Features Of Giant Cell Arteritis Research Articles

P029 Ocular syphilis: a mimicker for giant cell arteritis

Abstract Background/Aims Syphilis has seen a resurgence in the United Kingdom in recent years. It is a sexually transmitted disease caused by Treponema pallidum spirochaete infection, and typically presents as painless genital ulceration in its primary stage; left untreated it can progress rapidly to secondary and tertiary stages with multi-system involvement and wide-ranging symptoms, hence it being labelled ‘the great imitator’. We report a case of a patient presenting with classical features of giant cell arteritis (GCA), ultimately being diagnosed with neuro-ocular syphilis. Methods A 65-year-old man with a background of hypertension and migraines presented to our hospital with a 3-week history of temporal headache, scalp tenderness and jaw ache. He described lethargy, weakness and weight loss. On examination he had a prominent left temporal artery, with concurrent scalp tenderness; neurological and general medical examination were otherwise normal. Blood tests showed an erythrocyte sedimentation rate (ESR) of 66 and a C-reactive protein (CRP) of 115, leading to a strong clinical suspicion of GCA. He was commenced on 60mg of oral prednisolone with immediate improvement of his symptoms at this dose. Subsequent temporal artery ultrasound and temporal artery biopsy were negative for GCA. On tapering his steroids to 40mg he experienced partial relapse of his symptoms. Following Neurology review his steroid taper was adjusted to decrease more slowly. On 20mg of prednisolone the patient began experiencing stabbing retro-orbital pain and visual blurring, and was assessed by Ophthalmology with no local cause for his symptoms identified - he was therefore pulsed with intravenous methylprednisolone for a flare of GCA. Despite an initial response, the patient continued to experience intermittent symptoms on steroids; imaging with PET-CT did not show evidence of inflammation or malignancy and an MRI of his brain and orbits was normal. Results The patient eventually re-presented to Ophthalmology with floaters, pain and decreased vision and a spot diagnosis of posterior placoid chorioretinitis was made after retinal examination. Characteristic yellowish ovoid lesions confluent in the macular area were noted, as well as ellipsoid zone loss with nodular retinal pigment epithelium on optical coherence tomography (OCT) imaging. This condition is pathognomonic of neuro-ocular syphilis and serological testing confirmed the diagnosis, with a high rapid plasma reagin (RPR) titre of 1:512. He was treated with a course of intramuscular Ceftriaxone with a rapid resolution of his symptoms, and was successfully weaned off all steroid therapy. Conclusion Diagnosing GCA remains a clinical challenge for rheumatologists, especially in cases with typical symptoms and signs but negative confirmatory testing on imaging and histology. There are a wide range of conditions that resemble GCA, and neuro-ocular syphilis is one such diagnosis that has been reported in the literature as a mimic, as demonstrated by this important case. Disclosure S. Ali: None. S. Lekh: None. C. Tench: None.

Presenting Features of Giant Cell Arteritis with Active Versus Healed Arteritis on Biopsy

ABSTRACT Giant cell arteritis (GCA) is often categorised as ”active” or ”healed” on temporal artery biopsy (TAB). The purpose of this study was to compare the initial clinical presentation of patients with GCA according to active versus healed arteritis on TAB. A retrospective chart review was performed for patients with biopsy-proven GCA (BP-GCA) at a single academic medical institution from a previously reported cohort. The arteritis on TAB was categorised as “active” or “healed” based on the pathological reports. Demographic information, clinical presentation, past medical history, and test results were collected from the date of TAB. These baseline characteristics were entered into the GCA Risk Calculator. Of 85 patients with BP-GCA, 80% had active and 20% had healed disease according to histopathology. A higher percentage of those with active arteritis had ischaemic optic neuropathy (ION) (36% versus 6%, p = .03), elevated erythrocyte sedimentation rates (92% versus 63%, p = .01), elevated C-reactive protein levels (79% versus 46%, p = .049), GCA risk score > 7.5% (99% sensitivity, 100% versus 71%, p < .001), higher mean GCA risk calculator scores (neural network p = .001; logistic regression p = .002). Patients with healed arteritis were less likely to have visual manifestations than the active arteritis group (38% versus 71%, p = .04). Patients with active vasculitis on biopsy had higher rates of ION and elevated inflammatory markers, as well as higher predictive scores from the GCA risk calculator. Further research is needed regarding correlation of biopsy findings and risk of complications or relapses.

Giant cell arteritis: A population-based retrospective cohort study exploring incidence and clinical presentation in Canterbury, Aotearoa New Zealand.

