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Nature Reviews. Disease Primers Jan 2022Large-vessel vasculitis (LVV) manifests as inflammation of the aorta and its major branches and is the most common primary vasculitis in adults. LVV comprises two... (Review)
Review
Large-vessel vasculitis (LVV) manifests as inflammation of the aorta and its major branches and is the most common primary vasculitis in adults. LVV comprises two distinct conditions, giant cell arteritis and Takayasu arteritis, although the phenotypic spectrum of primary LVV is complex. Non-specific symptoms often predominate and so patients with LVV present to a range of health-care providers and settings. Rapid diagnosis, specialist referral and early treatment are key to good patient outcomes. Unfortunately, disease relapse remains common and chronic vascular complications are a source of considerable morbidity. Although accurate monitoring of disease activity is challenging, progress in vascular imaging techniques and the measurement of laboratory biomarkers may facilitate better matching of treatment intensity with disease activity. Further, advances in our understanding of disease pathophysiology have paved the way for novel biologic treatments that target important mediators of disease in both giant cell arteritis and Takayasu arteritis. This work has highlighted the substantial heterogeneity present within LVV and the importance of an individualized therapeutic approach. Future work will focus on understanding the mechanisms of persisting vascular inflammation, which will inform the development of increasingly sophisticated imaging technologies. Together, these will enable better disease prognostication, limit treatment-associated adverse effects, and facilitate targeted development and use of novel therapies.
Topics: Adult; Aorta; Giant Cell Arteritis; Humans; Takayasu Arteritis
PubMed: 34992251
DOI: 10.1038/s41572-021-00327-5 -
Journal of Internal Medicine Nov 2022Polymyalgia rheumatica (PMR) is the most common inflammatory rheumatic disease affecting people older than 50 years and is 2-3 times more common in women. The most... (Review)
Review
Polymyalgia rheumatica (PMR) is the most common inflammatory rheumatic disease affecting people older than 50 years and is 2-3 times more common in women. The most common symptoms are pain and morning stiffness in the shoulder and pelvic girdle and the onset may be acute or develop over a few days to weeks. General symptoms such as fatigue, fever and weight loss may occur, likely driven by systemic IL-6 signalling. The pathology includes synovial and periarticular inflammation and muscular vasculopathy. A new observation is that PMR may appear as a side effect of cancer treatment with checkpoint inhibitors. The diagnosis of PMR relies mainly on symptoms and signs combined with laboratory markers of inflammation. Imaging modalities including ultrasound, magnetic resonance imaging and positron emission tomography with computed tomography are promising new tools in the investigation of suspected PMR. However, they are still limited by availability, high cost and unclear performance in the diagnostic workup. Glucocorticoid (GC) therapy is effective in PMR, with most patients responding promptly to 15-25 mg prednisolone per day. There are challenges in the management of patients with PMR as relapses do occur and patients with PMR may need to stay on GC for extended periods. This is associated with high rates of GC-related comorbidities, such as diabetes and osteoporosis, and there are limited data on the use of disease-modifying antirheumatic drugs and biologics as GC sparing agents. Finally, PMR is associated with giant cell arteritis that may complicate the disease course and require more intense and prolonged treatment.
Topics: Antirheumatic Agents; Biological Products; Biomarkers; Female; Giant Cell Arteritis; Glucocorticoids; Humans; Inflammation; Interleukin-6; Polymyalgia Rheumatica; Prednisolone
PubMed: 35612524
DOI: 10.1111/joim.13525 -
Current Rheumatology Reports Aug 2020Giant cell arteritis (GCA) and Takayasu arteritis (TAK) are auto-inflammatory and autoimmune diseases with a highly selective tissue tropism for medium and large... (Review)
Review
PURPOSE OF REVIEW
Giant cell arteritis (GCA) and Takayasu arteritis (TAK) are auto-inflammatory and autoimmune diseases with a highly selective tissue tropism for medium and large arteries. In both diseases, CD4 T cells and macrophages form granulomatous lesions within the arterial wall, a tissue site normally protected by immune privilege. Vascular lesions can be accompanied by an extravascular component, typically an intense hepatic acute phase response that produces well-known laboratory abnormalities, e.g., elevated ESR and CRP. It is unclear whether GCA and TAK lie on a spectrum of disease or whether they represent fundamentally different disease processes.
