43. Large vessel vasculitis and sarcoidosis: co-existence or one disease?

Abstract Introduction Takayasu arteritis (TA) is a large-vessel vasculitis that preferentially affects the aorta and its major branches, is rare, predominately affects women of child-bearing age and its precise aetiology is unknown. TA causes chronic vascular inflammation. Sarcoidosis, too, is a systemic inflammatory condition which can affect any organ system; the pulmonary system is the most common site. Large-vessel vasculitis is rare in sarcoidosis, but overlap between the two conditions has been reported. It is unclear whether they co-exist or manifest as one disease entity. We report a case of a 50-year-old lady with pulmonary sarcoidosis on a background of TA. Case description A 40-year-old female presented in 2010 with constitutional symptoms, erythema nodosum (confirmed on biopsy), audible murmurs over her carotids and subclavian arteries and raised inflammatory markers (CRP 100). She was diagnosed with Takayasu arteritis following CT angiogram which demonstrated periarterial cuffing and thickening of her carotids, subclavian and thoracic aorta. Her medical history consist of pericarditis in 1992, a thromboembolic event in 1995, ulcerative keratitis in 2006 and incidental aortic regurgitation in 2009. She was treated with oral corticosteroids and started on azathioprine as a steroid sparing agent. Inflammatory markers normalised. Further cardiology assessments confirmed evidence of a dilated ascending aorta in 2015 and she was also diagnosed with corneal ulceration in September 2016. In July 2017, intermittent ankle swelling was reported which was associated with mildly raised inflammatory markers (CRP of 12, ESR of 27). Accentuating murmurs noted and in view of raised inflammatory markers, CT angiogram was repeated; that showed stable appearances of TA. In May 2018, her azathioprine was reduced to 100mg from 125mg as she remained clinically and radiologically stable. In July 2018, she reported recurrence of night sweats and she had marginally raised CRP of 7 and ESR of 8. PET-CT, to look for active TA, demonstrated high uptake on bilateral mediastinal lymph nodes and no evidence of active TA. It was noted retrospectively that mediastinal lymphadenopathy was present on her CT back in 2017. She then underwent endobronchial ultrasound bronchoscopy in August 2018 which showed reactive lymph nodes. Other potential causes were excluded by extensive microbiological and immunology studies. Mediastinoscopy and lymph node excision was arranged as a lymphoproliferative/infective disease needed to be excluded in view of prolonged immunosuppression. Biopsy supported the diagnosis of sarcoidosis showing granulomatous changes. Oral prednisolone 40mg initiated and azathioprine was increased to 125mg. ACE levels remained normal. Discussion This case report emphasises the need for consideration of other systemic conditions in patients with known inflammatory diseases as they can co-exist. Patients who are presented with symptoms that are not fully consistent with a specific phenotype of a disease as in this case the ocular symptoms (corneal ulceration, ulcerative keratitis) and the erythema nodosum, could raise the possibility of a different or co-existent disease. It does also suggest that the prevalence of TA, or related forms of arteritis, may be higher than expected and should be considered, especially in younger patients with non-characteristic cardiovascular symptoms and suspected systemic inﬂammatory disease. Moreover, the association with sarcoidosis in this and other previously described cases suggests that the two diseases may be related, and that TA or TA-like vasculitis may even be a complication of sarcoidosis. Other causes of large vessel vasculitis should be excluded as TB and lymphoproliferative diseases which can also present with lymphadenopathy especially as it is well known that large vessel vasculitis, especially in elderly population, could be part of a para-neoplastic syndrome. Other diseases have been reported associated with TA but rarely sarcoidosis. TA and sarcoidosis may be related as they are characterized by certain nonspeciﬁc immunoinﬂammatory abnormalities. In most case reports sarcoidosis precedes TA diagnosis. In this case, TA was found 9 years before the diagnosis of sarcoidosis was made. Key learning points TA can precede the diagnosis of sarcoidosis. In case of relapsing or refractory TA, further investigations should be considered to exclude other co-existent pathologies as sarcoidosis. TA and sarcoidosis may be related as they are characterized by certain nonspeciﬁc immunoinﬂammatory abnormalities. It has been reported that TA stands as pathology-associated with sarcoidosis. Complete vascular clinical examination should be performed to detect inﬂammatory arteritis, especially in cured sarcoidosis presenting a relapse of the biological inﬂammatory process. Conflicts of interest The authors have declared no conflicts of interest.