To determine the epidemiology and clinical features of giant cell arteritis (GCA) in Canterbury, Aotearoa New Zealand, with a particular focus on extra-cranial large vessel disease. Patients with GCA were identified from radiology and pathology reports, outpatient letters and inpatient hospital admissions in the Canterbury New Zealand from 1 June 2011 to 31 May 2016. Data was collected retrospectively based on review of electronic medical records. There were 142 cases of GCA identified. 65.5% of cases were female with a mean age of 74.2 years. The estimated population incidence for biopsy-proven GCA was 10.5 per 100,000 people over the age of 50 and incidence peaked between 80 and 84 years of age. 10/142 (7%) people were diagnosed with large vessel GCA, often presenting with non-specific symptoms and evidence of vascular insufficiency including limb claudication, vascular bruits, blood pressure and pulse discrepancy, or cerebrovascular accident. Those with limited cranial GCA were more likely to present with the cardinal clinical features of headache and jaw claudication. Patients across the two groups were treated similarly, but those with large vessel disease had greater long-term steroid burden. Rates of aortic complication were low across both groups, although available follow-up data was limited. This study is the first of its kind to describe the clinical characteristics of large vessel GCA in a New Zealand cohort. Despite small case numbers, two distinct subsets of disease were recognized, differentiating patients with cranial and large vessel disease. Our results suggest that utilization of an alternative diagnostic and therapeutic approach may be needed to manage patients with large vessel disease.

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.

Aortic aneurysm associated with rheumatoid aortitis

A 59-year-old female, presented with dry cough, burning micturition with urgency and frequency for 2 months. She was known to have hypertension, hypothyroidism and rheumatoid arthritis for the past 30 years; and was receiving oral methotrexate 20 mg per-week. She also had complianed of low backache for 2 months. No phenotypic features of Marfans/Ehler Danlos were evident. Cardiovascular system examination was unremarkable. Chest radiograph showed mediastinal widening with a large fusiform descending thoracic aortic aneurysm. Computed tomography (CT) of the chest showed subpleural fibrosis and fusiform dilatation of the aorta from the origin till the supracoeliac aorta. CT aortogram revealed an irregular non-enhancing wall thickening and calcification of the aorta with fusiform dilatation (4.5 cm × 4.6 cm in the ascending aorta and 4.4 cm × 4.3 cm in the distal descending aorta). The patient did not have any features of giant cell arteritis (GCA)/Takayasu's arteritis; Venereal Disease Research Laboratory (VDRL) test was negative. The patient was diagnosed to have aortic regurgitation due to long standing rheumatoid arthritis and was referred to the department of cardiothoracic and vascular surgery for further management.

P030 Antiphospholipid syndrome and giant cell arteritis: a coincidence or connection?

Abstract Background/Aims Antiphospholipid syndrome (APS) is a multisystem autoimmune disorder, which can be primary or associated with other conditions including systemic lupus erythematosus. Several studies have reported antiphospholipid antibodies, in patients with different vasculitides but little is known about the occurrence of APS and giant cell arteritis (GCA). We report a case of a patient diagnosed with concomitant extracranial GCA and APS. Methods A 74-year-old lady presented to the Emergency department following a fall at home. On further questioning, she reported a rapid history of weight loss (4kg in the previous 4 weeks) but no classical features of giant cell arteritis. Her temporal arteries were pulsatile bilaterally, not tender or thickened. Cranial nerve examination including fundoscopy was normal. Her gait was apraxic, with short stride length and veering to the right with a narrow base. The rest of the examination was unremarkable. Results Laboratory investigations showed an elevated CRP 98mg/L with a normal ESR 23mm/h, ferritin 380 ng/ml, negative ANCA and ANA, with an unremarkable myeloma screen. Microbiology investigations demonstrated negative serial blood cultures, hepatitis screen, and quantiferon. She subsequently underwent intracranial imaging [CT and MRI brain with Gadolinium] which did not demonstrate acute pathology. A PET CT confirmed features in keeping with a large vessel vasculitis without aneurysmal formation. She was treated with pulsed IV methylprednisolone 500mg for 3 consecutive days and started on 60mg oral prednisolone daily. Two days after starting this treatment she developed acute onset double vision: ophthalmologic examination revealed new onset right internuclear ophthalmolplegia (INO). A subsequent CT head angiogram showed non critical stenoses in aorta at origins of great vessels, left vertebral artery, and carotids. Interval MRI brain showed a new infarct in pons corresponding with her INO. Bloods revealed triple positive anti-phospholipid antibodies - [IgG anticardiolipin antibody 102 GPL (0-13), anti-β2 glycoprotein I antibodies 59.8 U/ml (0-18), Lupus anticoagulant detected]. She was commenced on warfarin [target INR 2.5] and aspirin. Due to mental health history and large vessel manifestations, she was started on IV Tocilizumab 8mg/kg and her prednisolone dose was reduced to 40mg when her CRP normalised. Following hospitalisation, she had persistent visual disturbance with ongoing poor spatial co-ordination. Although she remained ataxic, her functional mobility improved. She was transferred to a local stroke unit and was discharged home. Conclusion In summary, this is a case of GCA and APS, treated with prednisolone, aspirin, warfarin and tocilizumab. There is increasing evidence describing the presence of antiphospholipid antibodies in patients with vasculitis. However, the role of these antibodies in GCA and the clinical significance remains unclear. This case reports highlights the need for physicians to consider APS in patients who have a history of GCA and subsequently develop arterial or venous embolic events. Disclosure L. Sammut: None. E. Htut: None.

