Large Vessel Vasculitis Research Articles

Can a Giant Cell Arteritis (GCA) Risk Stratification Score Be Helpful in Clinical Practice?

Giant cell arteritis (GCA) is the most common type of large vessel vasculitis.The diagnosis of GCA is often challenging and there is a difficult balance of over- and underinvestigation. There have been several proposed scoring systems to help clinicians risk stratify patients who may present with suspected GCA. A retrospective cohort study was performed using electronic medical records of patients referred for a temporal artery biopsy (TAB) and temporal artery ultrasound scan (USS) for suspected GCA. All TABs performed at the Royal Wolverhampton NHS Trust between June 2014 and June 2018 and all USS procedures performed between January 2015 and January 2019 were analysed. Patients who undergo a USS for suspected GCA at our centre routinely have scanned bilateral temporal and axillary arteries. 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. The total number of patients who underwent a confirmatory diagnostic test (either TAB or USS) for suspected GCA was 187. Thirteen of these patients met the exclusion criteria, the remaining 174 patients were included for analysis. A total of 126 of 174 patients underwent a TAB and 63 of 174 had aUSS performed; 15 of 174 who had both these were included in the USS cohort because for all these patients, the ultrasound was the first diagnostic test performed. Our results appear to closely mirror the original multi-centre results with regardto the prediction of biopsy-positive GCA, with the centiles closely following those in the inception cohort. Also, 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, as two had a positive ultrasound. Our studyhighlights that a probability score for GCA derived from a large multi-centre cohort of patients who were biopsy positivepredicts ultrasound positivity with similar accuracy. Our work reveals that scoring systems are not infallible but can be helpful in guiding clinical decision making.

Semi-Quantitative [18F]FDG-PET/CT ROC-Analysis-Based Cut-Offs for Aortitis Definition in Giant Cell Arteritis.

[18F]fluorodeoxyglucose-positron emission tomography/computed tomography ([18F]FDG-PET/CT) is used to diagnose large vessel vasculitis in giant cell arteritis (GCA). We aimed to define a semi-quantitative threshold for identifying GCA aortitis from aortic atheroma or the control. Contrast enhanced computed tomography (CECT) was used as the reference imaging for aortic evaluation and to define aortitis, aortic atheroma and control aortas. [18F]FDG-PET/CT was performed on 35 GCA patients and in two different control groups (aortic atheroma (n = 70) and normal control (n = 35)). Aortic semi-quantitative features were compared between the three groups. GCA patients without aortitis on CECT were excluded. Of the GCA patients, 19 (54.3%) were not on glucocorticoids (GC) prior to [18F]FDG-PET/CT. The SUVmax, TBRblood and TBRliver aortic values were significantly higher in the GCA aortitis group than in the aortic atheroma and control groups (p < 0.001). Receiver operating characteristic curve analyses brought to light quantitative cut-off values allowing GCA aortitis diagnosis with optimal sensitivity and specificity versus control or aortic atheroma patients for each PET-based feature analyzed. Considering the overall aorta, a SUVmax threshold of 3.25 and a TBRblood threshold of 1.75 had a specificity of 83% and 75%, respectively, a sensitivity of 81% and 81%, respectively, and the area under the ROC curve (AUC) was 0.86 and 0.83, respectively, for aortitis detection compared to control groups in GCA cases with GC. A SUVmax threshold of 3.45 and a TBRblood threshold of 1.97 had a specificity of 90% and 93%, respectively, a sensitivity of 89% and 89%, respectively, with an AUC of 0.89 and 0.96, respectively, for aortitis detection compared to the control in GC-free GCA cases. Discriminative thresholds of SUVmax and TBRblood for the diagnosis of GCA aortitis were established using CECT as the reference imaging.

