Scalp Tenderness Research Articles

One Giant Step for Giant Cell Arteritis: Updates in Diagnosis and Treatment.

Purpose of ReviewGiant cell arteritis (GCA), a medium and large vessel vasculitis occurring in the aged, remains a formidable disease, capable of taking both vision and life, through a multitude of vascular complications. Our understanding of the spectrum of its manifestations has grown over the years, to include limb claudication, aortitis, and cardiac disease, in addition to the more classic visual complications resulting from of ischemia to branches of the external and internal carotid arteries. While a clinical presentation of headache, jaw claudication, scalp tenderness, fever and other systemic symptoms and serum markers are together highly suggestive of the disease, diagnosis can be challenging in those cases in which classic symptoms are lacking. The purpose of this review is to update the reader on advances in the diagnosis and treatment of giant cell arteritis and to review our evolving understanding of the immunological mechanism underlying the disease, which have helped guide our search for novel therapies.Recent FindingsThere is increasing evidence supporting the use of Doppler ultrasound, dedicated post-contrast T1-weighted spin echo MRI of the scalp arteries and PET scan, which can together improve our diagnostic accuracy in cases in which temporal artery biopsy is either inconclusive or not feasible. Advances in our understanding of the immunological cascades underlying the disease have helped guide our search for steroid-sparing treatments for the GCA, the most important of which has been the IL-6 receptor antibody inhibitor tocilizumab, which has been shown to reduce cumulative steroid dose in a large multicenter, placebo-controlled prospective study. Other biologic agents, such as abatacept and ustekinumab have shown promise in smaller studies.SummaryGCA is no longer a disease whose diagnosis is based exclusively on temporal artery biopsy and whose complications are prevented solely with the use of corticosteroids. Modern vascular imaging techniques and targeted immunologic therapies are heralding a new era for the disease, in which practitioners will hopefully be able to diagnosis it with greater accuracy and treat it with less ischemic complications and iatrogenic side effects.

Da li bi znaci gigantocelularnog arteritisa trebali biti detektovani kod pacijenata koji imaju herpes zoster?

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 Case of Invasive Paranasal Aspergillosis with Scalp Tenderness and Jaw Claudication

A Case of Invasive Paranasal Aspergillosis with Scalp Tenderness and Jaw Claudication

Beyond Giant Cell Arteritis and Takayasu's Arteritis: Secondary Large Vessel Vasculitis and Vasculitis Mimickers.

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

Atypical Large Vessel Vasculitis Presenting With Cholestatic Liver Abnormalities: Case-Based Review.

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.

EP31 Setting fire to sleeping giants: multiple autoimmune inflammatory diseases set off by chronic viral infection