Giant Cell Arteritis and COVID-19: Similarities and Discriminators. A Systematic Literature Review.

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. 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. 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. 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.

Clinical and Radiographic Features of Giant Cell Arteritis With Intracranial Involvement.

ObjectiveGiant cell arteritis (GCA) is a large‐vessel vasculitis that primarily affects the aorta and its branches. Extracranial branches of the carotid artery are frequently affected; however, intracranial involvement in GCA is rare.MethodsA retrospective medical record review was performed to identify all patients with intracranial GCA (IC‐GCA) from January 1996 through May 2018.ResultsNine patients with IC‐GCA were included (78% male; mean age, 72.1 years [SD: 7.9]). All patients met ACR criteria for GCA. The median time from onset of GCA to intracranial involvement was 0.6 months (interquartile range: 0.1‐5.1). All patients had neurologic symptoms, 89% had an ischemic cerebrovascular event. Transient or permanent vision loss was frequent (56% of patients). IC‐GCA was diagnosed by cranial imaging in all nine patients. Intracranial vasculitis most commonly affected the internal carotid artery (100%), followed by the vertebral artery (67%), posterior cerebral artery (67%), middle cerebral artery (44%), anterior cerebral artery (33%), and posterior inferior cerebral artery (11%). Intracranial vessel stenosis was present in 89%, occlusion in 33%, wall thickening in 33%, and dilation in 11%.All patients received glucocorticoids. Additional therapeutic agents included cyclophosphamide (67%) and tocilizumab (22%). Despite treatment, five patients had rapid deterioration and mortality. Comparing IC‐GCA patient survival to the expected rates from the US population, the standardized mortality ratio (95% CI) for IC‐GCA was 58.1 (18.9‐135.6).ConclusionAlthough rare, IC‐GCA is associated with significant morbidity and mortality. It occurs predominantly in men and presents with ischemic cerebrovascular events. Current treatment strategies appear to be of limited efficacy for IC‐GCA.

P51 Serious pathology mimicking GCA: two cases of carcinoma tongue

Abstract Background Jaw and tongue pain with constitutional symptoms and raised inflammatory markers are considered pathognomonic ischemic features of giant cell arteritis (GCA). Temporal artery ultrasound (US) (or biopsy) available in GCA fast-track clinics (FTC) for a rapid assessment of patients with suspected GCA. Atypical presentation and negative US or biopsy require further workup to look for an alternative diagnosis. ENT pathology can occur as a mimic of GCA. Herein we discuss two clinical cases of squamous cell cancer tongue presenting with signs and symptoms that resemble GCA. Methods We have put together a case report. Results Case-1: A 58-year-old male with a recent presumed diagnosis of relapsing GCA was referred with worsening visual symptoms and right eye pain despite ongoing steroid treatment (60mg), for consideration of Tocilizumab (TCZ). His initial presentation was 5 months ago with a right parietal and retro-orbital headache and blurred vision. He had a normal eye examination. His C-reactive protein (CRP) was raised (24). He was started on prednisolone 40 mg for GCA with complete resolution of symptoms with normalised CRP within weeks. Several weeks later, symptoms reoccurred. CT brain, abdomen and pelvis was normal and temporal artery biopsy negative. His prednisolone was increased to 60 mg. 6 months later, his jaw and tongue pain worsened, and he was treated with pulsed methylprednisolone. Due to partial response to steroids, he was referred to consider TCZ. He had tender left TMJ with normal temporal artery US. Urgent MRI head and neck revealed a left posterior tongue mass with the histology confirmed poorly differentiated squamous cell carcinoma. He was managed with chemo and radiotherapy. Case-2: A 75 years old female, presented with right scalp pain, tongue pain, painful swallowing and chewing. Her blood investigations were normal except a raised ESR (48) and presumed GCA she was started on steroids (60 mg). she had initially good response but, within a few weeks, her symptoms returned. She was then referred to our FTC. Temporal artery US and biopsy were normal. MRI of the head and neck showed a large mass seen in the right half of the posterior tongue extending into the deep aspect of the anterior tongue. Histology confirmed poorly differentiated Squamous cell carcinoma. She was treated with a combination of chemotherapy and radiotherapy. Conclusion GCA mimics represent a major diagnostic dilemma. FTC helps to stratify the GCA from mimics. Careful evaluation of the history, examination as well as a temporal artery US helps to exclude GCA and aids prompt requesting of appropriate tests to find an alternative diagnosis such as tongue cancers as in our cases. We have now introduced negative weightage for consideration of alternative diagnoses in our GCA probability score. Disclosures A. Kayani None. A. Sebastian None. L. Borukhson None. M. Whitlock None. B. Dasgupta Consultancies; Roche, Sanofi. Grants/research support; Roche.

