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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.

Introduction: This case report describes the delay in diagnosis and potentially life-threatening complications of a patient with large vessel vasculitis. The initial diagnosis was of vascular Ehlers-Danlos Syndrome, based on family history and imaging. However, recurrent episodes of chest pain and a rapidly expanding aortic aneurysm prompted further diagnostics which led to the diagnosis of giant cell arteritis with large vessel vasculitis. The patient required thoracic surgery due to expansion of the aortic aneurysm. This case demonstrates the risk of delayingthe diagnosis of large vessel vasculitis and the complexities of immunosuppression in the context of major surgery. Case description: A previously fit and well 69-year-old man presented with constitutional symptoms and intermittent chest pain for six months. Two months after symptom onset, he was admitted with chest tightness and shortness of breath. He had raised inflammatory markers with thrombocytosis and was given antibiotics fora probable chestinfection. A CT pulmonary angiogram revealed ascending aorta dilatation, 47mm with wall thickening. The radiologist reported this as being most likely due to systemic hypertension. There was no evidence of malignancy on abdominalandpelvic imaging.Theaorticchangeswereattributedtopossible underlying Ehlers-Danlos syndrome, as his daughter had a diagnosis of Ehlers-Danlos Type III. He was discharged despite persistently raised inflammatory markers and no positive microbiology. He was readmitted after six weeks with pyrexia and severe chest pain. A CT of his thoracic aorta showed further expansion of the aortic aneurysm, 59mm with a slightly thickened abdominal aorta wall. With a rapidly expanding aortic aneurysm and persistent raised inflammatory markers, the suspicion of large vessel vasculitis was raised. No symptoms of cranial temporal arteritis or polymyalgia rheumatica. A FDG-PET scan showed high uptake in the ascending, thoracic and upper abdominal aorta in keeping with extra-cranial large vessel vasculitis. He was treated with IV methylprednisolone for three days with subsequent oral prednisolone. Whilst on high dose glucocorticoids, he developed steroid induced diabetes and hypomania hence oral methotrexate 15 mg once weekly was added. The cardiothoracic team elected to delay cardiothoracic surgery until he was on less than 15 mg of oral prednisolone daily. However, reduction of oral prednisolone led to a flare of giant cell arteritis. The patient had to undergo elective aortic valve and ascending aorta replacement whilst on 40 mg of prednisolone. Tocilizumab was added two months after surgery and prednisolone was successfully reduced to 3mg. Discussion: Critically, appropriate treatment was delayed due to misdiagnosis of Ehlers-Danlos Syndrome; this was largely based on the family history. This red herring likely distracted clinicians from seeking a better explanation forthe patient's presentationand aortic changes. The initialoral steroidcourse wascomplicatedbysteroid induced complications and pending cardiothoracic surgery. The surgical team planned to delay surgery until the patient is on a lower prednisolone dose, due to the increased risk of infection and poor wound healing. The patient therefore had a rapid steroid tapering regime, reducing by 5mg two-weekly, from a starting oral dose of 65mg. Such rapid steroid tapering carries a risk of disease flare, and in fact two weeks prior to surgery, his inflammatory markers rose, and his prednisolone dose had to be increased from 25mg to 40mg. A collaborative decision between the rheumatologists and surgeons was then made to proceed with surgery despite the high-dose steroids, as simultaneous inflammation control and prompt surgical repair took precedence over the potentially increased post-operative risks. The patient also experienced steroid-related side-effects, but the switch to steroid-sparing agents was challenging. Starting tocilizumab as a further immunosuppressant had to be delayed until after surgery, as IL-6 inhibition would distort C-reactive protein as a measure of both disease activity and potential post-operative infection.
In summary, this case highlights the difficulty in diagnosing large vessel vasculitis and the potential delay in treatment can cause significant morbidity and mortality. Appropriateimaging is crucial toascertainadiagnosisof large vesselvasculitis. Immunosuppression must be tailored individually, accounting for side-effectsand competingrisks. Points for discussion include the use of newer imaging modalities in diagnosing large vessel vasculitis and monitoring disease activity. The efficacy and safety of tocilizumab as a steroid-sparing agent for this condition should also be investigated. Key learning points: The importance of early diagnosis and treatment in patients with large vesselvasculitis, as vascular complicationscan arise if treatment is delayed. The use of appropriate imaging modalities such as MR angiogram and FDG-PET is crucial fordiagnosis of large vesselvasculitis. Glucocorticoid therapy is the mainstay treatment for large vessel vasculitis, but side-effects can be limiting. Steroid sparing agents, such as IL-6 inhibitors should be considered early as an alternative immunosuppressant for patients with large vessel vasculitis. Careful planning of immunosuppression is required prior to major surgery.

Conflicts of interest:
The authors have declared no conflicts of interest.