RECENT FINDINGS
GCA and TAK share many clinical features, but there are substantial differences in genetics, epidemiology, disease mechanisms, response to treatment, and treatment complications that give rise to different disease trajectories. A significant difference lies in the composition of the wall-infiltrating immune cell compartment, which in TAK includes a significant population of CD8 T cells as well as natural killer cells, specifying disparate disease effector pathways mediating tissue damage and vessel wall remodeling. Despite the similarities in tissue tropism and histomorphology, GCA and TAK are two distinct vasculitides that rely on separate disease mechanisms and require disease-specific approaches in diagnosis and management.
Topics: CD8-Positive T-Lymphocytes; Diagnosis, Differential; Giant Cell Arteritis; Humans; Killer Cells, Natural; Macrophages; Takayasu Arteritis
PubMed: 32845392
DOI: 10.1007/s11926-020-00948-x -
Veterinary Journal (London, England :... 2023Steroid-responsive meningitis-arteritis (SRMA) occurs as an immune-mediated, inflammatory, and non-infectious disorder of juvenile and young-adult dogs. In principle,... (Review)
Review
Steroid-responsive meningitis-arteritis (SRMA) occurs as an immune-mediated, inflammatory, and non-infectious disorder of juvenile and young-adult dogs. In principle, SRMA is divided into two clinical courses: during the typical acute form, dogs are presented with fever, cervical hyperaesthesia, and reluctance to move. The more protracted form most probably emerges after insufficient immunosuppressive treatment or relapses, with additional neurologic deficits localized in the cervical and thoracolumbar spinal cord or multifocally. The trigger leading to SRMA still remains an unsolved riddle for immunologists and clinical neurologists. In the past, many attempts have been made to clarify the etiology of this disease without success. The purpose of writing this narrative review about SRMA is to summarize new insights on the pathogenesis of SRMA with a focus on immunologic dysregulation. Furthermore, unusual manifestations of the disease, new diagnostic approaches using possible laboratory biomarkers or diagnostic imaging tools, and potential innovative treatment strategies are discussed.
Topics: Animals; Dogs; Meningitis; Arteritis; Biomarkers; Steroids; Dog Diseases
PubMed: 37704169
DOI: 10.1016/j.tvjl.2023.106030 -
Autoimmunity Reviews Jun 2021The primary vasculitides constitute a heterogeneous group of immune mediated diseases of incompletely understood pathogenesis currently classified by the size of blood... (Review)
Review
The primary vasculitides constitute a heterogeneous group of immune mediated diseases of incompletely understood pathogenesis currently classified by the size of blood vessels affected (Chapel Hill classification). In recent years, several drugs with well-characterized immunological targets have been tested in clinical trials in large vessel vasculitis and small vessel vasculitis. Such trials provide "reverse translational" or bedside to bench information about underlying pathogenic mechanisms. Therefore, the aim of this systematic literature review was to examine the evidence base for a more refined mechanistic immunological classification of vasculitis. A total of 40 studies (20 randomized controlled trials (RCTs), 16 prospective studies, 1 retrospective cohort study and 3 case series) were included for full qualitative assessment. RCTs concerning biologic therapy for large vessel vasculitis mainly supports interleukin 6 receptor inhibition (tocilizumab). RCTs concerning biologic therapy for granulomatosis with polyangiitis and microscopic polyangiitis mainly support anti-CD20 treatment (rituximab) and complement inhibition with a small molecule C5a receptor antagonist (avacopan) is an emerging treatment option. The biologic treatment of eosinophilic granulomatosis with polyangiitis is centered around interleukin 5 inhibition (mepolizumab). Studies on tumor necrosis factor alpha inhibition (adalimumab, infliximab, and etanercept) showed negative results in giant cell arteritis but some effect in Takayasu arteritis. Taken together, clinical studies with cytokine and cell specific drugs are dissecting the heterogeneous immunopathogenic mechanisms of vasculitis and support a mechanistic immunological classification. Especially, cytokine antagonism is pointing towards immunological distinctions between eosinophilic granulomatosis with polyangiitis and granulomatosis with polyangiitis/microscopic polyangiitis and differences between giant cell arteritis and Takayasu arteritis.