Immunohistochemical Interplay in Associated Large Vessel Vasculitis and consequent Ascending Aortic Aneurysm and Review of Literature

Large vessel vasculitis (LVV), such as giant cell arteritis (GCA) and Takayasu’s arteritis (TA), are autoimmune diseases characterized by granulomatous inflammation of large vessels, including the aorta and its branch vessels. Disease etiology is still unknown. Comparative immunohistochemical (IMH) studies between GCA and TA cases might be important. The purpose of the study was to assess and to compare composition and distribution of the inflammatory infiltrate of the ascending aortic aneurysmal wall. Ascending aortic aneurysm (AAA) tissue blocks belonging to one male patient of 59 years and one female patient aged 36 years, diagnosed with GCA and TA, respectively, were used for histological and IMH analysis. Immunohistochemistry staining was performed using antibodies directed against markers of lymphocytes and macrophages. Histological analysis showed in both cases lymphocytes, macrophages, epithelioid histiocytes and multinucleated giant cells that infiltrate all layers of the arterial wall, intimal hyperplasia and adventitial fibrotic changes of different degrees. Immunohistochemical examination showed that the media and adventitia from GCA aortitis case had increased number of T cells and macrophages compared with TA aortitis case, and a less number of B cells in both. Our study made a morphologic and morphometric analysis of lymphocyte infiltration within aneurysm aortic tissue and demonstrated that immune inflammation is a typical feature of GCA and TA diseases. We also noted the utility of immunohistochemical staining of the aortic biopsies for evaluation of indeterminate or healing GCA and TA cases. Keywords: immunohistochemical, granulomatous inflammation, aortic aneurysm, large vessel vasculitis

