for the past four weeks. She denied visual changes, jaw claudication or polymyalgia rheumatica features. Examination identified bilateral thickened and tender temporal arteries. Ultrasound confirmed bilateral frontal temporal artery halo signs, with intimal thickening of 0.9mm and 0.4mm of the left and right respectively. Blood tests showed a rise in C-reactive protein to 46mg/L from 13mg/L. In view of these findings, procedural risks and recent immunosuppression biopsy was not sought. The patient was commenced on 40mg prednisolone before weaning by 5 mg 2-3 weekly. Her headache improved, without relapse and her CRP normalised within 2 weeks. Given tocilizumab had previously been ineffective in the patient’s RA management, she declined transition to this biologic and her methotrexate was increased to 25mg. Results Development of GCA in a patient with pre-established immunotherapy is rare. This report represents only the second case noted in the literature of the vasculitis occurring in a patient whilst on rituximab. Excluding tocilizumab (anti-IL6), a meta-analysis of other biological agents including infliximab, adalimumab, etanercept (anti-TNF) and abatacept (anti-CTLA4) have not been proven to have clinical significance over placebo in GCA. However, evidence of rituximab and its role in GCA is limited with no clinical trials. Contrary to this case of GCA, rituximab (anti-CD20) therapy has a well-recognised role in ANCA associated vasculitis and there are growing calls for trials in large vessel vasculitides. Recent case series of rituximab in Takayasu’s arteritis have presented contrasting outcomes of persistent disease activity versus clinical and laboratory remission. Robust clinical trials are therefore required to evaluate the long-term impact of rituximab on large vessel vasculitis. Conclusion Although rare, clinicians should be vigilant for the development of GCA despite established immunosuppression. Disclosure N. Chauhan: None. A. Kinder: None.

Efficacy of bilateral temporal artery biopsies and sectioning of the entire block of tissue for the diagnosis of temporal arteritis

Giant cell arteritis (GCA) is a systemic vasculitis of medium and large sized blood vessels. The incidence is greater in women as compared to men (3:1) and most often occurs in the elderly. The most common symptoms are unilateral headaches, visual disturbances and scalp tenderness. If untreated, GCA may result in irreversible blindness. Prompt treatment is necessary to prevent progression of the disease, but accurate diagnosis is vital to prevent unwarranted side effects of the therapy. Temporal artery biopsy (TAB) remains the gold standard for diagnosis. TAB is an invasive procedure that can require up to 40 minutes to perform but is important for pathological confirmation. Variation amongst centers and practitioners exists in the type of biopsies performed. In 2013, a survey of over 1000 specialists showed that 37% recommended unilateral biopsy alone, 29% recommended initial unilateral biopsy with biopsy of the contralateral side if the first side is negative, 18% recommended bilateral biopsy in all cases, and 16% stated that their preference depended upon the degree of suspicion. Studies have shown that bilateral TAB can enhance diagnostic accuracy by 3 to 12.7%. Furthermore, temporal arteritis can involve the artery in a discontinuous fashion and there is no standardization of the number of sections or levels that should be examined in a segment of temporal artery. This study aims to shed light on the benefits of a bilateral temporal artery biopsy as well as to determine the optimum level for block sectioning for the diagnosis of temporal arteritis.

A New Era for the Early and Non-Invasive Diagnosis of Giant Cell Arteritis: The Use of Fast-Track Ultrasound in Clinical Practice

Background: The assessment of giant cell arteritis (GCA) in fast-track assessment clinics (FTA) including the use of ultrasound (US) is becoming the preferred practice in specialized centers. Methods: Patients with suspected GCA referred to the FTA of the Rheumatology Department, University of Pavia, Italy, between 2016 and 2021 were included to analyze the clinical and US findings. Results: A total of 553 US examinations were performed on 347 patients. A total of 246 were female (71%), and the mean age was 73 ± 12. Of these, 287 US on newly referred patients led to a confirmed diagnosis of GCA in 111 (39%). The sensitivity of US was 81.98% (95% CI 73.55–88.63%), and the specificity 99.43% (95% CI 96.88–99.99%). Only 4 patients required temporal artery biopsy. The most specific symptoms to inform the pre-test probability of GCA and differentiate from patients with other conditions were: jaw or tongue claudication, scalp tenderness, and bilateral visual loss. Headache was not reported in 33% of patients. Systemic symptoms were significantly more frequent in GCA (42.3%), together with combinations of cranial, systemic, and/or polymyalgia rheumatica symptoms. Out of 88 patients, there were 52% with a confirmed relapse. Of these, 67% had a positive US. Conclusion: The use of FTA in clinical practice ensures an early diagnosis, avoiding invasive procedures for the patient. Our data support the increasingly recognized adjunctive role of US in the monitoring of GCA.

