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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 -
Rheumatic Diseases Clinics of North... Aug 2023Prolonged glucocorticoid tapers have been the standard of care for giant cell arteritis (GCA) and polymyalgia rheumatica (PMR), but recent advancements have improved... (Review)
Review
Prolonged glucocorticoid tapers have been the standard of care for giant cell arteritis (GCA) and polymyalgia rheumatica (PMR), but recent advancements have improved outcomes for patients with GCA while reducing glucocorticoid-related toxicities. Many patients with GCA and PMR still experience persistent or relapsing disease, and cumulative exposure to glucocorticoids for both diseases remains high. The objective of this review is to define current treatment approaches as well as new therapeutic targets and strategies. Studies investigating inhibition of cytokine pathways, including interleukin-6, interleukin-17, interleukin-23, granulocyte-macrophage colony-stimulating factor, Janus kinase-signal transduction and activator of transcription, and others, will be reviewed.
Topics: Humans; Polymyalgia Rheumatica; Giant Cell Arteritis; Glucocorticoids
PubMed: 37331730
DOI: 10.1016/j.rdc.2023.03.005 -
Lancet (London, England) Oct 2023Polymyalgia rheumatica is an inflammatory disease producing pain and stiffness, mainly in the shoulders and pelvic girdle, in people older than 50 years. Elevation of... (Review)
Review
Polymyalgia rheumatica is an inflammatory disease producing pain and stiffness, mainly in the shoulders and pelvic girdle, in people older than 50 years. Elevation of acute phase reactants is common due to the inflammatory nature of the disease. Since there are no specific diagnostic tests, diagnosis requires the exclusion of other diseases with similar presentations. Imaging has helped to identify the pathological substrate of polymyalgia rheumatica and it is increasingly used to support clinical diagnosis or to detect coexistent giant cell arteritis. Although polymyalgia rheumatica does not clearly impair survival or organ function, it can have a detrimental effect on quality of life. Glucocorticoids at 12·5-25·0 mg prednisone per day are effective in inducing remission in most individuals but, when tapered, relapses occur in 40-60% of those affected and side-effects are common. Assessment of disease activity can be difficult because pain related to common comorbidities such as osteoarthritis and tendinopathies, can return when glucocorticoids are reduced, and acute phase reactants are increased less during flares in individuals undergoing treatment or might increase for other reasons. The role of imaging in assessing disease activity is not yet completely defined. In the search for more efficient and safer therapies, tocilizumab and sarilumab have shown efficacy in randomised controlled trials and additional targeted therapies are emerging. However, judicious risk-benefit balance is essential in applying therapeutic innovations to people with polymyalgia rheumatica.
Topics: Humans; Polymyalgia Rheumatica; Giant Cell Arteritis; Quality of Life; Diagnosis, Differential; Glucocorticoids; Pain; Acute-Phase Proteins
PubMed: 37832573
DOI: 10.1016/S0140-6736(23)01310-7 -
International Journal of Molecular... Nov 2023Polyarteritis nodosa (PAN), also known as panarteritis nodosa, represents a form of necrotizing vasculitis that predominantly affects medium-sized vessels, although it... (Review)
Review
Polyarteritis nodosa (PAN), also known as panarteritis nodosa, represents a form of necrotizing vasculitis that predominantly affects medium-sized vessels, although it is not restricted to them and can also involve smaller vessels. The clinical presentation is heterogeneous and characterized by a significant number of patients exhibiting general symptoms, including asthenia, fever, and unintended weight loss. Although PAN can involve virtually any organ, it preferentially affects the skin, nervous system, and the gastrointestinal tract. Orchitis is a rare but specific manifestation of PAN. The absence of granulomas, glomerulonephritis, and anti-neutrophil cytoplasmic antibodies serves to distinguish PAN from other types of vasculitis. Major complications consist of hemorrhagic and thrombotic events occurring in mesenteric, cardiac, cerebral, and renal systems. Historically, PAN was frequently linked to hepatitis B virus (HBV) infection, but this association has dramatically changed in recent years due to declining HBV prevalence. Current epidemiological research often identifies a connection between PAN and genetic syndromes as well as neoplasia. This article provides a comprehensive review of PAN, specifically focusing on the progression of its clinical manifestations over time.
Topics: Male; Humans; Polyarteritis Nodosa; Vasculitis; Hepatitis B; Hepatitis B virus; Gastrointestinal Tract
PubMed: 38068989
DOI: 10.3390/ijms242316668 -
Nature Reviews. Rheumatology Jul 2023Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are closely related conditions characterized by systemic inflammation, a predominant IL-6 signature,... (Review)
Review
Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are closely related conditions characterized by systemic inflammation, a predominant IL-6 signature, an excellent response to glucocorticoids, a tendency to a chronic and relapsing course, and older age of the affected population. This Review highlights the emerging view that these diseases should be approached as linked conditions, unified under the term GCA-PMR spectrum disease (GPSD). In addition, GCA and PMR should be seen as non-monolithic conditions, with different risks of developing acute ischaemic complications and chronic vascular and tissue damage, different responses to available therapies and disparate relapse rates. A comprehensive stratification strategy for GPSD, guided by clinical findings, imaging and laboratory data, facilitates appropriate therapy and cost-effective use of health-economic resources. Patients presenting with predominant cranial symptoms and vascular involvement, who usually have a borderline elevation of inflammatory markers, are at an increased risk of sight loss in early disease but have fewer relapses in the long term, whereas the opposite is observed in patients with predominant large-vessel vasculitis. How the involvement of peripheral joint structures affects disease outcomes remains uncertain and understudied. In the future, all cases of new-onset GPSD should undergo early disease stratification, with their management adapted accordingly.