Case report - IntroductionAutoimmune diseases are a group of disorders where the body produces an immune response against its own tissue constituents. The understanding of the immune mechanisms underlying autoimmunity has significantly deepened and broadened recently.The viral infection is a well-known trigger of autoimmunity and chronic hepatitis is known to cause various auto-immune diseases; organ-specific or systemic.Self-reactivity is part of the normal regeneration and healing. Auto-reactive T&B cells are kept dormant by the concerted action of a variety of mechanisms.Hepatitis C can disrupt this by molecular mimicry, by providing adjuvant and even by directly affecting B cell function.Case report - Case descriptionWe report a case of a 77 yr old Caucasian lady from South Africa who presented to Addenbrooke's hospital complaining of general decline, multiple constitutional symptoms, and low-grade fever for nearly 6 weeks. She did not have any infective symptoms. She had a past medical history of Type 1 DM, Hypothyroidism, Sjögren’s syndrome Osteoporosis and chronic hepatitis C infection which was treated 4 years ago. Family history was not significant, and she was a non-smoker.She was found to be thin with a BMI of 18, pyrexical with a temperature 37-38'c; otherwise, examination findings were unremarkable.Initial investigations revealed high inflammatory markers with an ESR of 99 and CRP of 120. WCC was within the normal range as were her renal and liver function tests. She was thoroughly investigated for Pyrexia of Unknown Origin. Her septic screen including Echocardiogram was negative. There was no detectable serum Para-protein and tumour markers were negative. Her CT scan of the chest abdomen and pelvis, MRI scan of the spine and PET scan were reported to be normal. The Serum Pro-calcitonin was low.Rheumatology team was enlisted at this point. On further assessment, the patient mentioned of having an ongoing mild generalised headache - described as a 'fullness in the head' and mild generalised scalp tenderness. She also had an aching pain in her legs. There was no history of visual symptoms, jaw, or tongue pain. On examination, she had generalised scalp tenderness which was worse over the left temporal artery.She had a temporal artery biopsy which showed typical features of GCA (disruption of IEL and giant cell) Following which she was commenced on steroids to which she had a remarkable response. After a month she was commenced on Tocilizumab with quick wean off steroids in view of her elevated risk for fractures.Case report - DiscussionThis lady believes to have contracted Hepatitis C in the early 80s during a surgical procedure for removal retained products of conception following an incomplete miscarriage in South Africa.She was found to be positive for hepatitis C in the early 90s when she tried to donate blood in the UK. She had genotype 1 hep C. on diagnosis her fibro scan was normal and live biopsy showed mild fibrosis. After regular surveillance during the following years, she was treated with AbbVie 3D regime and ribavirin in 2016 and she was declared to be clear in 2017 (Hepatitis C was undetectable).This lady received a diagnosis of Sjögren’s syndrome in the early 2000s which was based on the fact that she had Sicca symptoms, Positive ANA of 9.2, positive anti-La and anti-Ro, Rheumatoid factor of 13 and lymphocytic clusters in lip biopsy. There are many studies linking Hepatitis C and Sjögren’s syndrome. Sicca symptoms are common in hepatitis C, as chronic sialadenitis is a well-known extrahepatic manifestation. A recent meta-analysis showed a strong association between Hepatitis C infection and Sjögren’s syndrome (OR3.31).This lady was diagnosed with hypothyroidism in the early 90s. Thyroid autoantibodies were not checked then. Thyroid autoimmunity is quite common in Hepatitis C positive patients. Higher prevalence of anti-TPO (21%) and anti-thyroglobulin antibodies (17%) has been demonstrated in Hepatitis C positive population even before they develop hypothyroidism.She was diagnosed with Type 1 Diabetes mellitus in 1997 at the age of 55 after she presented with Diabetic ketoacidosis. She was strongly positive for Anti-GAD antibody (>2000). Again, pancreatic autoimmunity is highly prevalent in non-diabetic Hepatitis C population (1.4% vs 0.4%). There are several case reports of patients who developed type 1 DM after acute hepatitis C infection. Case report - Key learning pointsAs chronic infections are known to trigger autoimmune diseases, it is advisable to screen for them in patients with multiple or atypical autoimmune diseases. Treating the infection itself will lead to complete remission of these autoimmune conditions.

Temporal Artery Biopsy in the Workup of Giant Cell Arteritis: Diagnostic Considerations in a Veterans Administration Cohort.

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

Optimal temporal artery biopsy length for diagnosis of giant cell arteritis.

Giant cell arteritis is a major type of large vessel vasculitis.1 Abrupt-onset headache with or without scalp tenderness, temporal artery abnormalities, and claudication (in the jaw, tongue, and limbs) might be clinically prominent, accompanied by constitutional symptoms and, in about half of patients, polymyalgia rheumatica.2 Patients with giant cell arteritis show no specific laboratory features, but most have elevated acute phase reactants at disease onset. The 2018 update of the European League Against Rheumatism (EULAR) recommendations for the management of large vessel vasculitis advise a confirmatory test in all patients with suspected giant cell arteritis, either by imaging (ie, ultrasound, MRI, CT, or PET-CT) or histology (ie, temporal artery biopsy).

