Large-vessel vasculitis

2013 ◽  
pp. 1113-1124
Author(s):  
Nicolò Pipitone ◽  
Annibale Versari ◽  
Carlo Salvarani
Keyword(s):  
Inflammatory Markers ◽  
Visual Loss ◽  
Imaging Techniques ◽  
Immunosuppressive Agents ◽  
Disease Onset ◽  
Vascular Complications ◽  
Large Vessel Vasculitis ◽  
Large Vessel ◽  
Biological Drugs ◽  
Temporal Arteries

Large-vessel vasculitis includes giant cell arteritis (GCA) and Takayasu’s arteritis (TAK). GCA affects patients aged over 50, mainly of white European ethnicity. GCA occurs together with polymyalgia rheumatica (PMR) more frequently than expected by chance. In both conditions, females are affected two to three times more often than males. GCA mainly involves large- and medium-sized arteries, particularly the branches of the proximal aorta including the temporal arteries. Vasculitic involvement results in the typical manifestations of GCA including temporal headache, jaw claudication, and visual loss. A systemic inflammatory response and a marked response to glucocorticoids is characteristic of GCA. GCA usually remits within 6 months to 2 years from disease onset. However, some patients have a chronic-relapsing course and may require longstanding treatment. Mortality is not increased, but there is significant morbidity mainly related to chronic glucocorticoid use and cranial ischaemic events, especially visual loss. The diagnosis of GCA rests on the characteristic clinical features and raised inflammatory markers, but temporal artery biopsy remains the gold standard to support the clinical suspicion. Imaging techniques are also used to demonstrate large-vessel involvement in GCA. Glucocorticoids are the mainstay of treatment for GCA, but other therapeutic approaches have been proposed and novel ones are being developed. TAK mainly involves the aorta and its main branches. Women are particularly affected with a female:male ratio of 9:1. In most patients, age of onset is between 20 and 30 years. Early manifestations of TAK are non-specific and include constitutional and musculoskeletal symptoms. Later on, vascular complications become manifest. Most patients develop vessel stenoses, particularly in the branches of the aortic artery, leading to manifestations of vascular hypoperfusion. Aneurysms occur in a minority of cases. There are no specific laboratory tests to diagnose TAK, although most patients have raised inflammatory markers, therefore, imaging techniques are required to secure the diagnosis. Glucocorticoids are the mainstay of treatment of TAK. However, many patients have an insufficient response to glucocorticoids alone, or relapse when they are tapered or discontinued. Immunosuppressive agents and, in refractory cases, biological drugs can often attain disease control and prevent vascular complications. Revascularization procedures are required in patients with severe established stenoses or occlusions.

Large-vessel vasculitis

2013 ◽  
pp. 1113-1124
Author(s):  
Nicolò Pipitone ◽  
Annibale Versari ◽  
Carlo Salvarani
Keyword(s):  
Inflammatory Markers ◽  
Visual Loss ◽  
Imaging Techniques ◽  
Immunosuppressive Agents ◽  
Disease Onset ◽  
Vascular Complications ◽  
Large Vessel Vasculitis ◽  
Large Vessel ◽  
Biological Drugs ◽  
Temporal Arteries