Do We Have a Culprit? An Association of Giant Cell Arteritis with Pulmonary Embolism.

Giant cell arteritis is the most common type of systemic vasculitis. An increased risk of venous thromboembolism has been described in these patients. We report the case of a 79-year-old woman with a history of polymyalgia rheumatica, who presented with left thoracic pain radiating to the neck and scapula plus temporal headache. She had no changes on physical examination, but work-up tests showed increased D-dimer levels and computed tomography pulmonary angiography revealed signs of a chronic/subacute embolism in the right inferior lobe. Anticoagulation with edoxaban was started after 5-day bridging with enoxaparin. Three weeks after the initial diagnosis the headache still persisted and she developed scalp tenderness. Giant cell arteritis was diagnosed and treated with prednisolone, with complete resolution of symptoms. Extensive diagnostic work-up was performed to identify an alternative cause of pulmonary thromboembolism; however, the investigations were negative. This case supports the hypothesis that this type of vasculitis could be related to the occurrence of pulmonary embolism.LEARNING POINTSAn increased risk of thromboembolism has been associated with giant cell arteritis.Early diagnosis is crucial; however, the role of antiplatelets or anticoagulants is not yet established.

Do We Have a Culprit? An Association of Giant Cell Arteritis with Pulmonary Embolism

Giant cell arteritis is the most common type of systemic vasculitis. An increased risk of venous thromboembolism has been described in these patients. We report the case of a 79-year-old woman with a history of polymyalgia rheumatica, who presented with left thoracic pain radiating to the neck and scapula plus temporal headache. She had no changes on physical examination, but work-up tests showed increased D-dimer levels and computed tomography pulmonary angiography revealed signs of a chronic/subacute embolism in the right inferior lobe. Anticoagulation with edoxaban was started after 5-day bridging with enoxaparin. Three weeks after the initial diagnosis the headache still persisted and she developed scalp tenderness. Giant cell arteritis was diagnosed and treated with prednisolone, with complete resolution of symptoms. Extensive diagnostic work-up was performed to identify an alternative cause of pulmonary thromboembolism; however, the investigations were negative. This case supports the hypothesis that this type of vasculitis could be related to the occurrence of pulmonary embolism.

Tocilizumab for the treatment of giant cell arteritis in Scotland: a report on behalf of the Scottish Society for Rheumatology standards subgroup.

ObjectivesThe aim was to describe a modern National Health Service (NHS) Scotland cohort of patients with GCA over 12 months of care to include clinical presentation, practices relating to assessment and treatment, and specifically, the use of tocilizumab.MethodsA multicentre audit of patients newly diagnosed with GCA between November 2019 and October 2021 was established on behalf of the Scottish Society for Rheumatology. Clinical data were collected retrospectively by rheumatology teams at participating NHS centres using electronic patient records. An extended cohort of patients from NHS Lothian was examined to investigate outcomes of tocilizumab use for >1 year.ResultsSixty-three patients from three NHS Scotland health boards were included, with analysis of data from 216 clinic episodes. Mean follow-up was 371 days. Mean age was 71 years; 62% were female. The most common presenting features were headache (93.6%), scalp tenderness (82.5%) and ocular symptoms (24%). At baseline, 63% of patients had at least one existing risk factor for adverse outcomes from high-dose CS use, namely hypertension (57.1%), diabetes (24%) and osteoporosis (11%). Thirty per cent of all patients (19 of 63) received tocilizumab, with only 11% (7 of 63) receiving tocilizumab owing to glucocorticoid risk factors at baseline. One-quarter of all patients (16 of 63) experienced relapse of GCA during follow-up, of whom six were subsequently treated with tocilizumab.ConclusionThis multicentre audit demonstrates that despite its availability for patients with risk factors for CS adversity and those who suffer relapse of GCA, tocilizumab is used in less than one-quarter of patients who might benefit. The reasons for this require further exploration.