Polymyalgia rheumatica mit und ohne Großgefäßvaskulitis

Polymyalgia rheumatica (PMR) is characterized by rapidly evolving shoulder and pelvic girdle pain with fatigue, weight loss, night sweats and elevated CRP and ESR. Giant cell arteritis (GCA) can occur in PMR and vice versa. Headache and scalp tenderness are typical for GCA. GCA may be complicated by visual loss or by strokes.Imaging, particularly ultrasound, is helpful for distinguishing PMR from similar conditions such as shoulder osteoarthritis, rheumatoid arthritis and chondrocalcinosis. Subdeltoid bursitis, biceps tenosynovitis and hip joint effusions are common in PMR. The diagnosis of GCA needs to be either confirmed by imaging or by histology. Ultrasound is the imaging method of choice provided that expertise and adequate equipment are available. Inflamed arteries exhibit a concentric wall thickening. Patients with extracranial GCA are younger, more often female. Vasculitis commonly involves the aorta, subclavian arteries, axillary arteries and other arteries. The diagnosis of extracranial GCA may be confirmed by ultrasound, CT, MRI or PET.Prednisolone with a starting dose of 15-25 mg/d for PMR and of 40-60 mg/d for GCA results in rapid improvement of symptoms. Fast-track clinics provide clinical and ultrasound examinations by experts within 24 hours. Their introduction led to a decrease of visual loss in GCA. The prednisolone dose can be discontinued within 1 year in about 50 % of GCA patients. Additional treatment with tocilizumab allows to reduce flares and decrease glucocorticoid doses. Tocilizumab is particularly useful in patients with relapses and with increased risk of glucocorticoid side effects.

AB0488 CAN A GCA RISK STRATIFICATION SCORE BE HELPFUL IN CLINICAL PRACTICE?

Background:Giant cell arteritis (GCA) is the most common type of large vessel vasculitis. Typically it presents in patients over the age of 50 with a combination of temporal headaches, scalp tenderness, jaw claudication, raised inflammatory markers and visual disturbance. The diagnosis of GCA is often challenging and there is a difficult balance of over and under investigation. There have been several proposed scoring systems to help clinicians risk stratify patients who may present with suspected GCA. One such scoring system, published in 2017, showed clinical utility in a large international multi-centre study. Following analysis by logistic regression on data from 530 biopsies, Ing et al. developed a parsimonious prediction model comprising 5 candidate criteria: age, jaw claudication, ischemia-related loss of visual acuity, platelet count and logCRP (Figure 1).[1]Objectives:Increasingly, ultrasound doppler imaging is recognised and accepted as satisfactory means of confirming the diagnosis of GCA, with the presence of the halo sign characteristic for GCA. The aim of our study was to determine whether this GCA prediction model accurately predicts positive temporal artery biopsies in a large, real world UK cohort. In addition, we assessed whether this model accurately predicts positive temporal artery ultrasounds.Methods:A retrospective cohort study was performed using electronic medical records of patients referred for temporal artery biopsy (TAB) and temporal artery ultrasound (USTA) for suspected GCA. All TAB performed at the Royal Wolverhampton NHS Trust between June 2014 - June 2018 and all USTA performed between January 2015 - January 2019 were analysed. Patients who undergo USTA for suspected GCA at our centre routinely have bilateral temporal and axillary arteries scanned. Patients were excluded if they already had a previous diagnosis of GCA (and the clinical question was suspected flare), or if there was insufficient information available.Results:The total number of patients who underwent a confirmatory diagnostic test (either TAB or USTA) for suspected GCA was 187. Thirteen of these patients met the exclusion criteria, the remaining 174 patients were included for analysis. 126/174 patients underwent a TAB, 63/174 had an USTA. 15/174 had both these were included in the USS cohort because for all these patients the ultrasound was the first diagnostic test performed (Table 1). Our results appear to closely mirror the original multi-centre results with regards to prediction of biopsy positive GCA, with the centiles closely following those in the inception cohort. 0% of the ‘low’ risk probability biopsy cohort were misclassified - none had a positive biopsy. However, 8% of the ‘low’ risk probability ultrasound cohort were misclassified - 2 had a positive ultrasound.Table 1.Investigation outcome summaryTotal number of patients who underwent TAB +/or USS TA for?GCA187 - 13 patients rejectedN = 174TAB = 111USS = 63Of these 15 patients hadbothUSS & TABPositive TAB =31 (28%)Negative TAB =80 (72%)Positive USS =24 (38%)Negative USS =39 (62%)Conclusion:Our study, highlights that a probability score for GCA derived from a large multi-centre cohort of patients who were biopsy positive, predicts ultrasound positivity with similar accuracy. Our work reveals that scoring systems are not infallible but can be helpful in guiding clinical decision making