Giant Cell Arteritis of the Superior Mesenteric Artery Presenting With Wernicke Encephalopathy From Thiamine Deficiency

Objective N/A. Background Giant cell arteritis (GCA) is one of the most common systemic vasculitides in adults over the age of 50 with incidence ranging from 15 to 35 per 100,000 individuals. The disorder is often included in the differential diagnosis of maladies producing atypical facial pain, headache, visual loss, amaurosis fugax, jaw pain, elevated inflammatory markers, and anemia. GCA is typically known to affect cranial arteries with physical exam findings of tenderness to palpation of the temporal arteries and cranial neuropathies. Clinical diagnosis is further supported by new headache, temporal artery abnormality, elevated ESR (= 50 mm/h), and abnormal artery biopsy. Design/Methods N/A. Results A 68-year-old female with history of primary generalized seizures presented to clinic with a 6-week history of paroxysms of acute confusional episodes, the inability to arise from a seated position due to lower extremity weakness bilaterally, alterations of consciousness without loss of consciousness, severe anorexia, and weight loss. MRI with contrast including Axial FLAIR/T2/Diffusion revealed bilateral pan-lobar cortical and subcortical atrophy with ex-vacuo ventriculomegaly and mild leukoaraiosis in the subcortical white matter tracts. PET-CT body revealed linear uptake involving the aortic root, extending into subclavian arteries bilaterally with segmental involvement of proximal common carotids, and extending inferiorly to the level of the common iliac arteries and the mesenteric arteries. Temporal artery biopsy revealed presence of granulomas with multinucleated giant cells. Serology panel revealed pan hypovitaminoses in Vitamins A, B1, B6, B12, and D. Conclusions Traditional GCA workup initially resulted inconclusive for the patient, whose condition deteriorated as the patient's altered mental status and dizziness spells continued unremittingly. This case highlights the link between large vessel vasculitis and malabsorption syndromes, with the involvement of the superior mesenteric artery, a medium sized vessel, in GCA previously unrecognized. Furthermore, this case is a superb example of multiple etiologies of treatable causes of reversible dementia.

Takayasu Arteritis is Associated with Impaired Arterial Stiffness: A Meta-Analysis of Observational Studies.

Takayasu arteritis, a large vessel vasculitis, is strongly associated with increased risk for cardiovascular and chronic kidney disease. Arterial stiffness represents an established prognostic marker of cardiovascular disease development in the general population. A few studies have assessed the effect of Takayasu arteritis on arterial stiffness indices. Herein, we sought to provide pooled effect estimates regarding the impact of Takayasu arteritis on arterial stiffness, by retrieving relevant, available observational studies. On 1st May, 2022, we searched two major electronic databases and grey literature sources for relevant observational studies. We set as primary outcome the mean difference in carotid femoral PWV (cfPWV) between patients with Takayasu arteritis compared to controls. Regarding the primary outcome, we pooled data from 3 studies in a total of 125 enrolled subjects, demonstrating that Takayasu arteritis is associated with a significant increase in cfPWV by 2.06 m/s (MD = 2.06, 95% CI; 1.29 - 2.83, I2 = 0%, p < 0.001), compared to controls. The present preliminary meta-analysis demonstrates the potential deleterious effects of Takayasu arteritis on arterial stiffness. Prognostic implications must be confirmed in larger, prospective studies.

Influence of the EULAR recommendations for the use of imaging in large vessel vasculitis in the diagnosis of giant cell arteritis: results of the ARTESER register

ObjectiveThe main study objective was to determine how giant cell arteritis (GCA) is diagnosed in our clinical practice and whether the EULAR recommendations have influenced the diagnostic procedures used.MethodsARTEritis of...

Takayasu's Arteritis: A Case Report.

aortitis syndrome; arteritis; case reports; pulseless disease; young female arteritis.

The neutrophil: A key resourceful agent in immune-mediated vasculitis.