48. Dry cough and pleural effusion as presenting features of giant cell arteritis

IntroductionGiant cell arteritis is a vasculitis which predominantly affects the carotid artery and its extra-cranial branches. Its classical presentation is that of headache with scalp tenderness but should be considered in anyone over the age of fifty years with transient visual symptoms, unexplained facial pain and/or jaw claudication. As a systemic vasculitis, clinical signs may vary, however respiratory complications compared to other vasculitides are uncommon. We present a case of new onset pleural effusion as a presenting feature of giant cell arteritis.Case descriptionA seventy-year-old man previously fit and well was referred to respiratory clinic after a CXR done for ongoing non-productive dry cough showed a small right sided pleural effusion. Accompanying symptoms included a reduced appetite with around 6kg weight loss over the preceding month.His past medical history included a TIA, occasional palpitations and prostatectomy for a benign prostatic hypertrophy. His only regular medication was aspirin. He never smoked and was a retired communications engineer with no known history of asbestos exposure.On examination he had no peripheral stigmata of respiratory disease and chest auscultation was clear. He had routine blood tests which revealed a normocytic anaemia (Hb 108), an elevated CRP (145.6) and elevated ESR (131). LFTs were mildly deranged and U&Es were unremarkable.A subsequent CT scan was arranged which confirmed a small right sided pleural effusion and a small pericardial effusion. General soft tissue oedema surrounding the upper abdominal organs was also noted. There was no evidence of malignancy.For workup of anaemia, he underwent a gastroscopy and colonoscopy. Gastroscopy was normal and colonoscopy revealed a single pedunculated sigmoid polyp which histologically was an adenoma. An echocardiogram showed no evidence of heart failure. 24 hour ECG telemetry was arranged due to palpitations but this was essentially normal.The patient eventually developed a bilateral temporal headache and malaise. He was commenced on 40mg prednisolone. While awaiting temporal artery biopsy he subsequently developed jaw claudication and prednisolone was increased to 60mg a day. Biopsy confirmed giant cell arteritis.All his symptoms improved with steroids including fatigue, headaches, cough and he started to regain weight. Similarly biochemically all abnormalities normalised. A subsequent CXR showed complete resolution of the pleural effusion.DiscussionGiant cell arteritis (GCA), like all systemic vasculitides, has been recognised to often involve the respiratory system. This has been reported to be in as high as 31% of patients with GCA. Notably, a persistent dry cough associated with fever has been recognised to be the most common respiratory symptom and has also been found to be one of the more atypical initial manifestations of GCA. This correlates with raised inflammatory markers and both of which were seen in the subject of this report. Although presence of a cough does not appear to correlate with other clinical symptoms of this particular vasculitis, it has been shown to markedly improve in response to steroids, suggesting its aetiology is GCA driven. The presence of pleural effusion however is far less common and indeed is exceptionally rare to be part of the initial presentation of GCA. There are a handful of cases in the literature, and most often the finding of pleural effusion coexists with more typical signs of GCA. The pleural effusion in our case was relatively small and was not considered safe for diagnostic aspiration. Rapid and complete resolution of an otherwise persistent pleural effusion with commencement of prednisolone points to GCA as the primary cause of the pleural effusion.Key learning pointsThis case shows that GCA can rarely present as cough and pleural effusion which may precede more typical clinical features often associated with GCA.The importance of recognition of GCA as the cause of pulmonary manifestations in such cases is that appropriate steroid therapy can induce complete resolution and avoid the need for further, potentially harmful invasive investigations. Our case further highlights the complexity of large vessel vasculitis such as GCA due to their varied presentation and symptoms. In patients with an unexplained weight loss, aside from malignancy the differential diagnoses should include vasculitis.Conflicts of interestThe authors have declared no conflicts of interest.

33. A case of ocular ischaemic syndrome caused by severe giant cell arteritis

IntroductionThis case describes a very aggressive form of giant cell arteritis (GCA) complicated by ocular ischaemic syndrome (OIS), complete bilateral visual loss and bilateral scalp muscle necrosis, despite treatment with high dose intravenous steroids. OIS, defined as ischaemia of all intraorbital and intraocular structures, is rare, estimated at 7.5 cases per million. This case also highlights the use of PET CT in management of complex cases of GCA.Case descriptionA 79-year-old lady presented with sudden onset pain above her right eye. A month earlier she had undergone curative surgery for infiltrating adenocarcinoma of the colon. 6 months earlier she had complained of bilateral shoulder pain. A month earlier she had developed pain on chewing and was unable to fully open her mouth. The next day she was reviewed in the eye hospital due to 24hrs of intermittent blurred vision. CT head was normal. Fundoscopy was normal and she was diagnosed with ocular migraine. Later that evening she lost vision in the right eye. Bloods showed CRP 58 and ESR 43. She was given 5 days of IV methylprednisolone (5x1g). Despite this she developed a right 6th nerve palsy. 2 days later her left eye developed visual loss, complete ptosis and loss of eye movements in all directions. MRI head with gadolinium showed temporoparietal juxtacortical diffuse white matter signal abnormalities.She underwent extensive investigation. ANCA was negative, lumbar puncture normal and temporal artery biopsy showed extensive fibrinoid necrosis of the vessel wall, with possible granulomata but no eosinophilia. Oral cyclophosphamide (2mg/kg) was started. 5 days later she developed severe bi-temporal pain and a rash which progressed to severe scalp necrosis. There were no vesicles to suggest shingles. Ten days later she suffered an upper gastro-intestinal bleed requiring a 4 unit transfusion. Gastroscopy showed mild gastritis. After this, cyclophosphamide was withheld, following discharge on steroids alone. Bilateral scalp necrosis remained a problem, complicated by pseudomonas infections. When a decreased left radial pulse was noted a PET CT was arranged. This showed active extensive vasculitis of the aorta. At this point methotrexate 15mg per week was added. The scalp lesions rapidly healed and the radial pulse returned to normal. The CRP has remained consistently below 5. DiscussionAn antiplatelet agent, clopidogrel was started due to the ischaemic cerebral findings on MRI, but it probably contributed, along with steroids, to her upper GI bleed. Antiplatelet therapy is no longer recommended routinely in the management of GCA. However in this case the diagnosis was still uncertain. None of us had ever seen orbital infarction syndrome as a complication of GCA. Perhaps a PET CT earlier on in the course of this lady’s disease would have made us more secure in the diagnosis. It could be argued that given the severity of her disease, methotrexate or even tocilizumab should have been commenced earlier. In fact, cyclophosphamide was used initially as it wasn’t clear if this was a medium vessel vasculitis. This was withheld when she had a large upper GI bleed (Hb dropped to 76). Once recovering, her CRP was down to 10. By this time the temporal artery biopsy was available, the ANCA was negative and GCA was felt to be the most likely diagnosis. It was also felt that given the bilateral visual loss, the most feared complication of GCA had already occurred so what was there to gain by using such strong immunosuppression? Hence cyclophosphamide was discontinued, and she was discharged on steroids alone. The addition of methotrexate had an impressive effect in terms of healing her weeping, secondarily infected scalp necrosis. Methotrexate was added in light of the PET CT which showed active large vessel vasculitis. The scalp necrosis, on reflection was due to an ongoing vasculitis that was being undertreated.There were missed opportunities for starting steroids earlier in the course of the disease: polymyalgic symptoms 6 months earlier, jaw claudication a month earlier. However it remains uncertain whether or not earlier treatment would have altered the course of this aggressive, severe disease. Key learning pointsScalp necrosis, which very easily can be mistaken for shingles or infections complicating steroid treatment, can be a feature of GCA. Orbital infarction syndrome can be a feature of GCA. Early PET CT might have clarified the diagnosis. Methotrexate should probably have been started once the limited response to IV steroids was apparent. Antiplatelet agents are no longer recommended in the updated BSR guidelines for managing GCA.Conflicts of interestThe authors have declared no conflicts of interest.