Two cases of giant cell arteritis following ChAdOx1 nCoV-19 vaccination – complication or coincidence?

This 66 years old woman had the ChAdOx1 nCoV-19 vaccine on 25 February 2021 and about 6 hours later, she started to get pain in the right side of her head. She also had flu-like symptoms which lasted a couple of days, but these settled down whilst the headaches persisted. She also developed scalp tenderness and obvious swelling of possibly frontal branch of right temporal artery (see figure 1). She did not develop any jaw claudication, visual or systemic symptoms. She was seen in the out of hours GP clinic on 29 of February 2021 and was started on Prednisolone 40 mg daily with resolution of symptoms in a few days. Her blood tests in February 2021 showed rise in CRP to 16.8 mg/L (NR 0-5) compared to December 2020 value of 3.3. Ultrasound (US) showed normal appearances in the temporal or axillary arteries, although this was delayed 2 weeks post corticosteroid (CS) therapy. Her visible temporofrontal abnormalities had disappeared within a few days of treatment. CT scan head with venogram was normal and treatment for GCA was continued.

O31 Giant cell arteritis - a rare ocular presentation

O31 Giant cell arteritis - a rare ocular presentation

A Practical Approach to the Diagnosis and Management of Classic Lichen Planopilaris.

Lichen planopilaris is a primary lymphocytic cicatricial alopecia that commonly presents with hair loss at the vertex or parietal scalp. Patients may also have associated scalp itching, burning or tenderness. Due to scarring, hair loss is typically permanent. The main goals of treatment are reducing symptoms and preventing disease progression and further hair loss. Currently, the literature has limited evidence on treatments for this difficult condition, and most available evidence is from case reports and case series. Furthermore, the evidence shows a varied response to therapy, with frequent reports of poor response. This article reviews the diagnosis of this rare disease, summarize the currently available treatments, and provide insights and practices from alopecia experts.

Safety and feasibility of rhenium-186 nanoliposome (186RNL) in recurrent glioma: The ReSPECT phase 1 trial.

2061 Background: While external beam radiation therapy (EBRT) remains a central component of the management of primary brain tumors, it is limited by tolerance of the surrounding normal brain tissue. Rhenium-186 NanoLiposome (186RNL) permits the delivery of beta-emitting radiation of high specific activity with excellent retention in the tumor. We report the results of the phase 1 study in recurrent glioma. Methods: A Phase 1 dose-escalation study of 186RNL in recurrent glioma utilizing a standard 3+3 design was undertaken to determine the maximum tolerated dose of 186RNL. 186RNL is administered by convection enhanced delivery (CED). Infusion is followed under whole body planar imaging and SPECT/CT. Repeat SPECT/CT imaging is performed immediately following, and at 1, 3, 5, and 8 days after 186RNL infusion to obtain dosimetry and distribution. Subjects were followed until disease progression by RANO criteria. Results: Eighteen subjects were treated across 6 cohorts. The mean tumor volume was 9.4 mL (range 1.1 – 23.4). The infused dose ranged from 1.0 mCi to 22.3 mCi and the volume of infusate ranged from 0.66 mL to 8.80 mL. From 1 – 4 CED catheters were used. The maximum catheter flow rate was 15 µl/min. The mean absorbed dose to the tumor volume was 239 Gy (CI 141 – 337; range 9 - 593), to normal brain was 0.72 Gy (CI 0.34 – 1.09; range 0.005 – 2.73), and to total body was 0.07 Gy (CI 0.04 – 0.10; range 0.001 – 0.23). The mean absorbed dose to the tumor volume when the percent tumor volume in the treatment volume was 75% or greater (n = 10) was 392 Gy (CI 306 – 478; range 143 – 593). Scalp discomfort and tenderness related to the surgical procedure did occur in 3 subjects. The therapy has been well tolerated, no dose-limiting toxicity has been observed, and no treatment-related serious adverse events have occurred despite markedly higher absorbed doses typically delivered by EBRT in patients with prior treatment. Responses have been observed supporting the clinical activity. Final results from the dose escalation will be presented. Conclusions: 186RNL administered by CED to patients with recurrent glioma results in a much higher absorbed dose of radiation to the tumor compared to EBRT without significant toxicity. The recommended Phase 2 dose is 22.3 mCi in 8.8 mL of infusate. Clinical trial information: NCT01906385. [Table: see text]