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

Rheumatology Advances in Practice ◽

10.1093/rap/rkz028.009 ◽

2019 ◽

Vol 3 (Supplement_1) ◽

Author(s):

Ursula Laverty ◽

Michelle McHenry

Keyword(s):

Disease Activity ◽

Conflicts Of Interest ◽

Temporal Artery ◽

Bleeding Risk ◽

Large Vessel Vasculitis ◽

Large Vessel ◽

Temporal Artery Biopsy ◽

Ct Angiogram ◽

Steroid Sparing ◽

Vessel Involvement

Abstract Introduction This 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 description A 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. Discussion This 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 points This 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 interest The authors have declared no conflicts of interest.

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32. My PET investigation

Rheumatology Advances in Practice ◽

10.1093/rap/rkz028.001 ◽

2019 ◽

Vol 3 (Supplement_1) ◽

Author(s):

Yik Long Man ◽

Manil Subesinghe ◽

Sujith Subesinghe

Keyword(s):

Rheumatoid Arthritis ◽

Pet Imaging ◽

Inflammatory Markers ◽

Conflicts Of Interest ◽

Large Vessel Vasculitis ◽

Large Vessel ◽

Pet Ct ◽

Occult Malignancy ◽

Rule Out

Abstract Introduction Polymyalgia rheumatica (PMR) is closely associated with giant cell arteritis, the most common cause of vasculitis in the elderly population. Diagnostic challenges may arise when patients present with marked systemic upset, raising concerns about a malignancy. Our patient initially presented with marked weight loss, anaemia and significantly raised CRP and ESR, prompting urgent referrals to gastroenterology and haematology to rule out a malignancy. He was eventually referred to the rheumatology team and a PET-CT demonstrated characteristic findings of polymyalgic-onset rheumatoid arthritis. We also explore the expanding role of PET imaging in other rheumatic disorders. Case description A 65-year-old Caucasian gentleman presented to his GP in September 2018 with significant weight loss and general malaise. He had been managing large joint arthralgia by self-medicating with aspirin 300mg daily. Initial investigations by his GP demonstrated a normocytic anaemia, neutropaenia and low iron levels (haemoglobin 103g/L, MCV 86.9fL, neutrophil count 0.5x109/L, iron level 4umol/L). His past medical history was significant of hypercholesterolemia and a right inguinal repair. He had no regular prescribed medications. He worked as an electrical engineer. He had a significant smoking history of 40-pack-years. He was initially referred to the colorectal and haematology teams under the 2-week-wait rule. Video capsule endoscopy, cross-sectional imaging and bone marrow trephine identified no occult malignancy. His ESR was markedly elevated at 133mm/h with a corresponding CRP of 134mg/L. Serum protein electrophoresis was consistent with chronic inflammation. He was subsequently referred to the rheumatology clinic in December 2018 by the haematologists in view of the raised inflammatory markers. At initial review, he reported right knee pain and symmetrical small joint arthralgia of the hands. He also described pain affecting the cervical spine, both shoulders and hips with significant early morning stiffness. Careful questioning revealed no symptoms