Patient-reported involvement of the eighth cranial nerve in giant cell arteritis

IntroductionThe frequency of eighth nerve lesions in patients with giant cell arteritis (GCA) has rarely been examined. However, sudden onset deafness has been recorded as a presenting feature of GCA on several occasions. This study sought to establish how common this and other symptoms of eighth nerve involvement are in a large retrospective survey.MethodsWe contacted 170 patients with GCA and 250 matched PMR patients, inviting them to participate in a questionnaire survey of symptoms of eighth nerve dysfunction. We compared the presence of deafness, tinnitus, loss of balance and vertigo in both groups and examined the relationship between the onset of these symptoms and other features of GCA.ResultsA total of 317 patients were recruited. The percentage of patients with symptoms of possible vestibulocochlear disease prior to commencement of steroid therapy was significantly greater among GCA patients than PMR patients for all domains. Hearing loss which was twice as common in GCA as in PMR (53% vs 26%) [p = 0.001]. Deafness was concurrent in 35% of GCA patients with other symptoms and 45% reported colocation with headache. Recovery with steroids occurred in 56% of these.ConclusionSymptoms of eighth nerve dysfunction are present in over half of patients with GCA. Recovery with steroids was predicted by concurrence with headache in terms of both timing and location. It appears that eighth nerve involvement, especially acute hearing loss, is a not infrequent feature of GCA and often responds well to steroid therapy. Clinicians should enquire about these symptoms when evaluating a patient for possible GCA.Key Points• Deafness is a frequent presenting feature of giant cell arteritis.• Vertigo, tinnitus and loss of balance are also often reported by GCA sufferers.• Steroid therapy is more likely to relieve these symptoms if they are ipsilateral and concurrent with headache.

Temporal artery biopsies in south-east Scotland: a five year review.

Temporal artery biopsy is the gold standard investigation for the diagnosis of giant cell arteritis. The aim of this retrospective study was to investigate the use of temporal artery biopsy in diagnosing giant cell arteritis in south-east Scotland over a five-year period. We aimed to quantify success rates, and predictive factors for a positive biopsy, as well as compare the different specialities performing the biopsies. The data should enable the development of better criteria for referral for investigation of giant cell arteritis. Methods Patients were identified using a database of temporal artery biopsies generated by the pathology department in NHS Lothian (south east Scotland), for all biopsies examined between January 2010 and December 2015. An electronic patient record was used to retrospectively examine the records of patients in the database. Results A total of 715 biopsies were included in the study, of which 250 (35.0%) showed features of giant cell arteritis. The main predictors for a positive biopsy were age at biopsy, specialty performing biopsy, erythrocyte sedimentation rate, jaw claudication/pain, and ophthalmic symptoms. The most important predictor of a positive biopsy was erythrocyte sedimentation rate. The length of biopsy was not found to be a predictor of positive biopsy; however, diameter of biopsy was predictive. Conclusions We have shown that many temporal artery biopsies are negative, and finding ways to reduce the number of patients unnecessarily undergoing biopsy will be essential in reducing workload and streamlining services. This study demonstrates some key predictive factors for patients with positive biopsies. The study also shows that a large proportion of biopsies taking place do not result in the recommended length of specimen, but this does not necessarily reduce the likelihood of a positive biopsy.