The term "vasculitis" refers to a group of rare immune-mediated diseases characterized by the dysregulated immune system attacking blood vessels located in any organ of the body, including the skin, lungs, and kidneys. Vasculitides are classified according to the size of the vessel that is affected. Although this observation is not specific to small-, medium-, or large-vessel vasculitides, patients show a high circulating neutrophil-to-lymphocyte ratio, suggesting the direct or indirect involvement of neutrophils in these diseases. As first responders to infection or inflammation, neutrophils release cytotoxic mediators, including reactive oxygen species, proteases, and neutrophil extracellular traps. If not controlled, this dangerous arsenal can injure the vascular system, which acts as the main transport route for neutrophils, thereby amplifying the initial inflammatory stimulus and the recruitment of immune cells. This review highlights the ability of neutrophils to "set the tone" for immune cells and other cells in the vessel wall. Considering both their long-established and newly described roles, we extend their functions far beyond their direct host-damaging potential. We also review the roles of neutrophils in various types of primary vasculitis, including immune complex vasculitis, anti-neutrophil cytoplasmic antibody-associated vasculitis, polyarteritis nodosa, Kawasaki disease, giant cell arteritis, Takayasu arteritis, and Behçet's disease.

Abstract 12089: Right Coronary Artery Aneurysm in Takayasu Arteritis

Background: Takayasu arteritis (TA) is a large vessel vasculitis that affects the aorta and its primary branches. The coronary arteries are uncommonly involved. Case Presentation: A 52-year-old man, with a history of TA not on immune suppressants due to recurrent active miliary tuberculosis, was scheduled for open abdominal repair of a multilobulated saccular thoracoabdominal aneurysm. Cardiology was consulted for pre-operative evaluation. His physical exam and EKG were unremarkable. Imaging of his coronaries with CT angiography showed proximal and distal right coronary artery dilation (14 mm) and a intraluminal thrombus. Revascularization surgically or percutaneously was not advised. Additional vascular interventions were postponed until the patient received adequate treatment for his tuberculosis and uncontrolled TA. The patient was started on ASA and warfarin for the intraluminal thrombus and coronary aneurysm. Discussion: Coronary artery aneurysms are rare in TA. When coronaries are involved, ostial stenosis or occlusion is the most common lesion. Invasive coronary angiography is difficult due to diffuse vascular disease and involvement of the aorta. The imaging modality of choice remains a debate. CT angiograms remain a reasonable option for the assessment of coronary involvement. There is no established guideline for the treatment of coronary thrombus in patients with TA. Because coronary aneurysms promote thrombus formation by stasis and abnormal flow conditions, it is reasonable to treat with ASA combined with warfarin as they do in Kawasaki disease. Conclusion: Although rare in TA, coronary aneurysm formation is possible. A coronary angiogram is technically difficult in this group of patients, and CT angiograms remains a safe option to define coronary anatomy. The treatment guidelines are lacking, and ASA combined with warfarin is a reasonable option to reduce myocardial infarction and death.

2022 American College of Rheumatology/EULAR Classification Criteria for Takayasu Arteritis.

To develop and validate new classification criteria for Takayasu arteritis (TAK). Patients with vasculitis or comparator diseases were recruited into an international cohort. The study proceeded in 6 phases: 1) identification of candidate criteria items, 2) collection of candidate items present at diagnosis, 3) expert panel review of cases, 4) data-driven reduction of candidate items, 5) derivation of a points-based classification score in a development data set, and 6) validation in an independent data set. The development data set consisted of 316 cases of TAK and 323 comparators. The validation data set consisted of an additional 146 cases of TAK and 127 comparators. Age ≤60 years at diagnosis and imaging evidence of large-vessel vasculitis were absolute requirements to classify a patient as having TAK. The final criteria items and weights were as follows: female sex (+1), angina (+2), limb claudication (+2), arterial bruit (+2), reduced upper extremity pulse (+2), reduced pulse or tenderness of a carotid artery (+2), blood pressure difference between arms of ≥20 mm Hg (+1), number of affected arterial territories (+1 to +3), paired artery involvement (+1), and abdominal aorta plus renal or mesenteric involvement (+3). A patient could be classified as having TAK with a cumulative score of ≥5 points. When these criteria were tested in the validation data set, the model area under the curve was 0.97 (95% confidence interval [95% CI] 0.94-0.99) with a sensitivity of 93.8% (95% CI 88.6-97.1%) and specificity of 99.2% (95% CI 96.7-100.0%). The 2022 American College of Rheumatology/EULAR classification criteria for TAK are now validated for use in research.