of a connective tissue disorder. Examination demonstrated marked, symmetrical synovitis over his metacarpophalangeal, proximal interphalangeal and wrist joints. Immunology tests were all negative. Due to ongoing concerns regarding a malignancy a PET-CT scan was requested. This demonstrated widespread FDG-avid symmetrical polyarthropathy in a rheumatoid pattern in addition to enthesopathy in keeping with PMR. There was no evidence of large vessel vasculitis. After 3 months of investigations under multiple specialties, the patient was diagnosed with polymyalgic-onset rheumatoid arthritis. He was started on prednisolone with an excellent clinical response. His CRP improved to 17mg/L. His full blood count also normalised which eventually allowed the introduction of methotrexate. Discussion Rheumatologists are frequently asked to assess patients with unexplained elevated inflammatory markers or multisystem diseases. It is important to remember that constitutional symptoms can be the first presentation of a rheumatic disorder, particularly when an occult malignancy and infection have been ruled out. Although this patient had clinically apparent synovitis on clinical examination, his inflammatory markers were extremely elevated with marked systemic upset. Interestingly, PET-CT demonstrated typical features of PMR which were not apparent from the initial consultation with no evidence of large vessel vasculitis. PET imaging has been used extensively in oncology to identify malignant lesions but it is important to understand its expanding role in rheumatology in identifying active inflammation. It has been used in the diagnosis of large vessel vasculitides, including giant cell arteritis and Takayasu’s arteritis, and IgG4-related disease. Moreover, it is very useful in identifying the extent of the disease which may guide treatment decisions. PET-CT can also be used in the diagnosis and to assess the extent of disease in PMR. Typically, there is FDG uptake in the shoulders, sternoclavicular and hip joints. There is often increased extra-articular uptake between the spinous processes and ischial tuberosities. The use of PET-CT to diagnose PMR is not widespread as the diagnosis of PMR is usually evident based on the history, clinical findings and inflammatory markers. However, when there is marked systemic upset and/or very high inflammatory markers, it is important to consider utilising PET-CT to assess for concurrent occult malignancy or large vessel vasculitis. More recently, PET imaging has also been used in the diagnosis of early rheumatoid arthritis and some studies have shown that it is more sensitive and specific than MRI at picking up subclinical synovitis. Key learning points This case highlights the usefulness of PET-CT as a diagnostic tool in patients who present with systemic illness. The use of PET-CT is two-fold; firstly, to rule out an occult malignancy and secondly, to look for other features that would support an alternative diagnosis. There are characteristic findings of PMR on PET-CT and as rheumatologists, we must be aware of the expanding role of PET-CT in our field so that it can be appropriately applied to our clinical practice. Conflicts of interest The authors have declared no conflicts of interest.