SAT0256 THE ROLE OF FAST-TRACK ULTRASOUND IN PREVENTING EARLY COMPLICATIONS AND RELAPSES IN GIANT CELL ARTERITIS

Background:Giant Cell Arteritis (GCA) is the most common form of primary systemic vasculitis, mainly affecting adults over 50 years old. Permanent visual loss (PVL) is one of the most feared complications, occurring in about 20% of cases, typically prior to initiation of high-dose glucocorticoid (GC) therapy. Color-duplex sonography (CDS) of temporal arteries (TAs) and large vessels (LVs) is recognized as a first-line diagnostic tool for patients with suspected GCA. A fast track approach (FTA), incorporating CDS has been associated to a significant reduction of PVL in two retrospective studies1,2.Objectives:To assess the impact of FTA on PVL and risk of relapses during follow-up compared to conventional care prior to the introduction of the FTA in our rheumatology clinic.Methods:Patients with new-onset GCA evaluated in our department from January 1998 to September 2019 were included in the study. The FTA approach for GCA was implemented since October 2016. The diagnosis of GCA was based on positive TAs and/or LVs CDS and/or a positive TA biopsy and clinical signs and symptoms of GCA. All patients were clinically examined by the same rheumatologist who performed the CDS. PVL was defined as total visual impairment in one or both eyes. Data on baseline clinical features and later outcomes were collected.Results:153 patients were included: 115 females (75.2%), mean age at diagnosis 71.6±8.2 years. Of these, 112 patients (73%) were evaluated conventionally and 41 (27%) with FTA. Patients in the FTA group were older (P=0.0002), presented more frequently with polymyalgia rheumatica symptoms, weight loss, jaw or tongue claudication and scalp tenderness (P<0.05 for all comparisons). The median duration of follow-up in the FTA group was shorter compared with the conventional group (1.5 vs 5.8 years). PVL occurred in 22 (19.6%) patients in the conventional group compared to 5 patient (12.2%) in the FTA, leading to a reduction of 37.9% in the relative risk of PVL with the FTA approach. Cumulative incidence of relapses and time to first relapse did not change after FTA introduction (P>0.05) (Fig. 1).Conclusion:The application of a FTA in GCA resulted in a significant reduction of PVL. However, the relapse rate did not seem to be influenced by the FTA, highlighting the need to implement further management strategies, besides earlier diagnosis and prompt initiation of GC, that would impact the course of the disease during long-term follow-up

Coventry fast track pathway for managing giant cell arteritis

Giant cell arteritis (GCA) is the commonest form of vasculitis seen in United Kingdom (UK). It usually presents with new onset headaches, scalp tenderness, jaw claudication or visual symptoms – acute visual loss is a catastrophic presentation and complication of untreated GCA. Hence, it is treated as a medical emergency. Most units have a protocol for assessing these patients and treating these patients promptly – usually this involves the general practitioner GP) starting steroids and sending the patient to secondary care for further evaluation. Recent utility of vascular Doppler ultrasound (US) has allowed many units to develop a fast track pathway for assessing and managing these patients. Coventry established its walk-in fast track pathway which included clinical assessment with rheumatology and/or ophthalmology along with US in 2013. Patients were normally seen within a few hours of referral and the pathway is unique in not needing GPs to start steroids in advance of their assessment in secondary care. This article describes the rationale for the way the pathway was designed and also describes the results of the initial pilot phase of this pathway.