Large-vessel vasculitis includes giant cell arteritis (GCA) and Takayasu’s arteritis (TAK). GCA affects patients aged over 50, mainly of white European ethnicity. GCA occurs together with polymyalgia rheumatica (PMR) more frequently than expected by chance. In both conditions, females are affected two to three times more often than males. GCA mainly involves large- and medium-sized arteries, particularly the branches of the proximal aorta including the temporal arteries. Vasculitic involvement results in the typical manifestations of GCA including temporal headache, jaw claudication, and visual loss. A systemic inflammatory response and a marked response to glucocorticoids is characteristic of GCA. GCA usually remits within 6 months to 2 years from disease onset. However, some patients have a chronic-relapsing course and may require longstanding treatment. Mortality is not increased, but there is significant morbidity mainly related to chronic glucocorticoid use and cranial ischaemic events, especially visual loss. The diagnosis of GCA rests on the characteristic clinical features and raised inflammatory markers, but temporal artery biopsy remains the gold standard to support the clinical suspicion. Imaging techniques are also used to demonstrate large-vessel involvement in GCA. Glucocorticoids are the mainstay of treatment for GCA, but other therapeutic approaches have been proposed and novel ones are being developed. TAK mainly involves the aorta and its main branches. Women are particularly affected with a female:male ratio of 9:1. In most patients, age of onset is between 20 and 30 years. Early manifestations of TAK are non-specific and include constitutional and musculoskeletal symptoms. Later on, vascular complications become manifest. Most patients develop vessel stenoses, particularly in the branches of the aortic artery, leading to manifestations of vascular hypoperfusion. Aneurysms occur in a minority of cases. There are no specific laboratory tests to diagnose TAK, although most patients have raised inflammatory markers, therefore, imaging techniques are required to secure the diagnosis. Glucocorticoids are the mainstay of treatment of TAK. However, many patients have an insufficient response to glucocorticoids alone, or relapse when they are tapered or discontinued. Immunosuppressive agents and, in refractory cases, biological drugs can often attain disease control and prevent vascular complications. Revascularization procedures are required in patients with severe established stenoses or occlusions.

Large-vessel vasculitis

2013 ◽  
Author(s):  
Nicolò Pipitone ◽  
Annibale Versari ◽  
Carlo Salvarani
Keyword(s):  
Inflammatory Markers ◽  
Visual Loss ◽  
Imaging Techniques ◽  
Immunosuppressive Agents ◽  
Vascular Complications ◽  
Large Vessel Vasculitis ◽  
Large Vessel ◽  
Musculoskeletal Symptoms ◽  
Temporal Artery Biopsy ◽  
Vascular Hypoperfusion

Large-vessel vasculitis includes giant cell arteritis (GCA) and Takayasu's arteritis (TAK). GCA affects patients aged over 50, mainly of white European ethnicity. GCA occurs together with polymyalgia rheumatica (PMR) more frequently than expected by chance. In both conditions, females are affected two to three times more often than males. GCA mainly involves large- and medium-sized arteries, particularly the branches of the proximal aorta including the temporal arteries. Vasculitic involvement results in the typical manifestations of GCA including temporal headache, jaw claudication, and visual loss. A systemic inflammatory response and a marked response to glucocorticoids is characteristic of GCA. GCA usually remits within 6 months to 2 years from disease onset. However, some patients have a chronic-relapsing course and may require long-standing treatment. Mortality is not increased, but there is significant morbidity mainly related to chronic glucocorticoid use and cranial ischaemic events, especially visual loss. The diagnosis of GCA rests on the characteristic clinical features and raised inflammatory markers, but temporal artery biopsy remains the gold standard to support the clinical suspicion. Imaging techniques are also used to demonstrate large-vessel involvement in GCA. Glucocorticoids are the mainstay of treatment for GCA, but other therapeutic approaches have been proposed and novel ones are being developed. TAK mainly involves the aorta and its main branches. Women are particularly affected with a female:male ratio of 9:1. In most patients, age of onset is between 20 and 30 years. Early manifestations of TAK are non-specific and include constitutional and musculoskeletal symptoms. Later on, vascular complications become manifest. Most patients develop vessel stenoses, particularly in the branches of the aortic artery, leading to manifestations of vascular hypoperfusion. Aneurysms occur in a minority of cases. There are no specific laboratory tests to diagnose TAK, although most patients have raised inflammatory markers, therefore, imaging techniques are required to secure the diagnosis. Glucocorticoids are the mainstay of treatment of TAK. However, many patients have an insufficient response to glucocorticoids alone, or relapse when they are tapered or discontinued. Immunosuppressive agents and, in refractory cases, biological drugs can often attain disease control and prevent vascular complications. Revascularization procedures are required in patients with severe established stenoses or occlusions.

