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Annals of the Rheumatic Diseases Jan 2020Since the publication of the European League Against Rheumatism (EULAR) recommendations for the management of large vessel vasculitis (LVV) in 2009, several relevant...
BACKGROUND
Since the publication of the European League Against Rheumatism (EULAR) recommendations for the management of large vessel vasculitis (LVV) in 2009, several relevant randomised clinical trials and cohort analyses have been published, which have the potential to change clinical care and therefore supporting the need to update the original recommendations.
METHODS
Using EULAR standardised operating procedures for EULAR-endorsed recommendations, the EULAR task force undertook a systematic literature review and sought opinion from 20 experts from 13 countries. We modified existing recommendations and created new recommendations.
RESULTS
Three overarching principles and 10 recommendations were formulated. We recommend that a suspected diagnosis of LVV should be confirmed by imaging or histology. High dose glucocorticoid therapy (40-60 mg/day prednisone-equivalent) should be initiated immediately for induction of remission in active giant cell arteritis (GCA) or Takayasu arteritis (TAK). We recommend adjunctive therapy in selected patients with GCA (refractory or relapsing disease, presence of an increased risk for glucocorticoid-related adverse events or complications) using tocilizumab. Methotrexate may be used as an alternative. Non-biological glucocorticoid-sparing agents should be given in combination with glucocorticoids in all patients with TAK and biological agents may be used in refractory or relapsing patients. We no longer recommend the routine use of antiplatelet or anticoagulant therapy for treatment of LVV unless it is indicated for other reasons.
CONCLUSIONS
We have updated the recommendations for the management of LVV to facilitate the translation of current scientific evidence and expert opinion into better management and improved outcome of patients in clinical practice.
Topics: Antibodies, Monoclonal, Humanized; Antirheumatic Agents; Aortitis; Giant Cell Arteritis; Glucocorticoids; Humans; Methotrexate; Takayasu Arteritis
PubMed: 31270110
DOI: 10.1136/annrheumdis-2019-215672 -
Circulation Jan 2021Systemic vasculitides are multisystem blood vessel disorders, which are defined by the size of the vessel predominantly affected, namely small, medium, or large vessels.... (Review)
Review
Systemic vasculitides are multisystem blood vessel disorders, which are defined by the size of the vessel predominantly affected, namely small, medium, or large vessels. The term "large vessel" relates to the aorta and its major branches; "medium vessel" refers to the main visceral arteries and veins and their initial branches. The most common causes of large-vessel vasculitis are giant cell arteritis and Takayasu arteritis, and those of medium-vessel arteritis are polyarteritis nodosa and Kawasaki disease. However, there is some overlap, and arteries of any size can potentially be involved in any of the 3 main categories of dominant vessel involvement. In addition to multisystem vasculitides, other forms of vasculitis have been defined, including single-organ vasculitis (eg, isolated aortitis). Prompt identification of vasculitides is important because they are associated with an increased risk of mortality. Left undiagnosed or mismanaged, these conditions may result in serious adverse outcomes that might otherwise have been avoided or minimized. The ethnic and regional differences in the incidence, prevalence, and clinical characteristics of patients with vasculitis should be recognized. Because the clinical presentation of vasculitis is highly variable, the cardiovascular clinician must have a high index of suspicion to establish a reliable and prompt diagnosis. This article reviews the pathophysiology, epidemiology, diagnostic strategies, and management of vasculitis.
Topics: Animals; Giant Cell Arteritis; Glucocorticoids; Humans; Mucocutaneous Lymph Node Syndrome; Polyarteritis Nodosa; Takayasu Arteritis; Vasculitis
PubMed: 33464968
DOI: 10.1161/CIRCULATIONAHA.120.046657 -
Presse Medicale (Paris, France : 1983) Apr 2020Aortitis and periaortitis are inflammatory diseases of the aorta and its main branches; they differ in the extension of inflammation, which is confined to the aortic... (Review)
Review
Aortitis and periaortitis are inflammatory diseases of the aorta and its main branches; they differ in the extension of inflammation, which is confined to the aortic wall in aortitis, and spreads to the periaortic space in periaortitis. Aortitis is classified as non-infectious or infectious. Non-infectious aortitis represents a common feature of large-vessel vasculitides but can also be isolated or associated with other rheumatologic conditions. Periaortitis can be idiopathic or secondary to a wide array of etiologies such as drugs, infections, malignancies, and other proliferative diseases. Notably, both aortitis and periaortitis may arise in the context of IgG4-related disease, a recently characterised fibro-inflammatory systemic disease. Prompt recognition, correct diagnosis and appropriate treatment are essential in order to avoid life-threatening complications.
Topics: Aorta; Aortitis; Diagnostic Imaging; Giant Cell Arteritis; Humans; Immunoglobulin G4-Related Disease; Retroperitoneal Fibrosis; Takayasu Arteritis
PubMed: 32234379
DOI: 10.1016/j.lpm.2020.104018 -
Heart (British Cardiac Society) Oct 2021Broadly defined, aortitis refers to inflammation of the aorta and incorporates both infectious and non-infectious aetiologies. As advanced imaging modalities are... (Review)
Review
Broadly defined, aortitis refers to inflammation of the aorta and incorporates both infectious and non-infectious aetiologies. As advanced imaging modalities are increasingly incorporated into clinical practice, the phenotypic spectrum associated with aortitis has widened. The primary large vessel vasculitides, giant cell arteritis and Takayasu arteritis, are the most common causes of non-infectious aortitis. Aortitis without systemic disease or involvement of other vascular territories is classified as clinically isolated aortitis. Periaortitis, where inflammation spreads beyond the aortic wall, is an important disease subset with a distinct group of aetiologies. Infectious aortitis can involve bacterial, viral or fungal pathogens and, while uncommon, can be devastating. Importantly, optimal management strategies and patient outcomes differ between aortitis subgroups highlighting the need for a thorough diagnostic workup. Monitoring disease activity over time is also challenging as normal inflammatory markers do not exclude significant vascular inflammation, particularly after starting treatment. Additional areas of unmet clinical need include clear disease classifications and improved short-term and long-term management strategies. Some of these calls are now being answered, particularly with regard to large vessel vasculitis where our understanding has advanced significantly in recent years. Work extrapolated from temporal artery histology has paved the way for targeted biological agents and, although glucocorticoids remain central to the management of non-infectious aortitis, these may allow reduced glucocorticoid reliance. Future work should seek to clarify disease definitions, improve diagnostic pathways and ultimately allow a more stratified approach to patient management.
Topics: Aorta; Aortitis; Humans; Tomography, X-Ray Computed
PubMed: 33593995
DOI: 10.1136/heartjnl-2020-318085 -
Journal of the American College of... Dec 2022The "2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease" provides recommendations to guide clinicians in the diagnosis, genetic evaluation and...
2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease: A Report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines.
AIM
The "2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease" provides recommendations to guide clinicians in the diagnosis, genetic evaluation and family screening, medical therapy, endovascular and surgical treatment, and long-term surveillance of patients with aortic disease across its multiple clinical presentation subsets (ie, asymptomatic, stable symptomatic, and acute aortic syndromes).
METHODS
A comprehensive literature search was conducted from January 2021 to April 2021, encompassing studies, reviews, and other evidence conducted on human subjects that were published in English from PubMed, EMBASE, the Cochrane Library, CINHL Complete, and other selected databases relevant to this guideline. Additional relevant studies, published through June 2022 during the guideline writing process, were also considered by the writing committee, where appropriate.
STRUCTURE
Recommendations from previously published AHA/ACC guidelines on thoracic aortic disease, peripheral artery disease, and bicuspid aortic valve disease have been updated with new evidence to guide clinicians. In addition, new recommendations addressing comprehensive care for patients with aortic disease have been developed. There is added emphasis on the role of shared decision making, especially in the management of patients with aortic disease both before and during pregnancy. The is also an increased emphasis on the importance of institutional interventional volume and multidisciplinary aortic team expertise in the care of patients with aortic disease.
Topics: United States; Humans; American Heart Association; Universities; Aortic Diseases
PubMed: 36334952
DOI: 10.1016/j.jacc.2022.08.004 -
Circulation Research Jan 2020Through localized delivery of rapamycin via a biomimetic drug delivery system, it is possible to reduce vascular inflammation and thus the progression of vascular...
RATIONALE
Through localized delivery of rapamycin via a biomimetic drug delivery system, it is possible to reduce vascular inflammation and thus the progression of vascular disease.
OBJECTIVE
Use biomimetic nanoparticles to deliver rapamycin to the vessel wall to reduce inflammation in an in vivo model of atherosclerosis after a short dosing schedule.
METHODS AND RESULTS
Biomimetic nanoparticles (leukosomes) were synthesized using membrane proteins purified from activated J774 macrophages. Rapamycin-loaded nanoparticles were characterized using dynamic light scattering and were found to have a diameter of 108±2.3 nm, a surface charge of -15.4±14.4 mV, and a polydispersity index of 0.11 +/ 0.2. For in vivo studies, ApoE mice were fed a high-fat diet for 12 weeks. Mice were injected with either PBS, free rapamycin (5 mg/kg), or rapamycin-loaded leukosomes (Leuko-Rapa; 5 mg/kg) once daily for 7 days. In mice treated with Leuko-Rapa, flow cytometry of disaggregated aortic tissue revealed fewer proliferating macrophages in the aorta (15.6±9.79 %) compared with untreated mice (30.2±13.34 %) and rapamycin alone (26.8±9.87 %). Decreased macrophage proliferation correlated with decreased levels of MCP (monocyte chemoattractant protein)-1 and IL (interleukin)-b1 in mice treated with Leuko-Rapa. Furthermore, Leuko-Rapa-treated mice also displayed significantly decreased MMP (matrix metalloproteinases) activity in the aorta (mean difference 2554±363.9, =9.95122×10). No significant changes in metabolic or inflammation markers observed in liver metabolic assays. Histological analysis showed improvements in lung morphology, with no alterations in heart, spleen, lung, or liver in Leuko-Rapa-treated mice.
CONCLUSIONS
We showed that our biomimetic nanoparticles showed a decrease in proliferating macrophage population that was accompanied by the reduction of key proinflammatory cytokines and changes in plaque morphology. This proof-of-concept showed that our platform was capable of suppressing macrophage proliferation within the aorta after a short dosing schedule (7 days) and with a favorable toxicity profile. This treatment could be a promising intervention for the acute stabilization of late-stage plaques.
Topics: 1,2-Dipalmitoylphosphatidylcholine; Animals; Aorta; Aortitis; Apolipoproteins E; Atherosclerosis; Biomimetics; C-Reactive Protein; Cryoelectron Microscopy; Cytokines; Drug Evaluation, Preclinical; Macrophage Activation; Macrophages; Mechanistic Target of Rapamycin Complex 1; Membrane Proteins; Mice; Mice, Inbred C57BL; Nanoparticles; Neovascularization, Pathologic; Organ Specificity; Phosphatidylcholines; Plaque, Atherosclerotic; Random Allocation; Sirolimus
PubMed: 31647755
DOI: 10.1161/CIRCRESAHA.119.315185 -
Clinics in Chest Medicine Sep 2019Immunoglobulin G4 (IgG4)-Related Disease (IgG4-RD) can cause fibroinflammatory lesions in nearly any organ and lead to organ dysfunction and irreversible damage. In... (Review)
Review
Immunoglobulin G4 (IgG4)-Related Disease (IgG4-RD) can cause fibroinflammatory lesions in nearly any organ and lead to organ dysfunction and irreversible damage. In addition to frequent involvement of the salivary glands, lacrimal glands, and/or pancreas, IgG4-RD often affects the chest. Thoracic manifestations include lung nodules and consolidations, pleural thickening, aortitis, and lymphadenopathy. The diagnosis is made after careful clinicopathologic correlation because there is no single diagnostic test with excellent sensitivity or specificity. Biopsy of pulmonary lesions can be useful for distinguishing IgG4-RD from common mimickers. Immunosuppressive regimens, such as glucocorticoids and/or glucocorticoid-sparing agents, form the cornerstone of treatment.
Topics: Humans; Immunoglobulin G4-Related Disease; Lung
PubMed: 31376893
DOI: 10.1016/j.ccm.2019.05.005 -
Current Opinion in Rheumatology Jan 2021Aortitis is the inflammation of the aorta due to various causes. Clinical presentations vary as well as the imaging findings. Exact pathogenetic mechanisms or triggering... (Review)
Review
PURPOSE OF REVIEW
Aortitis is the inflammation of the aorta due to various causes. Clinical presentations vary as well as the imaging findings. Exact pathogenetic mechanisms or triggering factors, as well as the best diagnostic and monitoring modalities and treatment strategies, are yet to be elucidated. We reviewed recent studies in aortitis and associated diseases.
RECENT FINDINGS
Multiple cohort studies reporting long-term outcomes in patients with noninfectious aortitis were recently published. Comparative features of isolated aortitis were described. Six angiographic clusters for giant cell arteritis and Takayasu have been identified. New classification criteria have been proposed for IgG4-related disease by a data-driven method. The ultrasonographic slope sign and a halo score were described as specific imaging parameters in giant cell arteritis. The promising role of PET-computed tomography, not only in the diagnosis of aortitis but also in monitoring disease activity, has been noted. Results of in-vitro studies on Janus kinase (JAK)/signal transducers and activators of transcription and mammalian target of rapamycin (mTOR) pathways, comparative studies with leflunomide as an induction therapy, and a long-term follow-up study with tocilizumab may contribute to the management of Takayasu arteritis.
SUMMARY
An impressive number of studies have addressed aortitis in recent years. However, there still is a lack of robust data on causes, monitoring disease activity by imaging and biomarkers, and drugs providing steroid-free remission in noninfectious aortitis.
Topics: Antibodies, Monoclonal, Humanized; Aorta; Aortitis; Female; Follow-Up Studies; Giant Cell Arteritis; Humans; Immunoglobulin G4-Related Disease; Immunosuppressive Agents; Janus Kinases; Leflunomide; Male; Positron Emission Tomography Computed Tomography; Takayasu Arteritis; Ultrasonography
PubMed: 33186247
DOI: 10.1097/BOR.0000000000000762 -
Rheumatic Diseases Clinics of North... Aug 2023The finding of aortitis, often incidentally noted on surgical resection, should prompt evaluation for secondary causes including large-vessel vasculitis. In a large... (Review)
Review
The finding of aortitis, often incidentally noted on surgical resection, should prompt evaluation for secondary causes including large-vessel vasculitis. In a large proportion of cases, no other inflammatory cause is identified and the diagnosis of clinically isolated aortitis is made. It is unknown whether this entity represents a more localized form of large-vessel vasculitis. The need for immunosuppressive therapy in patients with clinically isolated aortitis remains unclear. Patients with clinically isolated aortitis warrant imaging of the entire aorta at baseline and regular intervals because a significant proportion of patients have or develop abnormalities in other vascular beds.
Topics: Humans; Aortitis; Aorta; Diagnostic Imaging
PubMed: 37331731
DOI: 10.1016/j.rdc.2023.03.013 -
Ugeskrift For Laeger Aug 2023In this case report, we present a 70-year-old male who was brought to our hospital with signs of upper gastrointestinal bleeding. The patient was diagnosed with aortitis...
In this case report, we present a 70-year-old male who was brought to our hospital with signs of upper gastrointestinal bleeding. The patient was diagnosed with aortitis two and a half months prior. We suspected upper gastrointestinal bleeding, and the patient was taken to the operating room for an acute endoscopy, which showed blood in the oesophagus, ventricle, and duodenum, but no bleeding source. CT angiography showed erosion of aortic aneurism, at the site of known aortitis, with bleeding into the lung and pleura. The patient was transported to the nearest university hospital for thoracic endovascular repair and survived.
Topics: Male; Humans; Aged; Hematemesis; Hemoptysis; Aortitis; Gastrointestinal Hemorrhage; Aortic Aneurysm; Hospitals, University
PubMed: 37767877
DOI: No ID Found