Clinical Characteristics of Headache among a Sample of Iraqi Medical and Dental Students

Background: Headache is a painful condition related to a major lack of productivity, limitation of social activities and impairment of quality of life.Method: in this study a structured questionnaire was formulated in a form of multiple choices questions to gather information on the clinical characteristics, associated factors and the triggering factors along with the mode of treatment adopted by dental and medical undergraduate students of the University of Baghdad in the period from January to March 2019. Language of questionnaire was made simple and easily understandable to students. Students were assured of confidentiality and the participation was entirely voluntary.Results: in this study a total number of 340 students participated, 170 of them were medical students 76(44.7%) males and 94(55.3%) females, and the other 170 were dental students 45(26%) males and 125(74%) females .It was found that 170 medical students had 161 associated symptoms with headache , the most common associated symptoms was scalp tenderness 44(25.9%), while the least common associated symptom was diarrhea 4(2.4%).While 170 dental students had 162 associated symptoms, the most common associated symptoms was scalp tenderness 46 (27.2%), while the least common associated symptom was seizures 2 (1.2%). 170 medical students expressed 385 triggering factors for causation of headache. It was found that the most common triggering factors was irregular sleep 118(69.4%). And 170 dental students expressed 437 triggering factors for causation of headache. It was found that the most common triggering factors was stress 136 (80.5%). In this study the percentage of medical students who taking self medications was 88.82% and 92.35% for dental students also it was found that both medical & dental students had the highest percentage for using Paracetamol as analgesic, 119(70%) for medical students, and 126(74.6%) for dental students.Conclusions: the results of this study show that the majority of both medical and dental students suffer from headache with self medications used by the majority.

P36 Never say never: two cases of PET-CT negative but biopsy proven giant cell arteritis

Abstract Background Giant cell arteritis (GCA) usually affects the temporal arteries but can result in systemic vasculitis that can be seen on Positron Emission Tomography-Computed Tomography (PET-CT). Currently PET-CT is not used first line to diagnose cranial-GCA and temporal artery biopsy (TAB) remains the most common investigation to assess for this. There have been few studies performed to assess the use of PET-CT as an initial diagnostic tool in cranial-GCA. One study performed PET-CT for suspected GCA in their cohort of 64 patients. They found, when compared with TAB, global GCA assessment by PET-CT had a sensitivity of 92%, specificity of 85%, positive predictive value of 61% and negative predictive value of 98%. They concluded PET-CT had good diagnostic accuracy when compared with TAB and can be used as a first-line test to assess for GCA and rule out lower-risk GCA. Another study used PET-CT in steroid-naïve patients and also showed a high accuracy (sensitivity 64% and specificity of 100%) in diagnosing cranial artery inflammation. Methods We present two cases where the patient had symptoms of headache, constitutional symptoms and raised inflammatory markers, however PET-CT did not suggest GCA. The patients were subsequently re-referred later with ongoing symptoms and TAB confirmed GCA. Results Case 1: A 71-year-old woman presented with a one-month history of intermittent right sided headache and rigors. Her inflammatory markers were elevated (CRP of 115mg/L and ESR of 57mm/hr). Suspecting GCA, prednisolone 30mg od was commenced. Upon rheumatology review, it was felt her symptoms were atypical for GCA. Prednisolone was discontinued, and a PET-CT scan was arranged. This was performed ten days later and did not show any features of vasculitis. Six weeks later, the patient presented to ophthalmology reporting visual aura, ongoing headache with elevated inflammatory markers. Prednisolone 60mg od was commenced and a TAB was arranged. This was reported as partially treated GCA. Case 2: A 73-year-old gentleman had a prolonged admission for pyrexia of unknown origin. Headache was present throughout admission and rheumatology opinion was sought. A PET-CT did not show vasculitis. The patient was discharged when pyrexia improved however his inflammatory markers remained consistently high (CRP of 82mg/L and ESR 106mm/hr). One-month post-discharge, the patients GP contacted rheumatology concerned about ongoing headache and scalp tenderness. Prednisolone 40mg od was commenced and a TAB was arranged which reported features consistent with GCA. Both patients responded well to steroids. Conclusion As demonstrated, patients with biopsy proven GCA do not always exhibit vasculitis on imaging. A negative PET-CT did not reliably exclude GCA in our patients suggesting its use as a diagnostic investigation for GCA needs to be approached with caution. A TAB should also be performed when there is ongoing suspicion of GCA. Disclosures S. Khalid None. J. Maxwell Honoraria; BMS, Pfizer, Lilly, Abbvie. Other; Talks, BMS, Pfizer, Lilly, Abbvie.