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37. Non-cranial large vessel vasculitis in a 65-year-old woman

Rheumatology Advances in Practice ◽

10.1093/rap/rkz028.006 ◽

2019 ◽

Vol 3 (Supplement_1) ◽

Cited By ~ 1

Author(s):

Shawki El-Ghazali ◽

Maxine Hogarth

Keyword(s):

Takayasu Arteritis ◽

Older Patients ◽

Surgical Intervention ◽

Conflicts Of Interest ◽

Large Vessel Vasculitis ◽

Large Vessel ◽

High Dose ◽

Normal Range ◽

Systemic Symptoms ◽

Steroid Sparing

Abstract Introduction Large-vessel vasculitis in older patients is typically associated with giant cell arteritis (GCA) usually presenting with cranial features. GCA can also present with a primarily extra-cranial pattern as large-vessel GCA (LV-GCA). Takayasu arteritis is a large-vessel vasculitis that affects younger females and classically involves the aorta and its major branches. This condition can clinically manifest with claudication of the extremities, impaired peripheral pulsation and systemic symptoms. Features of Takayasu arteritis and LV-GCA can overlap. This case reviews that of a 65-year-old lady with features of non-cranial large-vessel vasculitis and the approach taken to manage her case. Case description A 65-year-old female developed pains affecting the shoulder and forearms over a period of weeks. She additionally noted pins and needles of the affected limbs. Symptoms were worse with use and consistent with claudication. She denied systemic symptoms, headache, visual changes or early morning stiffness. She was referred to the emergency department for assessment. Blood pressure could not be obtained and radial pulses were absent. She was subsequently arranged for further investigation via the vascular team. CT angiogram of the upper limbs demonstrated diffuse significant subclavian and axillary artery stenoses bilaterally with appearances deemed suggestive of underlying vasculitis. She was referred for rheumatological assessment. CRP was 50 and MRA aorta confirmed findings identified on CT scan. Prednisolone was initiated at a dose of 60mg daily for 4 weeks, and was subsequently decreased at an initial rate of 10mg every 2 weeks. Methotrexate was started at a dose of 15mg weekly. Inflammatory markers were noted to improve and CRP decreased to normal range. Despite this, although her paraesthesia resolved, she reported only a modest improvement of her upper limb claudication symptoms. Blood tests were monitored, however shortly after starting methotrexate, ALT significantly increased to 1002 IU/L. Autoimmune and viral hepatitis screen returned negative, US abdomen was suggestive of fatty liver changes but was otherwise unremarkable. Rise in ALT was attributed to methotrexate use and this was stopped, and levels improved. Mycophenolate was thus introduced as an alternative at a dose of 1g BD, but unfortunately resulted in recurrence of raised ALT. At present, she is being managed with prednisolone alone, of which is slowly being reduced. Her case is being additionally reviewed at our local vasculitis centre based at Hammersmith Hospital for consideration of tocilizumab should there be difficulties on steroid reduction. Discussion Takayasu arteritis is a rare systemic large vessel vasculitis. Incidence is 1-2 per million annually with a female preponderance of 80-90%. Based on ACR classification criteria, the above patient would meet the diagnosis of this condition. Takayasu arteritis would normally be expected for those aged <40 years and has a median onset of 25-30 years. Due to her age, the diagnosis of large vessel vasculitis would be therefore be more consistent with LV- GCA, primarily in the absence of cranial features. Aorta and branch involvement can occur in up to 15% of GCA cases. Histologically, both GCA and Takayasu arteritis share similar findings and cannot be relied upon for diagnostic differentiation. Due to overlap of features, formal diagnostic labelling can be difficult in cases such as the one described. In regards to the management of her case, the general principle was taken of initial high dose prednisolone which has been gradually reduced. Introduction of DMARD therapy was implemented early which would be more typical for Takayasu arteritis rather than GCA. Unfortunately due to deranged ALT attributed initially to methotrexate, and subsequently mycophenolate, she is not currently on any steroid sparing treatment. Studies have demonstrated efficacy of IL-6 inhibition in managing large vessel vasculitis and as mentioned, her case is being reviewed with our local vasculitis centre for consideration of tocilizumab should there be difficulties on prednisolone weaning. Although CRP is now within normal range with treatment, our patient has ongoing claudication symptoms of the upper limbs. It is felt that this is likely due to residual vascular stenotic changes rather than current active vasculitis. As such, following stabilisation of her condition, consideration would be made for vascular surgical intervention in future. Key learning points Diagnosis of Takayasu versus giant cell arteritis can present a diagnostic challenge in some older patients due to overlap of typical features. The underlying process of large vessel vasculitis and shared components in both these conditions suggest they are within the same spectrum of disease. We discussed our management approach of high dose prednisolone which would be typically utilised in either diagnosis. In our case, due to drug induced hepatitis from methotrexate and subsequent mycophenolate, current management is with prednisolone alone. In regards to long term steroid sparing therapy, other options including IL-6 inhibition is being considered pending response to current treatment. Vascular surgical intervention for residual stenoses will also be reviewed following stabilisation of underlying inflammation. Conflicts of interest The authors have declared no conflicts of interest.

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Case Report: Co-existence of sarcoidosis and Takayasu arteritis

Wellcome Open Research ◽

10.12688/wellcomeopenres.15837.2 ◽

2020 ◽

Vol 5 ◽

pp. 73

Author(s):

Jiwan Poudel ◽

Ujjwol Risal ◽

Keshav Raj Sigdel ◽

Buddhi Prasad Paudyal ◽

Sudeep Adhikari ◽

...