LARGE VESSEL VASCULITIS AND SARCOIDOSIS: CO-EXISTENCE OR ONE DISEASE?
Faidra Laskou 1 , Philip Sajik 1 , and Leena Yalakki 1 1 Rheumatology, Basingstoke and North Hampshire, Basingstoke, United Kingdom 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 backgroundof 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 thickeningofher carotids, subclavian andthoracicaorta. Her medical history consist of pericarditis in 1992, a thromboembolic event in 1995, ulcerativekeratitis in2006 and incidentalaortic regurgitation in2009. She was treated with oral corticosteroids and started on azathioprine as a steroid sparing agent.Inflammatorymarkersnormalised. Furthercardiology 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 inview 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 herCT back in2017. She then underwentendobronchial 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 erythemanodosum, could raise the possibility of adifferent orco-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 acomplicationof 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 partof apara-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 beforethe 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 consideredto excludeother 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 relapseof the biological inflammatory process.

Conflicts of interest:
The authors have declared no conflicts of interest.

BUERGER'S DISEASE WITH SEVERE EOSINOPHILIA AND ACUTE CRITICAL ISCHEMIA
Mansoor ul haq Keen 1 , Shouvik Dass 1 , and Aamir Aslam 1 1 Rheumatology Department, Leeds Teaching Hospitals, Leeds, United Kingdom Introduction: Buerger's disease usually presents with normal blood tests. We present a case with significant eosinophilia, a phenomenon rarely reported. Eosinophilia with acute onset Raynaud's and critical ischemia canbeatypical early manifestationof Buerger's disease (thromboangiitisobliterans). Case description: A case report of a 24-year-old male, smoker, with no other history, presented acutely, complaining of cool, painful hands with tricolour changes with worsening for 48 hours. On examination, apart from features of Raynaud's and tender fingers, he had good perfusion, as shown by Doppler testing. Blood tests showed eosinophilia; 13x10*9 (Normal 0.04-0.40 x10*9) and mild raised C-reactive protein 24 (normal range <10.0 mg/L). Chest radiograph, ECG, and echocardiography showed no abnormalities. The results of initial screening including Immunoglobulins, complements, ANA, ANCA, RF, APLA and CCP were all negative. As there were no critical features of ischemia, we considered the patient for discharge from the hospital with a plan for close review. We strongly advised him not to smoke. He re-admitted within a week with worsening of Raynaud's and features of critical ischemia with bluish nail and fingertip and some early features of mild digital necrosis. We began treatment with intravenous heparin, aspirin, and iloprost (prostacyclin) infusion for10 days. The patient'sdigital ischemiaand intermittent claudication showed some improvement after 1 week, but eosinophilia persisted at 11x10*9 with ruminant pain in digits. The importance of smoking cessation was re-emphasised. Magnetic resonance angiography showed no occlusion of the distal arteries of the upper and lower extremities. Capillaroscopy showed micro haemorrhages (no connective tissue disease/scleroderma pattern abnormality). Investigations for parasitic infections and malignancy were negative. HIV / Hepatitis B/C CMV/EBV/ Parvovirus IGM was all undetected. Schistosoma testing was negative. Haematology investigated him for the rare possibility of the genetic mutation for hypereosinophilic syndrome (PDGFRA and FIP1L1 genes) which was negative and underwent bone marrow biopsy which was also normal. We started him on oral prednisolone 1 mg/kg body weight with complete resolution of eosinophilia and ischemic symptoms plus Raynaud's. Smoking stopped with help. Discussion: Buerger's disease is a rare disease of exclusion and recognised as one form of vasculitis with a strong association with smoking and nicotine. There has been no consensus or any significant data for any specific therapies proven a benefit in this condition, more than in alone stopping smoking oreven oral tobacco products.The main characteristic features of this form are present in young smokers with no systemic disease manifestation, normal inflammatory response, and negative immunology.
Eosinophilia has only rarely been a feature of the presentation of Buerger's disease. Its presence does not imply a systemic vasculitis or a related syndrome. Although there is limited evidence for the use of steroids in this condition, inflammatory features such as eosinophilia could strengthen the rationale for using steroids in certain cases by halting the ongoing immune driven inflammatory cascade. The presence of eosinophilia might suggest alternative pathogenic mechanisms in certain cases of Buerger's. Key learning points: To recognise eosinophilia as a marker in atypical Buerger's disease presentation. Being a disease of exclusion we have to rule out all other possibilities including some rare ones as was done in this case by seeking advice from infectious disease and haematology team. Introduction: An 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 dissectionof the proximal right common iliacartery.The intermittentclaudication progressed to acute limb ischaemia, which responded well to conservative treatment withheparin. Case description: Headache was unilateral, intermittent and lasted a few minutes only, although it was described as severe. There were no visual symptoms, noscalp tendernessand nojaw ortongue claudication. His mobility was severely impacted by intermittent claudication. He was previously playing 3 rounds of golf per week but exercise tolerance reduced tofifty metres.Therewerenospecificriskfactorsforatherosclerotic disease. He was a retired head teacher and had never smoked. Alcohol intake was 3 units per week.He was not taking anymedication. 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-specificinflammation 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 commoniliac 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. Discussion: Whilst 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 i40 27 September 2019 POSTER PRESENTATIONS