Should Tocilizumab Be Used Routinely in New Patients With a Diagnosis of Giant Cell Arteritis?

Should Tocilizumab Be Used Routinely in New Patients With a Diagnosis of Giant Cell Arteritis?

Annals for Educators - 19 November 2019.

Annals for Educators - 19 November 2019.

41. A common rheumatology referral of vague constitutional symptoms and raised inflammatory markers

41. A common rheumatology referral of vague constitutional symptoms and raised inflammatory markers

40. A case of large vessel vasculitis presenting with limb claudication symptoms

IntroductionThis is a case report of extensive large vessel vasculitis which initially presented to the vascular team with limb claudication symptoms and an absent radial pulse. CT angiogram was suggestive of a large and medial vessel arteritis, without a significant elevation in ESR.Case descriptionA 71-year-old gentleman was referred from the vascular team to the rheumatology outpatient clinic. He initially attended orthopaedics and was awaiting shoulder surgery. He reported pain and intermittent colour changes in his left hand and was noted to have an absent radial pulse. CT angiogram was suggestive of a large and medium vessel arteritis such as giant cell arteritis. The CT aorta showed occlusion of the left axillary artery with significant narrowing of the left subclavian. He had a known history of cataracts, mixed frequency hearing loss, cervical spondylosis and previous arthroscopic sub acromial decompression surgery. He had intermittent mild jaw pain for a few months but no other clinical symptoms in keeping with vasculitis. He had no headache, scalp tenderness or constitutional symptoms. ESR was 41. Temporal artery biopsy was positive. Histology was in keeping with temporal arteritis but there was no giant cells or granulomata seen. CT PET showed increased tracer uptake within the subclavian, axillary and the common carotid arteries bilaterally. There was also slightly increased tracer extending inferiorly to the aortic arch and within the proximal descending thoracic aorta. He was commenced on 60mg prednisolone OD, omeprazole, adcal D3, alendronate, aspirin and cotrimoxazole. A month later, he presented with chest pain and was diagnosed with acute coronary syndrome. He had extensive imaging and there was no evidence of dissection. He had five coronary stents inserted and was commenced on dual anti platelet therapy for one year. He was commenced on tocilizumab as a steroid sparing agent taking into consideration the severity and extent of his disease, the need for dual antiplatelet therapy and increased bleeding risk.DiscussionThis was an interesting case of extensive large vessel vasculitis without a significant inflammatory response. He had extensive disease with both large vessel and cranial involvement. We held off on steroid treatment until he was investigated with CT PET and biopsy as his symptoms were longstanding. He was a high risk candidate for long term steroids given his recent MI and need for dual antiplatelet therapy. We opted for tocilizumab as a steroid sparing agent given the extent and severity of vessel involvement and increased bleeding risk on antiplatelet treatment. Tocilizumab is licensed for relapsing and remitting GCA and has the ability to limit steroid use and reduce the risk of relapse. Tocilizumab was used as an effective steroid sparing agent in this case.Key learning pointsThis case has shown that extensive large vessel involvement can be seen in vasculitis without a significantly inflammatory response. ESR was only 41 in this case despite the extent of vasculitis. Therefore we must keep an open mind when considering a diagnosis of vasculitis as inflammatory markers may be normal. Tocilizumab was used an effective steroid sparing agent and has helped to limit steroid use in the setting of increased bleeding risk. In patients presenting with these clinical findings, we would have a low threshold for requesting temporal artery biopsy. This patient has established damage due to the extensive vessel involvement and it will be difficult to assess whether symptoms are related to damage or disease activity. How do we best assess whether symptoms are related to damage or disease activity? What is the best imaging to monitor disease activity? How best do we manage this patient’s disease in the long term due to the extent of vessel involvement?Conflicts of interestThe authors have declared no conflicts of interest.