Large-vessel vasculitis

2016 ◽  
Author(s):  
Nicolò Pipitone ◽  
Annibale Versari ◽  
Carlo Salvarani
Keyword(s):  
Inflammatory Markers ◽  
Visual Loss ◽  
Imaging Techniques ◽  
Immunosuppressive Agents ◽  
Vascular Complications ◽  
Large Vessel Vasculitis ◽  
Large Vessel ◽  
Musculoskeletal Symptoms ◽  
Temporal Artery Biopsy ◽  
Vascular Hypoperfusion

Large-vessel vasculitis includes giant cell arteritis (GCA) and Takayasu’s arteritis (TAK). GCA affects patients aged over 50, mainly of white European ethnicity. GCA occurs together with polymyalgia rheumatica (PMR) more frequently than expected by chance. In both conditions, females are affected two to three times more often than males. GCA mainly involves large- and medium-sized arteries, particularly the branches of the proximal aorta including the temporal arteries. Vasculitic involvement results in the typical manifestations of GCA including temporal headache, jaw claudication, and visual loss. A systemic inflammatory response and a marked response to glucocorticoids is characteristic of GCA. GCA usually remits within 6 months to 2 years from disease onset. However, some patients have a chronic-relapsing course and may require longstanding treatment. Mortality is not increased, but there is significant morbidity mainly related to chronic glucocorticoid use and cranial ischaemic events, especially visual loss. The diagnosis of GCA rests on the characteristic clinical features and raised inflammatory markers, but temporal artery biopsy remains the gold standard to support the clinical suspicion. Imaging techniques are also used to demonstrate large-vessel involvement in GCA. Glucocorticoids are the mainstay of treatment for GCA, but other therapeutic approaches have been proposed and novel ones are being developed. TAK mainly involves the aorta and its main branches. Women are particularly affected with a female:male ratio of 9:1. In most patients, age of onset is between 20 and 30 years. Early manifestations of TAK are non-specific and include constitutional and musculoskeletal symptoms. Later on, vascular complications become manifest. Most patients develop vessel stenoses, particularly in the branches of the aortic artery, leading to manifestations of vascular hypoperfusion. Aneurysms occur in a minority of cases. There are no specific laboratory tests to diagnose TAK, although most patients have raised inflammatory markers, therefore, imaging techniques are required to secure the diagnosis. Glucocorticoids are the mainstay of treatment of TAK. However, many patients have an insufficient response to glucocorticoids alone, or relapse when they are tapered or discontinued. Immunosuppressive agents and, in refractory cases, biological drugs can often attain disease control and prevent vascular complications. Revascularization procedures are required in patients with severe established stenoses or occlusions.

scholarly journals Pericarditis Revealing Large Vessel Vasculitis

2011 ◽  
Vol 2011 ◽  
pp. 1-2 ◽  
Author(s):  
Bruno Couturier ◽  
Valerie Huyge ◽  
Muhammad S. Soyfoo
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Fdg Pet ◽  
Inflammatory Markers ◽  
Clinical Status ◽  
Large Vessel Vasculitis ◽  
Large Vessel ◽  
Temporal Arteries ◽  
Large Vessels

Large vessels vasculitis and more specifically, Giant cell arteritis, is characterized by increased inflammatory markers, headaches and altered clinical status. Diagnosis is confirmed by biopsy of temporal arteries showing the presence of granuloma and vasculitis. We hereby report the case of a patient presenting initially as pericarditis and revealing large vessel vasculitis using FDG-PET.

scholarly journals P188 Tocilizumab for giant cell arteritis: real world experience in a single UK centre

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_2) ◽  
Author(s):  
Gary Reynolds ◽  
Bridget Griffiths ◽  
Karolyn Houghton ◽  
Ben Thompson ◽  
Alice R Lorenzi ◽  
...  
Keyword(s):  
Urinary Tract ◽  
Adverse Events ◽  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Urinary Tract Infections ◽  
Visual Loss ◽  
Large Vessel Vasculitis ◽  
Large Vessel ◽  
Tract Infections ◽  
Clinical Records