18F]FDG PET/CT in Large Vessel Vasculitis: The Impact of Expertise and Confounders on Image Analysis.

Diagnosis of vasculitis is challenging. To avoid invasive approaches, clinical guidelines recommend the use of diagnostic imaging. This study aimed at evaluating the diagnostic accuracy of [18F]-fluorodeoxyglucose ([18F]FDG) position emission tomography/computed tomography (PET/CT) in large vessel vasculitis (LVV) and how this is affected by inter-operator variability. A total of 279 patients who performed [18F]-FDG PET/CT for suspicion of LVV were retrospectively analyzed. We tested the qualitative and semi-quantitative analysis and parameters influencing image quality and interpretation. Exams were evaluated by two readers with different experience and their performance was compared. LVV diagnosis was confirmed in 81 patients. [18F]-FDG PET/CT accuracy was 73% and 67% for the expert reader and less experienced reader, respectively. The expert reader overall performed better than the less experienced one, with higher accuracy in patients with normal BMI (77.3 vs. 63.8%), normal level of glycemia (73.3 vs. 65%), younger age (76.6 vs. 68.2%), and when no therapy was in course at time of imaging (76.7 vs. 66.7%). The diagnostic performance of both readers did not improve using semi-quantitative parameters. We confirmed the appropriateness of the recommended criteria for image acquisition and interpretation, underlining the importance of experience in image interpretation for the optimal diagnostic performance of [18F]FDG PET/CT in vasculitis.

Chronic disseminated intravascular coagulation in a patient with Takayasu arteritis.

Takayasu arteritis (TA) is a large-vessel vasculitis that predominantly affects the aorta and although it usually causes stenosis, aneurysms have been reported in ∼10-25% of the patients. Here, we present a male TA patient with chronic disseminated intravascular coagulation (DIC) as a rare complication of endovascular grafted aortic aneurysm (AA). Chronic DIC usually presents as mild or even hidden clinical symptoms, and early diagnosis of this condition can only be possible with laboratory tests. Due to the silent clinical scenario, this complication should be kept in mind among AA patients with unexplained thrombocytopenia or coagulation abnormalities.

Tocilizumab for relapsing and remitting giant cell arteritis: a case series

BackgroundGiant cell arteritis is a large vessel vasculitis of the arteries in the head and neck. The mainstay of management is with high-dose corticosteroids, and patients often face difficulties stopping or reducing steroids without recurrence of symptoms. Corticosteroids are well established to have numerous associated side effects, including osteoporosis, weight gain, and diabetes. Therefore, when tocilizumab was approved for up to 1 year for cases of relapsing or refractory giant cell arteritis by the National Institute of Health and Care Excellence (NICE) in April 2018, this offered an opportunity to benefit from new funding and to reduce steroid burden.Case presentationThis case series describes the impact of the establishment of a new hub and spoke referral pathway for the use of tocilizumab in refractory or relapsing giant cell arteritis, with case examples from consecutive patients who accessed the funding between August 2018 and April 2021. A total of 16 patients were identified: 11 female and 5 male, with an average age of 72.4 (range 61–82) years, with a majority of 11 ethnically white. The central assessing hub is St George’s University Hospitals NHS Foundation Trust Hospital, serving a population of 1.3 million in the south of England. This is the first large case series looking into the impact of the establishment of a regional clinical pathway for the new tocilizumab funding.ConclusionsThe case series demonstrates that the use of tocilizumab has reduced both the duration and the dose of corticosteroids in these 16 cases (mean prednisolone reduction 20.4 mg: 95% CI 13.0–27.8 mg), with 50% of patients continuing on tocilizumab after the initial 12 month funding period. The disease course, patterns of response, and maintenance of remission are discussed, and we describe the benefits of replicating this hub and spoke tocilizumab pathway in other centers.