45. Large vessel vasculitis associated with common iliac artery aneurysm and limb ischaemia

IntroductionAn 81-year-old gentleman with no prior medical history presented with a 5-month history of gradual onset malaise and reduced appetite. Weight loss of 2 stone was noted. Mild intermittent headache was present. After 3 months, he developed intermittent claudication of the right leg. A diagnosis of giant cell arteritis (GCA) was made. Disease was corticosteroid resistant, on the basis of clinical findings, biochemistry and imaging. Tocilizumab was commenced. Imaging also revealed dissection of the proximal right common iliac artery. The intermittent claudication progressed to acute limb ischaemia, which responded well to conservative treatment with heparin.Case descriptionHeadache was unilateral, intermittent and lasted a few minutes only, although it was described as severe. There were no visual symptoms, no scalp tenderness and no jaw or tongue claudication. His mobility was severely impacted by intermittent claudication. He was previously playing 3 rounds of golf per week but exercise tolerance reduced to fifty metres. There were no specific risk factors for atherosclerotic disease. He was a retired head teacher and had never smoked. Alcohol intake was 3 units per week. He was not taking any medication.The predominant features in the history were systemic upset and weight loss and the initial focus was on ruling out malignancy. Extensive investigations were performed by the general practitioner (GP). Erythrocyte sedimentation rate (ESR) was 80 and C-reactive protein (CRP) 74. A full blood count and serum biochemistry were otherwise unremarkable. Immunoglobulins were normal with no paraprotein detected. Thyroid stimulating hormone (TSH) was within the normal range. Prostate specific antigen (PSA) was raised at 17.8 but urology investigations revealed no evidence of malignancy.Computed tomography (CT) of the thorax, abdomen and pelvis showed non-specific inflammation of jejunum & mesenteric fat. Subsequent magnetic resonance imaging (MRI) of the small bowel showed resolution of these changes but noted a chronic focal area of dissection at the proximal right common iliac artery.The GP commenced prednisolone 40mg daily, increased after twelve days to 60mg daily due to partial response. Review in rheumatology clinic two weeks later noted ongoing intermittent claudication. Headache had resolved and weight stabilised. The right temporal artery was difficult to palpate and the right ulnar pulse was absent. Temporal artery ultrasound scan (TA USS) in clinic demonstrated bilateral ongoing active inflammation. Three pulses of 500mg intravenous methylprednisolone were arranged.DiscussionWhilst ESR had initially improved to 10 and CRP to < 3, they subsequently increased to 53 and 45 respectively. Subsequent positron emission tomography with computed tomography (PET-CT) showed diffuse metabolic activity in thoracic aorta, bilateral subclavian, axillary and femoral arteries.On the basis of bloods, ongoing claudicant symptoms and strongly positive TA-USS and PET-CT, the disease was felt to meet criteria for steroid non-responsiveness. As per NICE guidelines, permission was sought and granted from the local tertiary centre to commence tocilizumab. The patient was noted to have diverticulosis on the basis of imaging but had never been symptomatic. After appropriate patient counselling on the risks of gastrointestinal perforation, a decision was made to proceed with treatment.The finding of dissection at the proximal right common iliac artery prompted urgent referral to the vascular surgery team. However, whilst awaiting review, the patient developed acute limb ischaemia with pallor, weakness and pain of the right leg. He was admitted and managed conservatively with intravenous heparin, followed by subcutaneous heparin and clopidogrel. He responded well to medical therapy and remains under vascular follow up. Notably, the aneurysm was retrospectively noted on CT scan imaging, confirming that it predated corticosteroid treatment.Key learning pointsWhilst aneurysm formation is a recognised complication of giant cell arteritis, they are typically aortic and involvement of lower limb arteries is rareThere is no consensus opinion on optimal surveillance of extra-aortic aneurysms in GCA; decisions should be made on a case by case basisTocilizumab is an effective treatment for refractory GCA. The current NICE guideline on its usage is based on the GiACTA study findingsConflicts of interestThe authors have declared no conflicts of interest.