Correlation of clinical profile and specific histopathological features of temporal artery biopsies.

This study sought to correlate the clinical features of patients with giant cell arteritis (GCA) who present with ophthalmic symptoms and signs, with 2 specific histopathological findings-the presence of giant cells and arterial wall neoangiogenesis. The goal was to assess if these pathological features might be useful in guiding the approach to patient management. Medical charts were retrospectively reviewed from 58 patients who underwent a temporal artery biopsy at a single institution. Detailed information was collected about the clinical presentation and course, with an emphasis on visual function. Histopathological and immunohistochemical techniques were used to examine temporal artery biopsies for evidence of inflammation. Correlations were made between the clinical data and the presence of giant cells and neoangiogenesis. Twenty-one (34%) biopsies were positive for inflammation consistent with GCA. Although the percentage of positive biopsies with giant cells was high, neither the presence of giant cells nor neoangiogenesis was predictive of a patient's presenting visual symptoms, severity and bilaterality of vision loss, other ophthalmic manifestations of GCA, presence of headache or jaw claudication, or erythrocyte sedimentation rate. Giant cells were more common in patients with recent weight loss. Immunohistochemistry confirmed diagnoses but did not alter the clinical course or treatment plan. There was no correlation between the clinical, specifically visual, features of GCA and the presence or absence of giant cells or neoangiogenesis in temporal artery biopsy specimens. Although the presence of neoangiogenesis may be important in the pathogenesis of GCA, our study showed no correlation between this finding and the clinical course.

Clinical study of giant cell arteritis in our hospital.

To investigate clinical and laboratory features of giant cell arteritis (GCA). We included 24 patients (6 men, 18 women; mean age 69.8 years) in this study. GCA was diagnosed based on the American College of Rheumatology 1990 classification criteria. Mean serum C-reactive protein was 9.03 mg/dl. GCA was classified into three types: classic temporal arteritis type (cranial GCA, nine patients); large-vessel type, affecting the aorta and its major branches without temporal arteries (12 patients); generalized type, affecting both temporal arteries and large vessels (three patients). Swelling and tenderness of temporal arteries were recognized in temporal arteritis and generalized arteritis. Ten of these patients also had histopathologic findings of arteritis, including giant cells in biopsy specimens. Examination of HLA-class 1 expression showed that one patient with cranial GCA, three with generalized GCA, and seven with large-vessel GCA were positive for HLA-A24, and four patients with large-vessel GCA were positive for HLA-B39. One patient with cranial GCA, one with generalized GCA, and six with large-vessel GCA were positive for HLA-B52. Nine patients were positive for anti-phospholipid antibodies (seven for anti-cardiolipin antibody immunoglobulin G, seven for anti-cardiolipin β2-glycoprotein-1 antibody, one for lupus anticoagulant). Our study demonstrated that HLA-class 1 expression in GCA resembles that in Takayasu arteritis, suggesting that these two arteritis types share the same genetic background. In contrast, the difference in the prevalence of anti-phospholipid antibodies in GCA and Takayasu arteritis patients shows a difference in the characteristic aspects of these two arteritis types.

Temporal artery biopsies; do they make the cut?

Background and AimsTemporal artery biopsy is the gold standard investigation for giant cell arteritis. Guidelines recommend that specimens should measure no less than 1 cm and ideally more than 2 cm in length, as this influences the likelihood of biopsy positivity. This audit investigates the extent to which temporal artery biopsies acquired in our hospital meet these guidelines.MethodsHistopathology reports for all temporal artery biopsies performed at University Hospital Ayr between January 2011 and June 2013 were examined.ResultsFifty-six temporal artery biopsy specimens showed a range in length from 0.5 cm to 3.1 cm, with a mean of 1.4 cm. Thirty-seven biopsies measured ≥1 cm (66%) and 13 were ≥2 cm (23%). Therefore, 19 samples (34%) did not meet the recommended standard. Just seven biopsies showed features of giant cell arteritis, with six of these measuring ≥1 cm in length (86%).ConclusionThe guidelines for temporal artery specimens are not being met at our centre. Furthermore, biopsies measuring ≥1...