Outcome Measures and Biomarkers for Disease Assessment in Takayasu Arteritis.

Takayasu arteritis (TAK) is a less common large vessel vasculitis where histopathology of involved arteries is difficult to access except during open surgical procedures. Assessment of disease activity in TAK, therefore, relies on surrogate measures. Clinical disease activity measures such as the National Institutes of Health (NIH) score, the Disease Extent Index in TAK (DEI.TAK) and the Indian TAK Clinical Activity Score (ITAS2010) inconsistently associate with acute phase reactants (APRs). Computerized tomographic angiography (CTA), magnetic resonance angiography (MRA), or color Doppler Ultrasound (CDUS) enables anatomical characterization of stenosis, dilatation, and vessel wall characteristics. Vascular wall uptake of 18-fluorodeoxyglucose or other ligands using positron emission tomography computerized tomography (PET-CT) helps assess metabolic activity, which reflects disease activity well in a subset of TAK with normal APRs. Angiographic scoring systems to quantitate the extent of vascular involvement in TAK have been developed recently. Erythrocyte sedimentation rate and C-reactive protein have a moderate performance in distinguishing active TAK. Numerous novel biomarkers are under evaluation in TAK. Limited literature suggests a better assessment of active disease by combining APRs, PET-CT, and circulating biomarkers. Validated damage indices and patient-reported outcome measures specific to TAK are lacking. Few biomarkers have been evaluated to reflect vascular damage in TAK and constitute important research agenda.

Acute heart failure due to left common iliac arteriovenous fistula: A case of VEXAS syndrome.

We describe the case of a 78-year-old man presenting with multiple oedematous erythemas, fever, and arthralgia who subsequently developed neutrophil infiltration into the cartilage of the bilateral auricularis, consistent with relapsing polychondritis. A skin biopsy of the erythema on his right arm showed dense neutrophilic infiltration into the dermis, while a bone marrow aspirate revealed myelodysplastic syndromes with characteristic vacuoles in myeloid precursor cells. Although the patient achieved remission with high-dose oral prednisolone, the inflammatory symptoms relapsed, and he was resistant to colchicine and cyclosporine. The patient spontaneously developed left leg oedema and high-output cardiac failure caused by an arteriovenous fistula with a common iliac artery aneurysm. We successfully performed a two-stage surgery using internal iliac artery coil embolisation and endovascular aortic repair of the iliac aneurysm. We assumed the patient was suffering from large-vessel vasculitis such as giant cell arteritis or Takayasu's arteritis. We treated him with tocilizumab in addition to prednisolone, and the febrile events and elevated C-reactive protein levels improved. One year later, sequencing of ubiquitylation-initiating E1 enzyme using peripheral blood leucocytes revealed somatic variants (c.121A>C p.Met41Leu), confirming the diagnosis of vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome. This case suggests that arteriovenous fistula could be a complication of VEXAS syndrome with large-vessel vasculitis, and adequate surgical intervention and prompt diagnosis are essential for rescue. Although arteriovenous fistula is a rare complication of VEXAS syndrome, physicians should be aware of this complication to ensure prompt diagnosis and timely surgical intervention.