Abstract Background Tocilizumab was approved for the treatment of giant cell arteritis (GCA) by NICE in April 2018. This decision followed the GiACTA (Giant Cell Arteritis Actemra) study, a randomised control trial that demonstrated a beneficial effect of tocilizumab in reducing the frequency of disease flare and overall prednisolone requirements. However, as noted in the NICE appraisal, the extent to which the patient population in the GiACTA study reflects the population it is eventually used for in UK clinical practice is not clear. To address this, we analysed the records of all patients started on tocilizumab treatment for GCA in a single centre since its approval. Methods We performed a retrospective analysis of the clinical records of all patients started on tocilizumab for GCA at the Freeman Hospital in Newcastle. All patients are discussed in a regional connective tissue disease MDT prior to the initiation of therapy to ensure they fulfil NICE guidelines. At each subsequent visit adverse events related to both tocilizumab and corticosteroids and any flares of GCA or ischaemic events are recorded. Results In total 14 patients started tocilizumab since June 2018, with a cumulative exposure of 8 patient-years. The mean age was 74 years, slightly older than the average age in the GiACTA trial (69 years). In contrast to GiACTA where 48% of patients were newly diagnosed, all our patients had established disease. In our cohort 36% of patients had CT PET evidence of large vessel vasculitis, similar to the rate in GiACTA (40%) but higher than the national average of around 5%. Incidence of visual loss in our treated patients was higher at 29% than recorded in the general GCA population in the DC-VAS study (7.9%). In those treated for relapsing disease around half (56%) had recorded previous significant adverse events with steroids, including heart failure, hypertension and mood changes. Five patients had infections requiring antibiotics (cellulitis/ulcer in three, chest infection in one, urinary tract infections in two), with two serious infections requiring hospital admission (both urinary tract infections). No patients had further ischaemic events while on treatment. All patients were on a lower dose of prednisolone following treatment with an average of a 63% reduction in steroid dose. Conclusion The modest number of patients receiving tocilizumab for GCA suggests we are not treating everyone at first relapse. The higher rates of large vessel vasculitis, visual loss and previous steroid-induced complications suggest a preference for saving tocilizumab for a more severely affected subset of patients. In this preliminary data there were reassuringly no recorded ischaemic events following treatment and steroid doses were successfully reduced in all patients. Disclosures G. Reynolds None. B. Griffiths None. K. Houghton None. B. Thompson None. A.R. Lorenzi None. J. Heaney None.

scholarly journals Takayasu arteritis: a cohort of Italian patients and recent pathogenetic and therapeutic advances

2020 ◽  
Author(s):  
Franco Dammacco ◽  
Anna Cirulli ◽  
Annalisa Simeone ◽  
Patrizia Leone ◽  
Raffaele Pulli ◽  
...  
Keyword(s):  
Clinical Features ◽  
Takayasu Arteritis ◽  
Imaging Techniques ◽  
Immunosuppressive Agents ◽  
Disease Onset ◽  
Response To Therapy ◽  
Disease Classification ◽  
Vascular Involvement ◽  
Unknown Etiology ◽  
Biologic Drugs

Abstract Takayasu arteritis (TAK) is a rare granulomatous vasculitis of unknown etiology that mainly affects the aorta and its major branches. The aim is to describe the clinical features, diagnostic procedures, pathogenesis, and management of TAK in a longitudinal cohort of patients recruited within a single region of southern Italy. The cohort included 43 patients who were diagnosed with TAK and followed up according to a standard protocol, in a collaboration between four university tertiary referral centers and a regional hospital. Clinical and imaging classification criteria were those established by the American College of Rheumatology. Thirty-five patients (81.4%) were female, and the mean age at disease onset was 32.6 (range 16–54) years. Angiographic assessment of the vascular involvement allowed disease classification in five different types. Clinical features ranged from constitutional symptoms in the early inflammatory stage of the disease to cardiovascular ischemic symptoms in the late, chronic stage. Noninvasive imaging techniques were employed to assess the extent and severity of the arterial wall damage and to monitor the clinical course and response to therapy. Medical treatment, based on pathogenetic insights into the roles of humoral and cell-mediated immune mechanisms, included glucocorticoids mostly combined with steroid-sparing immunosuppressive agents and, in patients with relapsing/refractory disease, biologic drugs. Significant clinical and angiographic differences have been detected in TAK patients from different geographic areas. Patients with life-threatening cardiovascular and neurologic manifestations as well as sight-threatening ophthalmologic signs and symptoms should be promptly diagnosed, properly treated, and closely followed up to avoid potentially severe consequences.