Keyword(s):

Case Report ◽

Takayasu Arteritis ◽

Correct Diagnosis ◽

Granulomatous Inflammation ◽

Large Vessel Vasculitis ◽

Large Vessel ◽

Dramatic Improvement ◽

High Resolution Computed Tomography ◽

Ct Angiogram ◽

Bilateral Carotid

Takayasu arteritis is a rare systemic large vessel vasculitis affecting the aorta and its branches. Sarcoidosis, too, is an inflammatory disease. Both entities are granulomatous conditions with a questionable association in their etiopathogenesis. Only a few cases of their coexistence have been reported in the literature. To our knowledge, no such cases have been reported from Nepal. We report a Nepalese woman who presented with non-productive cough, progressive shortness of breath and chest tightness of 3 years duration. She had a history of recurrent bilateral granulomatous uveitis over the previous 3 years. Examination revealed clubbing of digits, absent pulses over the left radial, ulnar and brachial arteries, and a weak pulse over the right arm including the bilateral carotid arteries. Pulmonary function test showed restrictive pattern, a high-resolution computed tomography (HRCT) scan of the chest revealed findings suggestive of pulmonary sarcoidosis. A CT angiogram suggested large vessel vasculitis. Bronchoscopy with biopsy revealed granulomatous inflammation, negative for malignancy and tuberculosis. She was hence, diagnosed with co-existing Takayasu arteritis and sarcoidosis, and treated with Prednisolone 60 mg once daily with dramatic improvement over 4 days and was discharged stable on domiciliary oxygen. She is currently on azathioprine 50 mg, prednisolone 10 mg without the need for supplemental oxygen. This case report highlights the importance of a proper physical examination as a guide to the use of modern technology in making a correct diagnosis. Furthermore, in countries where tuberculosis is endemic, it should always come as the most important differential diagnosis of granulomatous inflammation.

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Diagnosis and Assessment of Disease Activity in Takayasu Arteritis: A Childhood Case Illustrating the Challenge

Case Reports in Rheumatology ◽

10.1155/2014/603171 ◽

2014 ◽

Vol 2014 ◽

pp. 1-6 ◽

Cited By ~ 2

Author(s):

L. Watson ◽

P. Brogan ◽

I. Peart ◽

C. Landes ◽

N. Barnes ◽

...

Keyword(s):

Disease Activity ◽

Takayasu Arteritis ◽

Inflammatory Markers ◽

Fatal Outcome ◽

Large Vessel Vasculitis ◽

Large Vessel ◽

Childhood Onset ◽

The Third ◽

Assessment Of Disease Activity ◽

Active Disease

Takayasu Arteritis (TA) is a rare, debilitating large vessel vasculitis occurring in patients of all ages, including infants, but the disease most commonly presents in the third decade. Diagnosis is often delayed and consequently TA is associated with significant morbidity and mortality. Accurate methods of monitoring disease activity or damage are lacking and currently rely on a combination of clinical features, blood inflammatory markers, and imaging modalities. In this report we describe a case of a 14-year-old boy with childhood-onset TA who, despite extensive negative investigations, did indeed have on-going active large vessel vasculitis with fatal outcome. Postmortem analysis demonstrated more extensive and active disease than originally identified. This report illustrates and discusses the limitations of current modalities for the detection and monitoring of disease activity and damage in large vessel vasculitis. Clinicians must be aware of these limitations and challenges if we are to strive for better outcomes in TA.

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AB1009 THE EFFICACY OF TOCILIZUMAB ON THE TREATMENT OF TAKAYASU ARTERITIS IN CHINESE CHILDREN

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.5778 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 1798.2-1798

Author(s):

C. Wang ◽

H. Song ◽

Z. Yu ◽

M. Quan

Keyword(s):