69 Large vessel vasculitis occurring after COVID-19 infection: 2 Takayasu cases

BackgroundTakayasu arteritis (TA) is a rare, chronic, granulomatous large vessel vasculitis that affects the aorta, its main branches, and the pulmonary artery. Although the aetiology of TA is not known entirely, it is thought to be multifactorial. To date, cases have been reported after Mycobacterium tuberculosis, human immunodeficiency virus (HIV), and influenza infection. Cases of postinfectious vasculitis have been reported in the literature during the COVID-19 pandemic. Here, we present two patients diagnosed with TA after COVID-19 infection.Case 1A thirteen-year-old female patient was admitted to the pediatric emergency department of our hospital with chest pain and syncope. His uncle had a COVID-19 infection 6 months ago and they stayed in the same house during the infection.On physical examination, respiratory sounds were less audible in the left lung baseline. The blood pressure was measured 128/71 mmHg in the right arm, 122/72 mmHg in the left arm, 106/67 mmHg in the right leg, and 107/74 mmHg in the left leg. Peripheral pulses were weaker in the lower extremities than in the upper extremities.High acute phase reactants and anaemia were detected in the laboratory. (Table 1) Anti-ds DNA was positive, and anti-neutrophil cytoplasmic antibody (cANCA) was negative. Infection screening was negative.In thorax and abdomen computed tomography (CT), 20 mm pericardium and 10 mm pleural effusion and mural thickening along the aorta of ∼20–25 cm from the descending thoracic aorta to the suprarenal level in the proximal 2 cm of the left subclavian artery and mild narrowing of the celiac and superior mesenteric artery orifices were detected. The patient was diagnosed with TA. She was treated with intravenous 30 mg/kg/day methylprednisolone for 3 days. The patient was discharged with oral prednisolone, methotrexate, and anti-TNF therapy.Case 2A 17-year-old female patient was admitted to our center with complaints of back pain for 2 months, dry cough, weight loss (3 kg in 2 months), and fever for 2 days. It was learned that the patient had a COVID-19 infection 5 months ago.On physical examination, blood pressure was measured 94/59 mmHg in the left arm, 103/60 mmHg in the right arm, 125/77 mmHg in the left leg, and 132/80 mmHg in the right leg. Peripheral pulses were weaker in the lower extremities compared with the upper ones. There was a 1/6 systolic murmur in the aortic focus. Other system examination was normal.Laboratory tests revealed elevated acute phase reactants, thrombocytosis, and anaemia. (Table 1) Positron emission tomography-CT (PET-CT) revealed increased FDG uptake in both common carotid, aortic arch, ascending and descending aortic walls. In MR angiography, localized enlargement and stenosis of the aortic arch, wide ascending aorta, stenosis in the left subclavian artery, and the origin level of the left renal artery, and then a slight enlargement was observed. Based on the present findings, the patient was diagnosed with TA. Infection screening was negative. The patient was given 30 mg/kg/day intravenous methylprednisolone for 3 days and continued with oral prednisolone, methotrexate, and etanercept.DiscussionTimely diagnosis and treatment in TA is very important in preventing irreversible vascular damage resulting in ischaemia of vital organs. It should be kept in mind that TA and other vasculitides may occur after COVID-19 infection.

Somatostatin receptor PET/MR imaging of large vessel inflammation in active compared with inactive vasculitis and atherosclerosis