Topics: Churg-Strauss Syndrome; Etanercept; Giant Cell Arteritis; Granulomatosis with Polyangiitis; Humans; Microscopic Polyangiitis; Randomized Controlled Trials as Topic; Rituximab; Takayasu Arteritis
PubMed: 33872767
DOI: 10.1016/j.autrev.2021.102829 -
Clinical & Experimental Optometry Sep 2020Temporal arteritis (TA), or giant cell arteritis, is a systemic autoimmune vasculitis affecting patients over 50 years of age. It can cause rapid, irreversible... (Review)
Review
Temporal arteritis (TA), or giant cell arteritis, is a systemic autoimmune vasculitis affecting patients over 50 years of age. It can cause rapid, irreversible bilateral vision loss in older adults and is therefore considered an ophthalmological emergency. Many of the symptoms and signs of TA can be vague, non-specific and gradual in onset, often leading to a delayed or inaccurate diagnosis. As such, it is important for a wide variety of primary optometrists and health practitioners to maintain a robust understanding of the clinical presentation, key investigations and time-sensitive management of this disease, as early initiation of treatment for TA can be vision- and life-saving.
Topics: Biopsy; Diagnosis, Differential; Disease Management; Giant Cell Arteritis; Humans; Positron Emission Tomography Computed Tomography; Temporal Arteries
PubMed: 31663193
DOI: 10.1111/cxo.12975 -
Eye (London, England) Jun 2020The landscape of the investigation and management of giant cell arteritis (GCA) is advancing. In this review we will outline the recent advances by searching the current... (Review)
Review
The landscape of the investigation and management of giant cell arteritis (GCA) is advancing. In this review we will outline the recent advances by searching the current English literature for relevant articles using key words of giant cell arteritis, temporal arteritis, Horton's disease, investigation, and treatment. Delay in diagnosis, diagnostic uncertainty and glucocorticoid (GC) morbidity are among the highest concerns of clinicians and patients in this disease area. The positive news is that fast track pathways, imaging techniques and new therapies are emerging for routine management of GCA. Future directions for intervention in the treatment paradigm will be discussed.
Topics: Giant Cell Arteritis; Glucocorticoids; Humans
PubMed: 31582795
DOI: 10.1038/s41433-019-0608-7 -
Best Practice & Research. Clinical... Mar 2023The recent publication of the American College of Rheumatology (ACR)-European Alliance of Associations for Rheumatology (EULAR) classification criteria for large vessel... (Review)
Review
The recent publication of the American College of Rheumatology (ACR)-European Alliance of Associations for Rheumatology (EULAR) classification criteria for large vessel vasculitis and anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) has provided modern criteria for the classification of these conditions, which incorporate contemporary methods of investigation and diagnosis. Further validation is required in independent cohorts, especially from populations that were not well represented in the development cohort. Studies of the occurrence of large vessel vasculitis report that Takayasu arteritis is a rare disease in most populations, and giant cell arteritis is the most common vasculitis in older populations. The incidence of AAV appears to have plateaued, but the prevalence is increasing as a result of lower mortality. The new classification criteria may affect the reported incidence and prevalence, and studies will be needed to confirm this. The impact of COVID-19 on the occurrence of the vasculitides is not completely known, but there is evidence of reduced occurrence of Kawasaki disease and IgA-associated vasculitis following lockdowns with reduced transmission of possible trigger infectious agents.