Takayasu Arteritis ◽

Case Series ◽

Large Vessel Vasculitis ◽

Chinese Children ◽

Large Vessel ◽

Efficacy And Safety ◽

Imaging Data ◽

Eular Recommendations ◽

Interleukin 6 Receptor ◽

Sparing Effect

Background:Takayasu arteritis (TA) is the most prevalent large-vessel vasculitis in children. Patients with TA have a high mobidity and mortality.It remains a therapeutic challenge because corticosteroids monotherapy can rarely cure TAK and the relapse rate is high during GC tapering.Objectives:The aim of this study is to investigate the efficacy and safety of tocilizumab (TCZ)in Chinese children with Takayasu arteritis(TAK).Methods:We retrospectively studied 6 TAK children treated with TCZ in our hospital from July 2017 to October 2018. The demographic and clinical data, laboratory examination results and vascular imaging data were collected.Results:Six pediatric patients with critical or refractory TAK treated with TCZ were analyzed, including 3 males and 3 females.The diagnosis age was ranging in age from 2 to 13 years(median age:7 years).Three patients were initially treated with TCZ and Mycophenolate Mofetil(MMF) as the first-line regimen without corticosteroid or with a quite rapid GC taper duration,two of which had lifte-threatening coronary arteries involved and heart failure.The other three paitients were swcithed to TCZ from conventional disease modifying anti-rheumatic drugs (DMARDs) or other biologics due to being refractory to them and recurrent relapses.Four patients were given TCZ at 4 weeks regular intervals for 10 to 22 months,while two patients withdrew TCZ because of disease deterioration and unbearable abdominal or chest pain after the second dose.After 6 months follow-up,four patients experienced significant clinical and biological improvement with angiographically progression in one patient. A corticosteroid-sparing effect is obvious. Drug-related side effects occur in 1 patients manifesting as a mild elevated liver fuction. Neither neutropenia nor infection was observed.Conclusion:Our study shows a clinical, biological, and radiological response in patients with refractory TAK treated with TCZ.References :[1]Hellmich B, Agueda A, Monti S,et al.2018 Update of the EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis 2019;0:1–12. doi:10.1136/annrheumdis-2019-215672.[2]BravoMancheño B, Perin F, Guez Vázquez Del ReyMDMR, García Sánchez A, Alcázar Romero PP. Successful tocilizumab treatment in a child with refractory Takayasu arteritis.Pediatrics 2012;130(6):e1720-724.[3]Goel R, Danda D, Kumar S, Joseph G. Rapid control of disease activity by tocilizumab in 10 «difficult-to-treat» cases of Takayasu arteritis. Int J Rheum Dis 2013;16(6):754–61.[4]Cañas CA, Cañas F, Izquierdo JH, Echeverri A-F, Mejía M, Bonilla-Abadía F, et al. Efficacy and safety of anti-interleukin 6 receptor monoclonal antibody (tocilizumab) in Colombian patients with Takayasu arteritis. J Clin Rheumatol Pract Rep Rheum Musculoskelet Dis 2014;20(3):125–9.[5]Batu ED, Sönmez HE, Hazirolan T, Özaltin F, Bilginer Y, Özen S. Tocilizumab treatment in childhood Takayasu arteritis: case series of four patients and systematic review of the literature. Semin Arthritis Rheum 2017 Feb;46(4):529–35.Disclosure of Interests:None declared

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P205 The contemporary presentation and management of giant cell arteritis with large vessel vasculitis

Rheumatology ◽

10.1093/rheumatology/keab247.200 ◽

2021 ◽

Vol 60 (Supplement_1) ◽

Author(s):

Owen Cronin ◽

Neil D McKay ◽

Hannah Preston ◽

Helen Harris ◽

Barbara Hauser

Keyword(s):