scholarly journals P35 Vasculitis by any other name would present just the same

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_2) ◽  
Author(s):  
Sajida Rasul ◽  
Morven Dockery ◽  
Rachel Tattersall ◽  
Dan Hawley ◽  
Sarah Maltby ◽  
...  
Keyword(s):  
Inflammatory Bowel Disease ◽  
Bowel Disease ◽  
Inflammatory Markers ◽  
Large Vessel Vasculitis ◽  
Large Vessel ◽  
Adult Care ◽  
Secondary Failure ◽  
Adolescent Patients ◽  
Night Sweats ◽  
Inflammatory Bowel

Abstract Background Vasculitis can present in many ways and large vessel vascultis is reported rarely to co-present with inflammatory bowel disease. We would like to present two adolescent patients who presented in very similar ways via the gastroenterology team with a seemingly clear diagnosis of inflammatory bowel disease but who were found to have large vessel vasculitis later in their disease journey. The presentation is to raise awareness of this rare co-presentation and to discuss treatment challenges in particular those apparent in adolescent patients crossing the transition bridge. Methods Patient A is a 17 year old boy who has had a long and rocky road to control of his inflammatory bowel disease which presented when he was 2 years old. Histologically it fitted a Crohn’s classification. His journey included moderate response to oral steroids and little to no response to a range of DMARDs, biologics (including infliximab and adalimumab which both had secondary failure) and elemental nutrition, over a period of 12 years. Vedolizumab was introduced this year with almost immediate improvement of gut symptoms, but with ongoing raised inflammatory markers (CRP 79, ESR 86). Incidental investigations of neck pain following the start of vedolizumab revealed significant abnormality in the external carotids, with 70% stenosis. MR angiography confirmed a typical pattern of stenotic large vessel vasculitis. The second patient, B is also 17 and was diagnosed with histological ulcerative colitis aged 14. He has an older brother with IBD but has recently been found to have small bowel disease and is likely therefore to have Crohn’s disease. He is on infliximab 10mg/kg 4 weekly but presented with a 3-month history of high inflammatory markers, malaise towards the end of the 4 week infliximab cycle and drenching night sweats. CT Chest confirmed vasculitis in the thoracic aorta, subclavians and carotids. On PET CT there is mural thickening and no stenosis. Results Patient A presented in paediatric care and B in adult care but because of the seamless rheumatology service and combined MDT with gastroenterology both patients’ care has been widely discussed amongst relevant adult and paediatric teams. Conclusion Large vessel vasculitis might be driving the inflammatory bowel disease in both patients as such the life threatening element of the disease ought to be managed immediately, while ensuring safe transition to between paediatric and adult care. Disclosures S. Rasul None. M. Dockery None. R. Tattersall None. D. Hawley None. S. Maltby None. A. McMahon None.

scholarly journals Semi-Quantitative and Quantitative [18F]FDG-PET/CT Indices for Diagnosing Large Vessel Vasculitis: A Critical Review

Diagnostics ◽  
2021 ◽  
Vol 11 (12) ◽  
pp. 2355
Author(s):  
Olivier Gheysens ◽  
François Jamar ◽  
Andor W. J. M. Glaudemans ◽  
Halil Yildiz ◽  
Kornelis S. M. van der Geest
Keyword(s):  
Fdg Pet ◽  
Imaging Techniques ◽  
Visual Assessment ◽  
Large Vessel Vasculitis ◽  
Large Vessel ◽  
Liver Uptake ◽  
Fdg Uptake ◽  
Pet Ct ◽  
18F Fdg ◽  
Fdg Pet Ct