Topics: Humans; Aged; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Giant Cell Arteritis; Takayasu Arteritis; Rheumatology
PubMed: 37468418
DOI: 10.1016/j.berh.2023.101845 -
Current Rheumatology Reports Jun 2020Vasculitides are characterized by mostly autoimmunologically induced inflammatory processes of vascular structures. They have various clinical and radiologic... (Review)
Review
PURPOSE OF REVIEW
Vasculitides are characterized by mostly autoimmunologically induced inflammatory processes of vascular structures. They have various clinical and radiologic appearances. Early diagnosis and reliable monitoring are indispensable for adequate therapy to prevent potentially serious complications. Imaging, in addition to laboratory tests and physical examination, constitutes a key component in assessing disease extent and activity. This review presents current standards and some typical findings in the context of imaging in vasculitis with particular attention to large vessel vasculitides.
RECENT FINDINGS
Recently, imaging has gained importance in the management of vasculitis, especially regarding large vessel vasculitides (LVV). Recently, EULAR (European League Against Rheumatism) has launched its recommendations concerning the diagnosis of LVVs. Imaging is recommended as the preferred complement to clinical examination. Color-coded duplex sonography is considered the first choice imaging test in suspected giant cell arteritis, and magnetic resonance imaging is considered the first choice in suspected Takayasu's arteritis. Due to diversity of clinical and radiologic presentations, diagnosis and therapy monitoring of vasculitides may constitute a challenge. As a result of ongoing technological progress, a variety of non-invasive imaging modalities now play an elemental role in the interdisciplinary management of vasculitic diseases.
Topics: Giant Cell Arteritis; Humans; Magnetic Resonance Imaging; Takayasu Arteritis; Vasculitis
PubMed: 32562073
DOI: 10.1007/s11926-020-00915-6 -
Deutsches Arzteblatt International Jun 2022Polymyalgia rheumatica (PMR) is among the most common inflammatory rheumatic diseases in older adults. Presumed risk factors include female sex, previous infections, and... (Review)
Review
BACKGROUND
Polymyalgia rheumatica (PMR) is among the most common inflammatory rheumatic diseases in older adults. Presumed risk factors include female sex, previous infections, and genetic factors. No epidemiological data on PMR in Germany have been available until now.
METHODS
This review is based on publications retrieved by a selective literature search in PubMed. Moreover, the administrative incidence and prevalence of PMR in the years 2011-2019 was determined from data of the AOK Baden-Württemberg statutory health insurance carrier for insurees aged 40 and older. In addition, we quantified the number of consultations with physicians involved in the diagnosis.
RESULTS
The annual age- and sex-standardized incidence and prevalence of PMR from 2011 to 2019 were 18.6/100 000 persons and 138.8/100 000 persons, respectively. The incidence was higher in women than in men (21.8/100 000 vs. 12.8/100 000 persons per year). 60% of the cases were diagnosed in primary care practices. The treatment of PMR with orally administered glucocorticoids usually results in a treatment response within a few days to weeks. Approximately 43% of patients experience recurrent symptoms within a year, requiring adjustment of the glucocorticoid dose. For older patients with impaired physical ability, additional non-pharmacological treatment with exercise programs plays an important role.
CONCLUSION
PMR usually takes an uncomplicated course under treatment and can be managed in primary care, but these patients are often multimorbid and require frequent follow-up. Along with research on the etiology of the disease, further studies are needed to identify the risk factors for a chronic course and to evaluate the potential effects of non-pharmacological measures.
Topics: Adult; Aged; Female; Giant Cell Arteritis; Glucocorticoids; Humans; Incidence; Male; Middle Aged; Polymyalgia Rheumatica; Prevalence
PubMed: 35635433
DOI: 10.3238/arztebl.m2022.0218