Abstract Background Use of 18F-FDG PET in large vessel vasculitis (LVV) is limited by non-specific uptake due to arterial remodelling and/or atherosclerosis leading to diagnostic uncertainty. Purpose To investigate somatostatin receptor 2 (SST2) as a novel inflammation-specific PET imaging target in LVV. Methods In a prospective observational cohort study, we tested the ability of PET/MRI using two somatostatin receptor tracers (68Ga-DOTATATE and 18F-FET-βAG-TOCA) to differentiate active from inactive LVV, and aortic atherosclerosis in patients with recent myocardial infarction. Ex vivo mapping of the imaging target was performed using immunofluorescence microscopy, imaging mass cytometry, and bulk, single-cell and single-nuclei RNA sequencing of temporal artery biopsies from LVV patients. Results Sixty-one participants were included (LVV, n=27; myocardial infarction ≤2 weeks, n=25; control subjects with an oncological indication for imaging, n=9). LVV patients (mean age 58 [SD 16] years; 78% female; 63% active or grumbling disease) had giant cell arteritis (n=13), Takayasu arteritis (n=13), or unspecified LVV (n=1). Baseline index vessel SST2 PET maximum tissue-to-blood ratio (TBRmax) was 61.8% (95% CI 31.5–99.0%, p&amp;lt;0.0001) higher in patients with active/grumbling LVV than inactive LVV, and 34.6% (95% CI 15.1–57.6%, p=0.0002) higher than recent myocardial infarction (Fig. 1a–c; arrow: PET signal; arrowhead: aortic thickening; asterisk: aortic atherosclerosis), with good diagnostic accuracy (AUC ≥0.86, p&amp;lt;0.001 for both). None of the control subjects without LVV or MI had increased arterial SST2 PET signal (Fig. 1d). Mean aortic TBRmax was strongly correlated with Indian Takayasu Clinical Activity Score (r=0.82 [95% CI 0.46–0.95], p=0.001) and maximum wall thickness on MRI (r=0.68 [95% CI 0.31–0.87], p=0.002). SST2 PET/MRI was generally consistent with 18F-FDG PET/CT in LVV patients with contemporaneous scans (Fig. 1a, b), but with very low background signal in the brain and heart allowing for unimpeded assessment of nearby coronary, myocardial, and intracranial artery involvement. On follow-up imaging after a mean 9.3 (SD 3.2) months, clinically effective treatment for LVV was associated with a 0.49 ±SEM 0.24 (p=0.04; 22.3%) reduction in SST2 PET TBRmax, with good scan-scan repeatability in inactive LVV patients with no change in treatment (ICC 0.86, 95% CI 0.04–0.99). SST2 localised to macrophages, pericytes, and perivascular adipocytes in inflamed arterial specimens (Fig. 2; a: H&amp;E; b: imaging mass cytometry; arrow: SST2/CD68 co-staining). SSTR2-expressing macrophages co-expressed pro-inflammatory markers (S100A8, S100A9). Specific SST2 radioligand binding was confirmed by autoradiography in LVV specimens. Conclusion This is the first study to examine SST2 PET/MRI in LVV and to provide histological and gene expression data for validation. Here we show this novel approach holds major promise for diagnosis and therapeutic monitoring in LVV. Funding Acknowledgement Type of funding sources: Foundation. Main funding source(s): Wellcome Trust; Imperial NIHR Biomedical Research Centre

S2789 A Rare Triad of Ulcerative Colitis, Large Vessel Vasculitis and Celiac Disease

Introduction: Inflammatory bowel disease (IBD) has been associated with large-vessel vasculitis (LVV), with the diagnosis of IBD preceding that of LVV by years. We present for the first time in known literature a triad of concurrent ulcerative colitis (UC), aortitis and celiac disease. Case Description/Methods: A 58 year old Hispanic man with a history of hypertension and gout presented with two weeks of intractable temporomandibular headaches, and two months of non-bloody diarrhea and weight loss. Physical exam was unremarkable. Labs showed hemoglobin 6.9 g/dL, erythrocyte sedimentation rate 120 mm/hr, C-reactive protein 281 mg/dL and IgA tissue transglutaminase antibody 23 U/mL. ANA, C3, C4, proteinase-3 and myeloperoxidase antibodies were within normal limits. Colonoscopy showed pancolitis from rectum to ascending colon. The terminal ileum was normal. Abdominal MRI found aortic wall hyperintensity from the renal arteries to common iliac bifurcation. CT angiogram showed wall thickening of the left carotid artery, aortic arch, descending thoracic and abdominal aorta, consistent with vasculitis. Patient was given stress dose steroids with improvement in headache and normalization of ESR and CRP. Temporal artery biopsy was unremarkable. Four months after hospitalization, repeat colonoscopy with duodenal biopsies for celiac disease revealed mild increase in intraepithelial lymphocytes with preserved villous architecture. He was started on a gluten free diet and adalimumab in combination with methotrexate for UC and LVV. Discussion: About 10 case reports of patients with both UC and either Takayasu (TAK) or giant cell arteritis (GCA) have been described, with UC typically diagnosed 15-45 years before the vasculitis. Vasculitis in the GI tract can mimic IBD, making colonoscopy and biopsy crucial for diagnosis. HLA haplotypes A24, B52, and DR2 are associated with both UC and aortitis and Interleukin-9, observed in temporal arteritis lesions, may be implicated in the pathogenesis of UC. Shared chromosomal variants between patients with UC and celiac disease may explain why IBD risk is up to 9-fold higher in patients with celiac disease. Our patient may have presented with isolated aortitis or an early form of GCA. Methotrexate is used to treat LVV and is combined with an anti-TNF agent to treat UC, as in our case. This is the first known report of co-occurring UC, celiac disease and aortitis; however, whether the three inflammatory conditions are mechanistically related warrants further research.