To confirm the diagnosis of large vessel vasculitis (LVV) with high accuracy, one of the recommended imaging techniques is [18F]Fluoro-2-deoxy-d-glucose positron emission tomography with computed tomography ([18F]FDG-PET/CT). Visual assessment of [18F]FDG uptake in the arterial wall compared to liver uptake is the mainstay for diagnosing LVV in routine clinical practice. To date, there is no consensus on the preferred semi-quantitative or quantitative parameter for diagnosing LVV. The aim of this review is to critically update the knowledge on the available evidence of semi-quantitative and quantitative [18F]FDG uptake parameters for diagnosing LVV and to provide future directions for methodological standardization and research.

scholarly journals Imaging Tests in the Early Diagnosis of Giant Cell Arteritis

2021 ◽  
Vol 10 (16) ◽  
pp. 3704
Author(s):  
Diana Prieto-Peña ◽  
Santos Castañeda ◽  
Isabel Martínez-Rodríguez ◽  
Belén Atienza-Mateo ◽  
Ricardo Blanco ◽  
...  
Keyword(s):  
Early Diagnosis ◽  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Early Recognition ◽  
Imaging Modality ◽  
Imaging Techniques ◽  
Vascular Complications ◽  
Temporal Artery ◽  
Temporal Artery Biopsy ◽  
Temporal Arteries

Early recognition of giant cell arteritis (GCA) is crucial to avoid the development of ischemic vascular complications, such as blindness. The classic approach to making the diagnosis of GCA is based on a positive temporal artery biopsy, which is among the criteria proposed by the American College of Rheumatology (ACR) in 1990 to classify a patient as having GCA. However, imaging techniques, particularly ultrasound (US) of the temporal arteries, are increasingly being considered as an alternative for the diagnosis of GCA. Recent recommendations from the European League Against Rheumatism (EULAR) for the use of imaging techniques for large vessel vasculitis (LVV) included US as the first imaging option for the diagnosis of GCA. Furthermore, although the ACR classification criteria are useful in identifying patients with the classic cranial pattern of GCA, they are often inadequate in identifying GCA patients who have the extracranial phenotype of LVV. In this sense, the advent of other imaging techniques, such as magnetic resonance imaging (MRI), computed tomography (CT), and positron emission tomography (PET)/CT, has made it possible to detect the presence of extracranial involvement of the LVV in patients with GCA presenting as refractory rheumatic polymyalgia without cranial ischemic manifestations. Imaging techniques have been the key elements in redefining the diagnostic work-up of GCA. US is currently considered the main imaging modality to improve the early diagnosis of GCA.

scholarly journals Giant Cell Arteritis: Current Advances in Pathogenesis and Treatment

2020 ◽  
Author(s):  
Marília A. Dagostin ◽  
Rosa M.R. Pereira
Keyword(s):  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Visual Loss ◽  
Imaging Techniques ◽  
Limb Ischemia ◽  
Temporal Artery ◽  
Medical Emergency ◽  
Common Symptom ◽  
Irreversible Damage ◽  
Temporal Arteries

Giant cell arteritis (GCA) is the most common vasculitis in adults, with the incidence increasing with the advancing age. The aorta and its branches, especially the carotid extracranial branches, are the classic targets of inflammation in GCA. Visual loss, upper limb ischemia, and stroke are complications described. Suspicion of GCA is a medical emergency, and patients need to be quickly diagnosed/treated to prevent irreversible damage. Headache is the most common symptom, and a new-onset headache in older adults should always raise the suspicion of GCA. Patients may also present with scalp tenderness or tongue/jaw pain. GCA is often found to be the cause of an obscure-origin fever in older patients. A positive temporal artery biopsy is considered the gold standard for the diagnosis, but imaging techniques enable the assessment of cranial and extracranial arteries and the aorta. Ultrasound of temporal arteries is recommended and noncompressible “halo” sign is the typical finding. PET, MRI, or CT may be useful for the detection of the disease in the aorta and other vessels. The treatment must be started promptly with prednisone 1 mg/kg/day. When visual symptoms/unilateral visual loss is present, methylprednisolone pulse is recommended. Methotrexate, leflunomide and tocilizumab may be effective and well-tolerated glucocorticoid-sparing agents in GCA. Cardiovascular diseases are the leading causes of death in patients.

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