Topics: Humans; Giant Cell Arteritis; Polymyalgia Rheumatica; Glucocorticoids; Diagnostic Imaging
PubMed: 37308659
DOI: 10.1038/s41584-023-00976-8 -
Annals of the Rheumatic Diseases May 2024To update the EULAR recommendations for the use of imaging modalities in primary large vessel vasculitis (LVV).
OBJECTIVES
To update the EULAR recommendations for the use of imaging modalities in primary large vessel vasculitis (LVV).
METHODS
A systematic literature review update was performed to retrieve new evidence on ultrasound, MRI, CT and [F]-fluorodeoxyglucose positron emission tomography (FDG-PET) for diagnosis, monitoring and outcome prediction in LVV. The task force consisted of 24 physicians, health professionals and patients from 14 countries. The recommendations were updated based on evidence and expert opinion, iterating until voting indicated consensus. The level of agreement was determined by anonymous votes.
RESULTS
Three overarching principles and eight recommendations were agreed. Compared to the 2018 version, ultrasound is now recommended as first-line imaging test in all patients with suspected giant cell arteritis, and axillary arteries should be included in the standard examination. As an alternative to ultrasound, cranial and extracranial arteries can be examined by FDG-PET or MRI. For Takayasu arteritis, MRI is the preferred imaging modality; FDG-PET, CT or ultrasound are alternatives. Although imaging is not routinely recommended for follow-up, ultrasound, FDG-PET or MRI may be used for assessing vessel abnormalities in LVV patients with suspected relapse, particularly when laboratory markers of inflammation are unreliable. MR-angiography, CT-angiography or ultrasound may be used for long-term monitoring of structural damage, particularly at sites of preceding vascular inflammation.
CONCLUSIONS
The 2023 EULAR recommendations provide up-to-date guidance for the role of imaging in the diagnosis and assessment of patients with LVV.
Topics: Humans; Giant Cell Arteritis; Takayasu Arteritis; Ultrasonography; Magnetic Resonance Imaging; Fluorodeoxyglucose F18; Positron-Emission Tomography; Vasculitis; Tomography, X-Ray Computed; Axillary Artery
PubMed: 37550004
DOI: 10.1136/ard-2023-224543 -
Ugeskrift For Laeger Nov 2023Cranial and large vessel giant cell arteritis is a systemic vasculitis frequently found in Denmark. In recent years, considerable new knowledge has been gained in the... (Review)
Review
Cranial and large vessel giant cell arteritis is a systemic vasculitis frequently found in Denmark. In recent years, considerable new knowledge has been gained in the field of diagnostics and treatment of giant cell arteritis. In this review, the clinical and radiological findings, treatment options and effect and side effects of treatment with tocilizumab are reviewed in a Danish context.
Topics: Humans; Giant Cell Arteritis
PubMed: 37987451
DOI: No ID Found -
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 -
Current Opinion in Allergy and Clinical... Aug 2023Giant cell arteritis (GCA) is an idiopathic and persistent condition characterized by granulomatous vasculitis of the medium and large vessels with overlapping... (Review)
Review
PURPOSE OF REVIEW
Giant cell arteritis (GCA) is an idiopathic and persistent condition characterized by granulomatous vasculitis of the medium and large vessels with overlapping phenotypes, including conventional cranial arteritis and extra-cranial GCA, also known as large-vessel GCA. Vascular problems linked with large vessel involvement may partly be caused by delayed diagnosis, emphasizing the necessity of early detection and the fast beginning of appropriate therapy. Glucocorticoids are the cornerstone of treatment for GCA, but using them for an extended period has numerous, often severe, side effects.
RECENT FINDINGS
clinical practice and novel discoveries on the pathogenic pathways suggest that steroid-free biologic treatments may be efficient and safe for GCA patients.
SUMMARY
since now, only Tocilizumab is approved for GCA treatment, but several drugs are currently used, and ongoing trials could give both researchers and patients novel therapeutic strategies for induction, maintenance, and prevention of relapse of GCA. The aims of this work is to synthesize evidence from current studies present in scientific literature about innovative treatment of Giant cell artheritis.
Topics: Humans; Giant Cell Arteritis; Glucocorticoids
PubMed: 37357797
DOI: 10.1097/ACI.0000000000000923 -
Rheumatic Diseases Clinics of North... Aug 2023Predictions for a general path forward in vasculitis care and research are provided based on advances made in the past 20 years. Prospects for advances in translational... (Review)
Review
Predictions for a general path forward in vasculitis care and research are provided based on advances made in the past 20 years. Prospects for advances in translational research with potential to improve care are highlighted, including identification of hemato-inflammatory diseases, autoantigens, disease mechanisms in animal models, and biomarkers. A list of active randomized trials is provided, and areas of potential paradigm shifts in care are highlighted. The importance of patient involvement and international collaboration is noted, and a plea is made for innovative trial designs that would improve access of patients to trials and to clinical experts at referral centers.
Topics: Animals; Humans; Takayasu Arteritis; Biomarkers; Forecasting; Giant Cell Arteritis
PubMed: 37331742
DOI: 10.1016/j.rdc.2023.03.014