Open Bypass For Rapid Expansion Of Saccular Left Common Carotid Artery Aneurysm In Takayasu's Arteritis Patient

Takayasu’s arteritis (TA) is a rare systemic inflammatory disease occurring in young patients, characterized by large vessel vasculitis. Although the complete etiology is unknown, it is thought to be caused by inflammatory granulomatous vasculitis leading to transmural fibrosis thickening of arterial walls and degeneration of elastic fibers, leading to vascular obstruction, ischemia, and aneurysms. Diagnosis is based on arteriogram and clinical presentation is inclusive of diminished or absent pulses, vascular bruits, and hypertension.

S2738 Isolated Celiac Artery Vasculitis Presenting as Ileus in a Patient With Ulcerative Colitis

Introduction: Small case series have reported an association between IBD and large vessel vasculitides, ANCA-associated vasculitis, and cutaneous vasculitis. Gastrointestinal (GI) involvement of vasculitis can be a severe complication, resulting in mesenteric ischemia, ileus, or GI bleeding, and often requires aggressive medical or surgical interventions. We report a case of celiac artery vasculitis presenting as abdominal pain and ileus in a patient with ulcerative colitis (UC). Case Description/Methods: A 69 year old male with an 11 year history of UC in remission on infliximab and azathioprine (AZA) presented to the emergency room with abdominal pain and constipation for five days. He had decreased AZA from 200 mg to 100 mg one month prior. Initial CT abdomen and pelvis showed stranding surrounding the celiac artery, fluid-filled small bowel loops, and segmental areas of distal colon collapse with proximal colon distention. He developed progressive small bowel and colonic distention with cecum diameter of 10 cm. A colonoscopy was performed for decompression. He had no evidence of active UC. EGD demonstrated dusky appearing gastroesophageal junction and inflammation with cratered ulcers in the gastric cardia and body. Biopsies confirmed ischemic necrosis. CTA demonstrated perivascular inflammation of a patent celiac artery without evidence for dissection, suggestive of acute vasculitis. ESR and CRP were 50 mm/hr and 7.8 mg/dL respectively. Myeloperoxidase, serine-proteinase 3, rheumatoid factor, ANA, were negative. He was started on methylprednisolone IV with oral prednisone taper at discharge, and AZA was increased to 200 mg daily. A CT one month later showed minimal residual inflammation. ESR and CRP had normalized. (Figure) Discussion: We present the first case of isolated celiac artery vasculitis in a patient with IBD. A diagnosis of IBD often precedes vasculitis, and patients are typically on immunosuppressive therapy at the time of onset. Anti-TNF agents have been implicated as a potential inciting cause. Although it is unclear whether long-term anti-TNF therapy precipitated vasculitis in our patient, the dose reduction in AZA prior to presentation may have unmasked vasculitis. This was supported by his rapid clinical and radiographic improvement with combination of steroids and increased AZA dose despite continuation of infliximab. This case highlights the importance of maintaining a high degree of suspicion for vasculitis in patients with IBD on immunosuppressive therapy presenting with ischemia or ileus.Figure 1.: A 69 year old male underwent EGD for evaluation of abdominal pain and NG tube placement; he was found to have inflammation with a dusky appearing mucosa at the gastroesophageal junction (GEJ) (A). Non-bleeding gastric ulcers with overlying pigment (Forrest Class IIc) were identified (B). H&E stain image shows the GEJ biopsy with extensive ischemic necrosis and hemorrhage (C). Initial CTA abdomen/pelvis shows fat stranding surrounding the celiac artery (D).

LOOKING BEYOND THE ROUTINE CAUSES OF HEART FAILURE

LOOKING BEYOND THE ROUTINE CAUSES OF HEART FAILURE