POS0808 CHARACTERISTICS OF GIANT CELL ARTERITIS FLARES AFTER SUCCESSFUL TREATMENT WITH TOCILIZUMAB: RESULTS FROM THE LONG-TERM EXTENSION OF A RANDOMIZED CONTROLLED PHASE 3 TRIAL

Background:GiACTA investigated tocilizumab (TCZ) for the treatment of giant cell arteritis (GCA).Objectives:To investigate disease flare characteristics after successful treatment with TCZ in GiACTA.Methods:We report a post hoc analysis from part 2 of GiACTA. Part 1 was a 52-week, double-blind, randomized controlled period and part 2 was a 2-year open-label follow-up. In part 1, patients received TCZ 162 mg subcutaneously every week or every other week with a 26-week prednisone taper or placebo plus a 26- or 52-week prednisone taper. Patients who were in remission at week 52 were to enter part 2 on no TCZ treatment. Part 2 treatment was at the investigator’s discretion. We report characteristics of first disease flare in patients assigned to TCZ in part 1 who were in sustained remission at week 52 and experienced flare in part 2. Flare was defined as reappearance of cranial symptoms (headaches, jaw claudication, visual manifestations, scalp tenderness) or polymyalgia rheumatica (PMR) symptoms or elevation of erythrocyte sedimentation rate (ESR) ≥30 mm/h attributable to GCA that required treatment.Results:Of 149 patients assigned to TCZ in part 1, 81 (54%) were in sustained remission on entering part 2. Of these 81 patients, 37 (46%) experienced at least one flare in part 2, including 17 with new-onset GCA and 20 with relapsing GCA at baseline. Median time to flare was 26.6 weeks. In patients with new-onset GCA, flares included cranial (53%) more often than PMR symptoms (18%). Cranial and PMR symptoms were balanced (both 60%) at the time of flare in patients with relapsing GCA. Visual manifestations occurred in two patients (5%) (Table 1). ESR and CRP were elevated in 65% and 36% of patients, respectively, at the time of flare. Three (8%) flares occurred with elevated ESR without clinical symptoms.Table 1.Clinical manifestations during flare in part 2Part 1 TreatmentaTCZ QW+PredTCZ Q2W+PredAll TCZNew-onset diseasePatients, n281442Patients with ≥1 flare, n (%)b9 (32.1)8 (57.1)17 (40.5)Patients with ESR ≥30 mm/h during flare, n (%)b6 (66.7)6 (75.0)12 (70.6)Patients with CRP ≥10 mg/L during flare, n (%)b4 (44.4)2 (25.0)6 (35.3)Patients with GCA signs or symptoms during flare, n (%)c7 (77.8)7 (87.5)14 (82.4)PMR symptoms1 (11.1)2 (25.0)3 (17.6)Cranial symptomsd4 (44.4)5 (62.5)9 (52.9) Amaurosis fugax000 Blurred vision1 (11.1)01 (5.9) Diplopia000 Blindness000 Ischemic optic neuropathy000Fever1 (11.1)01 (5.9)Othere3 (33.3)2 (25.0)5 (29.4)Relapsing diseasePatients, n281139Patients with ≥1 flare, n (%)b14 (50.0)6 (54.5)20 (51.3)Patients with ESR ≥30 mm/h during flare, n (%)b8 (57.1)4 (66.7)12 (60.0)Patients with CRP ≥10 mg/L during flare, n (%)b5 (35.7)3 (50.0)8 (40.0)Patients with GCA signs or symptoms during flare, n (%)c14 (100)6 (100)20 (100)PMR symptoms8 (57.1)4 (66.7)12 (60.0)Cranial symptomsd8 (57.1)4 (66.7)12 (60.0)Amaurosis fugax1 (7.1)01 (5.0)Blurred vision000Diplopia000Blindness000Ischemic optic neuropathy1 (7.1)01 (5.0)Fever000Othere6 (4.3)1 (16.7)7 (35.0)aPatients from part 1 TCZ+Pred groups who were in sustained remission at week 52 entered part 2 on no treatment.bPercentage based on N in disease-onset group.cPercentage based on number of flare patients in disease-onset group. Individual signs or symptoms are shown as number of patients with each symptom; patients could have ≥1 sign or symptom at the time of flare.dNew-onset localized headache, scalp tenderness, temporal artery tenderness or decreased pulsation, ischemia-related vision loss, or jaw pain claudication.eIncludes fatigue, malaise, subjective weakness, and night sweats.Conclusion:Overall, 46% of GCA patients successfully treated with TCZ for 1 year experienced disease flare within the next 2 years. Flares in patients with new-onset disease occurred more often with cranial than PMR symptoms. Visual manifestations were rare, and no blindness occurred. ESR and CRP were normal in a sizable percentage of patients experiencing flare.Disclosure of Interests:Sebastian Unizony Consultant of: Sanofi and Kiniksa Pharmaceuticals, Grant/research support from: Genentech, Inc., Shalini Mohan Shareholder of: Genentech, Inc., Employee of: Genentech, Inc., Jian Han Shareholder of: Genentech, Inc., Employee of: Genentech, Inc., John H. Stone Consultant of: Roche/Genentech and Sanofi