46. GCA: Diagnosed with a heavy heart

IntroductionGiant cell arteritis (GCA) represents a common cause of uncertainty for physicians. GCA can present as an indolent or aggressive process with a wide spectrum of symptoms and signs. The key to diagnosis is good clinical acumen and awareness of both common and rare associated conditions. Here we describe a rare and atypical presentation of a patient with GCA and chest pain.Case descriptionAn 83-year old male was admitted to the medical take with 3 hours of crushing chest pain associated with diaphoresis and nausea. He had been started on 60mg prednisolone by his GP 3 days preceding this for suspected GCA (scalp tenderness, jaw claudication, polymyalgia). Past medical history included: hypertension, recent strokes (bilateral cerebellar infarcts 3 months prior), previous DVTs and COPD. Examination revealed no abnormalities. Admission ECGs showed sinus rhythm with fixed T-wave inversion in aVL and V3. Chest radiograph was normal. Bloods tests revealed mildly raised inflammatory markers (CRP, ESR 24) and a dynamic troponin (324 – 1241 – 1431 ng/L). He was diagnosed with a non-ST elevation myocardial infarction (NSTEMI) and was referred to Cardiology for an angiogram. He was started on dual antiplatelets and high dose atorvastatin. On review by Rheumatology, his recent history of bilateral posterior circulation strokes was investigated further. Multi-level bilateral vertebral artery stenosis was seen on CT angiogram at the regional hyper-acute stroke unit and diagnosed as atheromatous in origin due to: a negative right-sided temporal artery biopsy, no GCA symptoms and modest inflammatory markers (CRP in the 30s and ESR in the 20s). Due to the onset of clear and acute GCA symptoms a left-sided temporal artery biopsy and PET scan was scheduled. Prednisolone was reduced to 40mg OD due to concern over its atherogenicity during a suspected NSTEMI. Coronary angiogram went on to show clear arteries and a subsequent cardiac MRI confirmed acute myocarditis. The left temporal artery biopsy revealed chronic inflammation with scattered giant cells. The patient was diagnosed with: GCA, extra-cranial vasculitis and myocarditis. He was treated with intravenous methylprednisolone, a weaning course of prednisolone and started on methotrexate (15mg weekly). The patient’s chest pain settled soon after admission and his troponin and inflammatory markers fell with prednisolone. DiscussionGCA seldom presents with myocarditis and can lead to significant morbidity and mortality. This remains an important differential in a patient with clear GCA features and chest pain. This case report adds to the growing body of evidence that myocarditis is a recognised feature of GCA. A literature review revealed 8 articles and a total of 9 patients with GCA who presented with or developed myocarditis.The initial diagnosis of an NSTEMI was reasonable in this case and the most probable cause of the patient’s symptoms, signs and blood results at the time. To this end, starting dual anti-platelets was justified. His prednisolone was reduced to 40mg due to concerns over accelerated atherosclerosis with high dose glucocorticoids in an already high-risk male with smoking history, hypertension and a stroke history. In retrospect, this was an unnecessary step, but arguably reasonable when weighing the risks and benefits of one treatment against a simultaneous acute illness (NSTEMI).Each subsequent investigation helped to diagnose and exclude certain conditions: the angiogram was necessary to exclude an acute coronary syndrome; cardiac MRI then went on to provide evidence of myocarditis; temporal artery biopsy confirmed GCA and a PET scan excluded any significant large vessel involvement. Interestingly, the first right-sided temporal artery biopsy 3 months prior was negative but the second left-sided biopsy was diagnostic. The BSR states that “contralateral biopsy is usually unnecessary”; however, a few studies have found discordant results when carrying out simultaneous bilateral biopsies with an increased diagnostic yield (Boyev et al., 1999; Breuer et al., 2009 and Durling et al., 2014), suggesting bilateral temporal artery biopsies could lead to less treatment delays and more accurate diagnoses.Key learning pointsMyocarditis is a recognised complication of giant cell arteritis.The key to diagnosis here was a thorough history and an open mind with regards to his background, particularly the bilateral strokes. Investigations initially did not support GCA-associated myocarditis. But the preceding typical GCA symptoms and recent strokes raised the suspicion of a more systemic vasculitis.Working off others’ diagnosis can be misleading. The very recent bilateral posterior circulation strokes and vertebral artery abnormalities were overlooked on admission due to being thoroughly worked up and disregarded as vasculitic by a tertiary stroke centre.Conflicts of interestThe authors have declared no conflicts of interest.