Temporal artery biopsies: do they make the cut?

Temporal artery biopsy is the gold standard investigation for giant cell arteritis. Guidelines recommend that specimens should measure no less than 1 cm and ideally more than 2 cm in length, as this influences the likelihood of biopsy positivity. This audit investigates the extent to which temporal artery biopsies acquired in our hospital meet these guidelines. Histopathology reports for all temporal artery biopsies performed at University Hospital Ayr between January 2011 and June 2013 were examined. Fifty-six temporal artery biopsy specimens showed a range in length from 0.5 cm to 3.1 cm, with a mean of 1.4 cm. Thirty-seven biopsies measured ≥1 cm (66%) and 13 were ≥2 cm (23%). Therefore, 19 samples (34%) did not meet the recommended standard. Just seven biopsies showed features of giant cell arteritis, with six of these measuring ≥1 cm in length (86%). The guidelines for temporal artery specimens are not being met at our centre. Furthermore, biopsies measuring ≥1 cm are much more common in the small group of positive results. This validates the minimum recommended biopsy length and displays the importance of achieving this standard. We suggest changes to increase the number of biopsies meeting these guidelines, improving the accuracy of this invasive test.

Biopsy-negative, varicella zoster virus (VZV)-positive giant cell arteritis, zoster, VZV encephalitis and ischemic optic neuropathy, all in one

A 72-year-old man developed clinical features of giant cell arteritis (GCA) and ipsilateral ophthalmic-distribution zoster, followed within 2weeks by VZV encephalitis and 2months later by ischemic optic neuropathy. Temporal artery biopsy was histopathologically negative for GCA, but contained VZV antigen and VZV DNA in multiple non-contiguous (skip) areas. The collective clinical and laboratory findings revealed a remarkably close temporal association of zoster, multifocal VZV vasculopathy with temporal artery infection, biopsy-negative VZV-positive GCA and VZV encephalitis.

Leg Ulcers Associated With Giant Cell Arteritis Relapse

Giant cell arteritis (GCA) is a granulomatous vasculitis of the aorta and its major branches, with a predilection for the extracranial branches of the carotid artery. In June 2012, an 81-year-old Caucasian woman was referred to our dermatology service because of nonhealing ulcers on the right leg. The patient has a personal history of GCA diagnosed in 1991 and was treated with methotrexato and prednisone. She did not complain of asthenia, jaw claudication, and headache. On admission, her inflammatory markers were elevated: erythrocyte sedimentation rate was 52 mm/h and C-reactive protein level was 1.16 mg/dL. The rest of the laboratory tests and coagulative screening were within normal limits. On admission, she resumed methotrexate 5 mg/ week, folic acid 5 mg/week, prednisone 5 mg/day, and pantoprazole 40 mg/day. On physical examination, we found 3 crusted leg ulcers surrounded with a zone of erythema (Figure 1). The patient underwent to a 3-mm punch biopsy of the lesions and an arteriovenous echo color Doppler of the leg. After debridement, the wound was dressed with medicated bandage. The histological finding was atypical for leg ulcers (Figure 2). We observed ulceration; granulation tissue; sparse, perivascular, interstitial mixed mostly lymphocytes; neutrophils; intraluminal thrombi; extravasated erythrocytes; and granulomatous infiltrate made up of histiocytes in the mid and reticular dermis. The ultrasonographic examination showed preserved vascular function and atherosclerosis of the right femoropopliteal arteries, even without significant stenosis. We made the diagnosis of leg ulcers associated with GCA relapse. After treatment with higher doses of systemic glucocorticoids, the ulcers improved in about 1 month. The histological features of GCA were first recognized in 1932 by Horton et al, and so is also called “Horton’s giant cell arteritis.” GCA usually occurs in individuals aged 50 years and older, more often in women. Genetic or ambient factors probably contribute to the development of GCA, but the etiology is still unknown. GCA is responsible for a broad spectrum of manifestations due to the inflammatory involvement of vessel wall with lymphocytic and macrophagic infiltrates in the tunica media that can extend from the tunica intima to the avventitia and sometimes involving internal elastic lamina. Giant cells can be present. Granulomas and fibrinoid necrosis are seen infrequently. Acute-phase 476518 IJLXXX10.1177/1534734613476518The International Journal of Lower Extremity WoundsMisciali et al © The Author(s) 2011