Abducens Nerve Palsy as the First Manifestation of Type Two Diabetes Mellitus

Abstract Introduction: Diabetes mellitus has varied presentations at different times from onset. One of the uncommon presentations is cranial nerve palsy secondary to microvascular ischemia. Approximately 20% of isolated abducens nerve palsies are secondary to vascular microangiopathy. Clinical Case: A 53-year-old female with no significant past medical history presented to the emergency department with concerns of double vision. The patient first noticed her symptoms five days prior to presentation. The patient endorsed blurry vision and stated that she has double vision in certain gazes that resolves with shutting either eye. Furthermore, she has also developed headaches during that period, described as a pressure-like sensation in her forehead and behind her eyes. She otherwise denied any tearing, redness, or pain. On review of systems, she denied numbness, tingling, changes in hearing, changes in speech, or extremity weakness. Due to the persistence of symptoms the patient presented to the emergency department. On exam, the patient’s vitals were normal. Pupils were equal and briskly reactive to light with no relative afferent pupillary defect. External examination was unremarkable without scalp tenderness, proptosis, or ptosis. Color vision was intact. Ocular motility testing revealed limited abduction of the left eye causing double vision on lateral gaze. Confrontation visual fields were full in each eye. Bilateral lower extremity exam revealed decreased sensation in the sole of the foot. The rest of the physical exam was unremarkable. Laboratory work revealed blood glucose level of 305 mg/dl (Reference range 70–99 mg/dl). HBA1C was 12.3% (Reference range 3.8–5.6%). CT head and CTA of the neck was performed and were unremarkable. Given the normal imaging and findings on examinations, her ocular motor findings were attributed to diabetes mellitus. The patient was started on insulin, aspirin, and received diabetic education for lifestyle modification, and was scheduled for outpatient follow up. The patient’s acute isolated left sixth cranial nerve palsy was most likely owing to microvascular ischemia from previously undiagnosed diabetes mellitus. A study of 59 patients with an isolated sixth cranial nerve palsy showed a 6-fold increase in the odds of having diabetes compared with controls.(1) Conclusion: Given the infrequent presentation of Diabetes with Abducens nerve palsy, diagnosis is usually delayed with the expense of ordering costly investigations that put a financial and psychological burden on patients. Thus, we urge clinicians’ awareness when encountering cases of isolated cranial nerve palsies.

