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Cureus May 2024This case report details the diagnostic challenge and management of an 88-year-old man who presented to a rural Japanese community hospital with sepsis-like symptoms,...
This case report details the diagnostic challenge and management of an 88-year-old man who presented to a rural Japanese community hospital with sepsis-like symptoms, initially suspected of acute bacterial cholangitis based on his physical and laboratory findings. Despite the antibiotic treatment of tazobactam and piperacillin, the patient's symptoms persisted, leading to further investigations that revealed no signs of infection but notable aortic arch wall thickening on contrast-enhanced computed tomography scans. These findings, combined with the patient's clinical presentation and lack of antibiotic response, redirected the diagnosis toward giant cell arteritis (GCA). The administration of prednisolone of 60 mg daily significantly alleviated symptoms and prevented potential severe complications such as blindness and irreversible neurological damage. This case underscores the importance of considering GCA in elderly patients presenting with systemic inflammatory symptoms and the necessity of timely intervention. It also highlights the challenges in managing high-dose steroid therapy in elderly patients and suggests the potential benefits of integrating immunosuppressants to reduce steroid dependency. This report emphasizes the need for heightened awareness and a comprehensive diagnostic approach in atypical presentations of GCA, particularly in geriatric populations within resource-limited healthcare settings.
PubMed: 38915961
DOI: 10.7759/cureus.61074 -
Current Neurology and Neuroscience... Jun 2024To briefly review the latest updates in management in giant cell arteritis, an autoimmune vasculitis affecting the medium to large vessels. (Review)
Review
PURPOSE OF THE REVIEW
To briefly review the latest updates in management in giant cell arteritis, an autoimmune vasculitis affecting the medium to large vessels.
RECENT FINDINGS
Here, we review the known and newer trends in management of giant cell arteritis. While high dose glucocorticoids remain the mainstay of therapy, immunosuppressive medications are increasingly utilized to reduce the burden and risk of long-term glucocorticoid use. Published guidelines by the American College of Rheumatology (ACR) and European League Against Rheumatism (EULAR) suggest early use of steroid-sparing immunosuppressive medications in patients with recently diagnosed or relapsing giant cell arteritis. Immunosuppressive medications include oral small molecules such as methotrexate and leflunomide and biologics, including the recently Federal Drug Administration (FDA) approved tocilizumab. Glucocorticoids remain the cornerstone of management for newly diagnosed disease but with the increasing use of medications such as IL-6 inhibitors, patients are decreasing steroid use within weeks, thereby limiting risks associated with long-term steroid use.
PubMed: 38913278
DOI: 10.1007/s11910-024-01348-9 -
European Journal of Internal Medicine Jun 2024Aortitis in Giant Cell Arteritis (GCA-aortitis) is a frequent complication that may lead to aneurysms. Tocilizumab (TCZ) was approved in GCA, but the efficacy in...
OBJECTIVE
Aortitis in Giant Cell Arteritis (GCA-aortitis) is a frequent complication that may lead to aneurysms. Tocilizumab (TCZ) was approved in GCA, but the efficacy in GCA-aortitis and aneurysms has not been analyzed to date. Our aim was to assess the effectiveness and safety of TCZ in a wide series of GCA-aortitis and aneurysms.
METHODS
Multicentre observational study with GCA-aortitis treated with TCZ. GCA was diagnosed by: a) ACR criteria, b) temporal artery biopsy, and/or c) imaging techniques. Aortitis was diagnosed mainly by PET/CT. Main outcomes were EULAR and imaging remission. Others were clinical remission, analytical normalization, corticosteroid-sparing effect, and the prevention and improvement of aneurysms.
RESULTS
196 patients with GCA-aortitis treated with TCZ. After 6 months, 72.2% reached EULAR remission but only 12% an imaging remission; increasing up-to 81.4% and 31.8%, respectively, at 24 months. A rapid clinical remission, ESR and CRP normalization was observed in 47.4%, 84.3% and 55.6%, at 1 month, increasing to 89.6%, 85.3% and 80.3% at 24 months, respectively. Aneurysms were present in 10 (5%) patients. Five of them required early surgery, while 3 others enlarged. No patient on TCZ therapy developed aneurysms during follow-up.
CONCLUSION
In patients with GCA-aortitis treated with TCZ, a rapid and maintained clinical and analytical improvement was observed. However, there was an uncoupling between clinical and EULAR remission with imaging remission.
PubMed: 38908981
DOI: 10.1016/j.ejim.2024.06.013 -
European Journal of Dermatology : EJD Apr 2024
Topics: Humans; Temporal Arteries; Diagnosis, Differential; Arteritis; Male; Female; Immunoglobulin G4-Related Disease; Aged; Immunoglobulin G; Giant Cell Arteritis; Middle Aged
PubMed: 38907551
DOI: 10.1684/ejd.2024.4647 -
AJNR. American Journal of Neuroradiology Jun 2024Giant cell arteritis (GCA) is the most common primary large vessel systemic vasculitis in the western world. Even though the involvement of scalp and intracranial... (Review)
Review
Giant cell arteritis (GCA) is the most common primary large vessel systemic vasculitis in the western world. Even though the involvement of scalp and intracranial vessels has received much attention in the neuroradiology literature, GCA, being a systemic vasculitis can involve multiple other larger vessels including aorta and its major head and neck branches. