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RMD Open Aug 2023To update the evidence on imaging for diagnosis, monitoring and outcome prediction in large vessel vasculitis (LVV) to inform the 2023 update of the European Alliance of... (Meta-Analysis)
Meta-Analysis
Imaging in diagnosis, monitoring and outcome prediction of large vessel vasculitis: a systematic literature review and meta-analysis informing the 2023 update of the EULAR recommendations.
OBJECTIVES
To update the evidence on imaging for diagnosis, monitoring and outcome prediction in large vessel vasculitis (LVV) to inform the 2023 update of the European Alliance of Associations for Rheumatology recommendations on imaging in LVV.
METHODS
Systematic literature review (SLR) (2017-2022) including prospective cohort and cross-sectional studies (>20 participants) on diagnostic, monitoring, outcome prediction and technical aspects of LVV imaging. Diagnostic accuracy data were meta-analysed in combination with data from an earlier (2017) SLR.
RESULTS
The update retrieved 38 studies, giving a total of 81 studies when combined with the 2017 SLR. For giant cell arteritis (GCA), and taking clinical diagnosis as a reference standard, low risk of bias (RoB) studies yielded pooled sensitivities and specificities (95% CI) of 88% (82% to 92%) and 96% (95% CI 86% to 99%) for ultrasound (n=8 studies), 81% (95% CI 71% to 89%) and 98% (95% CI 89% to 100%) for MRI (n=3) and 76% (95% CI 67% to 83%) and 95% (95% CI 71% to 99%) for fluorodeoxyglucose positron emission tomography (FDG-PET, n=4), respectively. Compared with studies assessing cranial arteries only, low RoB studies with ultrasound assessing both cranial and extracranial arteries revealed a higher sensitivity (93% (95% CI 88% to 96%) vs 80% (95% CI 71% to 87%)) with comparable specificity (94% (95% CI 83% to 98%) vs 97% (95% CI 71% to 100%)). No new studies on diagnostic imaging for Takayasu arteritis (TAK) were found. Some monitoring studies in GCA or TAK reported associations of imaging with clinical signs of inflammation. No evidence was found to determine whether imaging severity might predict worse clinical outcomes.
CONCLUSION
Ultrasound, MRI and FDG-PET revealed a good performance for the diagnosis of GCA. Cranial and extracranial vascular ultrasound had a higher pooled sensitivity with similar specificity compared with limited cranial ultrasound.
Topics: Humans; Cross-Sectional Studies; Fluorodeoxyglucose F18; Prospective Studies; Giant Cell Arteritis; Positron-Emission Tomography
PubMed: 37620113
DOI: 10.1136/rmdopen-2023-003379 -
The Lancet. Rheumatology Jun 2024Giant cell arteritis is the principal form of systemic vasculitis affecting people over 50. Large-vessel involvement, termed large vessel giant cell arteritis, mainly... (Review)
Review
Giant cell arteritis is the principal form of systemic vasculitis affecting people over 50. Large-vessel involvement, termed large vessel giant cell arteritis, mainly affects the aorta and its branches, often occurring alongside cranial giant cell arteritis, but large vessel giant cell arteritis without cranial giant cell arteritis can also occur. Patients mostly present with constitutional symptoms, with localising large vessel giant cell arteritis symptoms present in a minority of patients only. Large vessel giant cell arteritis is usually overlooked until clinicians seek to exclude it with imaging by ultrasonography, magnetic resonance angiography (MRA), computed tomography angiography (CTA), or [F]fluorodeoxyglucose-PET-CT. Although the role of imaging in treatment monitoring remains uncertain, imaging by MRA or CTA is crucial for identifying aortic aneurysm formation during patient follow up. In this Series paper, we define the large vessel subset of giant cell arteritis and summarise its clinical challenges. Furthermore, we identify areas for future research regarding the management of large vessel giant cell arteritis.
Topics: Giant Cell Arteritis; Humans; Positron Emission Tomography Computed Tomography; Magnetic Resonance Angiography; Computed Tomography Angiography
PubMed: 38574745
DOI: 10.1016/S2665-9913(23)00300-4 -
Annals of the Rheumatic Diseases Jan 2024To develop treat-to-target (T2T) recommendations in giant cell arteritis (GCA) and polymyalgia rheumatica (PMR).
OBJECTIVES
To develop treat-to-target (T2T) recommendations in giant cell arteritis (GCA) and polymyalgia rheumatica (PMR).
METHODS
A systematic literature review was conducted to retrieve data on treatment targets and outcomes in GCA/PMR as well as to identify the evidence for the effectiveness of a T2T-based management approach in these diseases. Based on evidence and expert opinion, the task force (29 participants from 10 countries consisting of physicians, a healthcare professional and a patient) developed recommendations, with consensus obtained through voting. The final level of agreement was provided anonymously.
RESULTS
Five overarching principles and six-specific recommendations were formulated. Management of GCA and PMR should be based on shared decisions between patient and physician recognising the need for urgent treatment of GCA to avoid ischaemic complications, and it should aim at maximising health-related quality of life in both diseases. The treatment targets are achievement and maintenance of remission, as well as prevention of tissue ischaemia and vascular damage. Comorbidities need to be considered when assessing disease activity and selecting treatment.
CONCLUSION
These are the first T2T recommendations for GCA and PMR. Treatment targets, as well as strategies to assess, achieve and maintain these targets have been defined. The research agenda highlights the gaps in evidence and the need for future research.
Topics: Humans; Giant Cell Arteritis; Polymyalgia Rheumatica; Quality of Life; Comorbidity
PubMed: 36828585
DOI: 10.1136/ard-2022-223429 -
Annual Review of Medicine Jan 2024Giant cell arteritis (GCA) and Takayasu arteritis (TAK) are large-vessel vasculitides affecting the aorta and its branches. Arterial damage from these diseases may... (Review)
Review
Giant cell arteritis (GCA) and Takayasu arteritis (TAK) are large-vessel vasculitides affecting the aorta and its branches. Arterial damage from these diseases may result in ischemic complications, aneurysms, and dissections. Despite their similarities, the management of GCA and TAK differs. Glucocorticoids are used frequently but relapses are common, and glucocorticoid toxicity contributes to significant morbidity. Conventional immunosuppressive therapies can be beneficial in TAK, though their role in the management of GCA remains unclear. Tumor necrosis factor inhibitors improve remission rates and appear to limit vascular damage in TAK; these agents are not beneficial in GCA. Tocilizumab is the first biologic glucocorticoid-sparing agent approved for use in GCA and also appears to be effective in TAK. A better understanding of the pathogenesis of both conditions and the availability of targeted therapies hold much promise for future management.
Topics: Humans; Glucocorticoids; Giant Cell Arteritis; Takayasu Arteritis
PubMed: 37683286
DOI: 10.1146/annurev-med-060622-100940 -
Mayo Clinic Proceedings Jul 2023
Topics: Humans; Takayasu Arteritis
PubMed: 37419571
DOI: 10.1016/j.mayocp.2023.03.001 -
The Medical Clinics of North America Sep 2023Vasculitis is a diverse group of disorders involving inflammation of the blood vessels. Approaching the diagnosis of vasculitis can be challenging, given the differing... (Review)
Review
Vasculitis is a diverse group of disorders involving inflammation of the blood vessels. Approaching the diagnosis of vasculitis can be challenging, given the differing clinical presentation and organ manifestations. Often vasculitis is a diagnosis that is considered too late, given the heterogeneous presentation and various mimics. This article aims to provide physicians with a diagnostic approach to vasculitis.
Topics: Humans; Vasculitis; Inflammation; Tomography, X-Ray Computed; Giant Cell Arteritis
PubMed: 37541712
DOI: 10.1016/j.mcna.2023.05.005 -
The Journal of Dermatology Feb 2024Autoinflammatory diseases (AIDs) characterized by recurrent episodes of localized or systemic inflammation are disorders of the innate immune system. Skin lesions are... (Review)
Review
Autoinflammatory diseases (AIDs) characterized by recurrent episodes of localized or systemic inflammation are disorders of the innate immune system. Skin lesions are commonly found in AIDs and cutaneous vasculitis can coexist with AIDs and even present as the most striking feature. This review aims to focus on the frequent cutaneous vasculitis association in three monogenic AIDs including familial Mediterranean fever (FMF), deficiency of adenosine deaminase type 2 (DADA2), and the recently identified adult-onset VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome. Cutaneous vasculitis in FMF is characterized by: (1) small-vessel vasculitis similar to IgA vasculitis with palpable purpura but increased intussusception complication and less vascular IgA deposit, and (2) cutaneous arteritis-like vasculitis presenting as subcutaneous nodules most often with higher glomerular involvement. DADA2 has a wide spectrum of clinical presentations ranging from fatal systemic vasculitis with multiple strokes, especially in pediatric patients, to limited cutaneous disease in middle-aged patients. DADA2 shares similar clinical and histopathological features with polyarteritis nodosa (PAN). As a result, DADA2 is commonly initially misdiagnosed as childhood PAN. Livedo racemosa reveals the most common cutaneous manifestation of cutaneous vasculitis in patients with DADA2. VEXAS syndrome is a life-threatening disease. A diagnosis of VEXAS syndrome should be strongly considered or could be made in patients with skin lesions characterized by Sweet syndrome-like eruption, livedo racemosa, concomitant relapsing polychondritis, deep venous thrombosis, pulmonary involvement, and progressive hematologic abnormalities such as myelodysplastic syndrome with a unique finding of cytoplasmic vacuoles in myeloid and erythroid precursor cells from bone marrow aspirate smear. As skin involvement is common in AIDs and may present as the most frequent manifestation, especially in DADA2 (70% to 90%) and VEXAS syndrome (83% to 91%), dermatologists play a crucial role in contributing to the early diagnosis of these AIDs with early initiation of the appropriate therapy to avoid progressing fatal outcomes.
Topics: Adult; Humans; Child; Middle Aged; Adenosine Deaminase; Livedo Reticularis; Intercellular Signaling Peptides and Proteins; Vasculitis; Polyarteritis Nodosa; Skin Diseases; Familial Mediterranean Fever; Mutation; Agammaglobulinemia; Myelodysplastic Syndromes; Skin Diseases, Genetic; Severe Combined Immunodeficiency
PubMed: 37955334
DOI: 10.1111/1346-8138.17030 -
Innere Medizin (Heidelberg, Germany) Feb 2024The immune-mediated small vessel vasculitis is known as Schoenlein-Henoch purpura predominantly from pediatrics and in these cases occurs more frequently after... (Review)
Review
The immune-mediated small vessel vasculitis is known as Schoenlein-Henoch purpura predominantly from pediatrics and in these cases occurs more frequently after infections of the upper airways. In adults, immunoglobulin A (IgA) vasculitis often proceeds more severely und recurrently with the classical tetrad of skin manifestations in the sense of leukocytoclastic vasculitis, joint affection, gastrointestinal involvement and IgA nephritis, in contrast to the mostly mild and self-limiting course in children. The background of this systemic vasculitis with formation of IgA immune complexes is considered to be an altered glycosylation of IgA, as this causes the exposure of binding sites for autoantibodies so that an immune complex reaction can be elicited. This ultimately leads to perivascular deposition of IgA and a further activation of neutrophils. Groundbreaking in the diagnostics is the histological detection of leukocytoclastic vasculitis and in cases of renal manifestations a kidney biopsy with characteristic deposits of immune complexes, which cannot be clearly differentiated from IgA nephropathy. The treatment is aimed at the respective manifestation and is mostly based on consensus recommendations due to the lack of randomized studies. In addition to immunosuppressive medication, in the presence of a chronic kidney disease general nephroprotection is becoming increasingly more important also by inhibition of sodium-glucose transporter 2 (SGLT2). The type and extent of kidney involvement and also rare cardiac manifestations are the main determinants of the prognosis. Continuous medical accompaniment of those affected is necessary due to the possible progression of the disease and the risk of recurrence.
Topics: Adult; Humans; Child; IgA Vasculitis; Antigen-Antibody Complex; Immunoglobulin A; Vasculitis; Polyarteritis Nodosa; Vasculitis, Leukocytoclastic, Cutaneous
PubMed: 38236411
DOI: 10.1007/s00108-023-01650-7 -
Zeitschrift Fur Rheumatologie Sep 2023IgA vasculitis (IgAV) is an immune complex-mediated vasculitis characterized by IgA1-dominant immune deposits in small vessels. It is the most common systemic vasculitis...
IgA vasculitis (IgAV) is an immune complex-mediated vasculitis characterized by IgA1-dominant immune deposits in small vessels. It is the most common systemic vasculitis in childhood with a mostly uncomplicated and self-limiting course. Adults are less affected but the course is frequently more complicated and more frequently accompanied by renal involvement. IgAV characteristically manifests itself on the skin with palpable purpura and in joints, the kidneys and the gastrointestinal tract. In cases of incomplete or atypical symptoms a differential diagnostic work-up is required. A number of triggers have been suggested, especially infections and drugs. Disease management is tailored to organ manifestations and the severity of the symptoms. For children, optimized supportive care and targeted symptom relief are usually sufficient. Management of renal and gastrointestinal manifestations follows recommendations for ANCA-associated vasculitis and IgA nephropathy. Treatment options include glucocorticoids and immunosuppressive agents with varying and mostly insufficient evidence.
Topics: Adult; Child; Humans; IgA Vasculitis; Glomerulonephritis, IGA; Immunoglobulin A; Skin; Immunosuppressive Agents; Polyarteritis Nodosa; Giant Cell Arteritis; Granulomatosis with Polyangiitis
PubMed: 37266676
DOI: 10.1007/s00393-023-01355-0 -
Ugeskrift For Laeger Dec 2023In Denmark, around 4,500 people are diagnosed with colorectal cancer (CRC) annually. This review investigates that while the efficacy of immunotherapy in CRC is still... (Review)
Review
In Denmark, around 4,500 people are diagnosed with colorectal cancer (CRC) annually. This review investigates that while the efficacy of immunotherapy in CRC is still being studied, immunotherapy is currently only indicated in the treatment of mismatch-repair deficient (dMMR) metastatic CRC, which accounts for 10-15% of patients. Recent studies indicate high rates of pathologic response in dMMR CRC treated with pre-operative immunotherapy while large-scale studies on novel immunotherapy combinations are ongoing.
Topics: Humans; Ischemic Stroke; Takayasu Arteritis; Colorectal Neoplasms; Colonic Neoplasms; Immunotherapy
PubMed: 38105737
DOI: No ID Found