P040 An unusual case of giant cell arteritis

Abstract Background/Aims A 49-year-old female of Nepalese heritage was referred with right-sided headache, scalp tenderness, and a painful swelling overlying the right temple. She denied any visual or claudicant symptoms but felt systemically unwell with a fever. There were no symptoms suggestive of an inflammatory arthritis, underlying connective tissue disease or vasculitis. She was normally fit and well with no past medical history. She did not take any regular medications and denied using over the counter or illicit drugs or recent travel. On review she had a low grade fever. There was a large tender, erythematous swelling overlying the right temple. Bilaterally the temporal arteries were palpable and pulsatile. Peripheral pulses were normal with no bruits. There was no evidence of shingles (HSV) or local infection. Full systemic examination revealed no other abnormalities. Laboratory tests showed: PV 2.56, CRP 101, total white cell count 14.38 (eosinophils 0.4), albumin 33, Hb 115. Urine dip was normal. Renal function, liver function and immunoglobulins were normal. ANCA was negative. Hypoechogenicity surrounding the right frontal branch of the right temporal artery was seen on ultrasound. There were no discrete masses suggestive of cysts, abscess or tumours. Temporal artery biopsy confirmed the presence of vasculitis; histology demonstrated transmural lymphohistiocytic inflammation, disruption of the elastic lamina and intimal proliferation. Prednisolone was started at 40mg daily. Four weeks after initially presenting she was asymptomatic and her inflammatory markers had normalised. Methods The case is discussed below. Results Temporal arteritis, or GCA, is primarily a disease of older adults; with age 50 often used as an inclusion criteria, and is more common in Caucasian populations. Limited reports exist of GCA in younger cohorts, but these are rare. An important differential in younger patients, such as ours, is juvenile temporal arteritis. This rare localised vasculitis affects almost exclusively the temporal artery. It is typically a disease of young males, who present with non-tender temporal swelling. Systemic symptoms are unusual and inflammatory markers are normal. Clinical or laboratory evidence of organ involvement, peripheral eosinophilia or fibrinoid necrosis on histology should prompt consideration of an AAV or PAN. Incidence of GCA increases in correlation with Northern latitude, with highest rates reported in Scandinavian and North American populations. GCA is rare in Asian populations. Higher diagnostic rates in countries where physicians have increased awareness of GCA proposed as an explanation for this difference; however differences in incidence are still observed between Asian and Caucasian populations presenting to the same healthcare providers. Conclusion GCA is an uncommon diagnosis in younger and non-Caucasian patients. Thorough investigation through ultrasound and biopsy helped increase our diagnostic confidence in this unusual case. Rheumatologists must be alert to atypical presentations in order to deliver prompt and potentially sight-saving treatment. Disclosure J. Ellis: None. K. Austin: None. S. Emerson: None.

P206 Rethinking old biomarkers: novel predictors of giant cell arteritis

Abstract 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 ( &amp;gt; =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.

A 78-year-old female with severe tongue pain: benefit of modern ultrasound

BackgroundGiant 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 presentationWe 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.ConclusionHigh-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.

A Review of the Dermatological Complications of Giant Cell Arteritis.

Giant cell arteritis (GCA) is characterized by granulomatous inflammation of large and medium-sized vessels. It is the most common vasculitis among elderly people in Europe and North America. GCA usually presents with ischemic cranial manifestations such as headache, scalp tenderness, visual manifestations, and claudication of the tongue and jaw. Thickness and tenderness of temporal arteries are the most recognizable signs of GCA on physical examination. Laboratory tests usually show raised acute phase reactants. Skin manifestations are uncommon in GCA and are rarely found as a presenting symptom of GCA. Necrosis of the scalp and tongue is the most common ischemic cutaneous manifestation of GCA. Although infrequent, when present it reflects severe affection and poor prognosis of GCA. Panniculitis-like lesions have been reported in the setting of GCA, with nodules being the most common finding. Other entities, such as generalized granuloma annulare or basal cell carcinoma have been occasionally described in GCA patients. Prompt recognition and initiation of therapy are crucial to prevent serious complications of GCA. When high suspicion of GCA exists, immediate administration of glucocorticoids is recommended. It is advisable to refer the patient to a specialist GCA team for further multidisciplinary assessment.

Clinical Experience With a Multidisciplinary Model of Vascular Ultrasound for the Evaluation for Giant Cell Arteritis

ObjectiveVascular 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.MethodsPatients 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.ResultsOf 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%).ConclusionWe 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.