Giant Cell Arteritis ◽

Giant Cell ◽

Polymyalgia Rheumatica ◽

Large Vessel Vasculitis ◽

Large Vessel ◽

Ct Angiogram ◽

Pet Ct ◽

The Mean ◽

Vessel Involvement

Abstract Background/Aims Giant cell arteritis with large vessel vasculitis (LV-GCA) represents a distinct, less researched sub-category of giant cell arteritis (GCA). In comparison to cranial GCA, the patient’s diagnostic pathway is less well described and it is thought that LV-GCA is underdiagnosed, including in patients with polymyalgia rheumatica and cranial-GCA. Advances in imaging (e.g. PET-CT) and treatment (tocilizumab), have provided additional options in the diagnosis and management of LV-GCA. The aim was to describe the contemporary clinical journey for patients diagnosed with LV-GCA. Methods The electronic patient health record system in NHS Lothian (TrakCare) was used to collect relevant data. Patients with imaging-confirmed large vessel vasculitis, diagnosed with GCA after 1 January 2017 were included. Follow-up was until August 2020. Results Eighteen patients with LV-GCA were included. The mean age was 65 years and 66.7% were female. Two patients had known cranial-GCA but 89% of patients were diagnosed exclusively with large vessel involvement. The most common symptoms were malaise (55%), weight loss (55%), polymyalgia rheumatica (55%) and limb claudication (44%). Pyrexia of unknown origin was a feature in only 17% of patients. Two patients were asymptomatic and were investigated on the basis of raised inflammatory markers. Mean CRP at baseline was 99mg/L and ESR 85mm/hour. The mean time from symptom-onset to diagnosis was 6.8 months (range 1 to 15 months). Sixteen patients (89%) were reviewed by at least one other secondary care specialist. One third of patients were referred from General Medicine followed by Vascular Surgery (16%) and General Practice (16%). 7/18 patients were inpatients at the time of referral. 56% of patients required two modalities of imaging to confirm large vessel involvement. The most commonly used imaging techniques (in descending order) were CT-Chest/Abdomen/Pelvis, CT-angiogram, PET-CT and Vascular Ultrasound. 50% of patients underwent follow-up imaging, most commonly MR- or CT-angiography. Mean follow-up was for 1.6 years. The mean prednisolone dose at 3 months (n = 18) was 24mg daily and 8mg at 12 months (n = 12). 28% of patients relapsed during the follow-up period at 4, 5, 8, 9 and 24 months post-diagnosis. 7/18 patients were commenced on methotrexate for steroid-side effects or for relapse. 8/18 received subcutaneous tocilizumab in combination with methotrexate in two cases. Three patients were started on azathioprine but only one continued. Conclusion In modern-day clinical practice, patients with LV-GCA experience a longer time to diagnosis than those with cranial symptoms. Patients with LV-GCA can experience an array of constitutional symptoms. Frequently, more than one imaging modality is required to confirm LV-GCA and the majority of patients will have seen other hospital specialists or have been admitted to hospital before diagnosis. Methotrexate and tocilizumab are the most frequently-used and effective steroid-adjunct in this single-centre cohort. Disclosure O. Cronin: None. N.D. McKay: Consultancies; Gilead. Other; Has received support for conference attendance from Pfizer and Gilead, Has received educational support from UCB, Gilead, Celgene, Biogen, Sanofi, Abbvie, Novartis, Pfizer. H. Preston: None. H. Harris: None. B. Hauser: None.

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Reducing the Toxicity of Long-Term Glucocorticoid Treatment in Large Vessel Vasculitis

Current Rheumatology Reports ◽

10.1007/s11926-020-00961-0 ◽

2020 ◽

Vol 22 (12) ◽

Author(s):

Andriko Palmowski ◽

Frank Buttgereit

Keyword(s):

Adverse Events ◽

Takayasu Arteritis ◽

Term Treatment ◽

Large Vessel Vasculitis ◽

Large Vessel ◽

Serious Adverse Events ◽

Glucocorticoid Treatment ◽

Long Term Treatment ◽

Higher Doses

Abstract Purpose While glucocorticoids (GCs) are effective in large vessel vasculitis (LVV), they may cause serious adverse events (AEs), especially if taken for longer durations and at higher doses. Unfortunately, patients suffering from LVV often need long-term treatment with GCs; therefore, toxicity needs to be expected and countered. Recent Findings GCs remain the mainstay of therapy for both giant cell arteritis and Takayasu arteritis. In order to minimize their toxicity, the following strategies should be considered: GC tapering, administration of conventional synthetic (e.g., methotrexate) or biologic (e.g., tocilizumab) GC-sparing agents, as well as monitoring, prophylaxis, and treatment of GC-related AEs. Several drugs are currently under investigation to expand the armamentarium for the treatment of LVV. Summary GC treatment in LVV is effective but associated with toxicity. Strategies to minimize this toxicity should be applied when treating patients suffering from LVV.

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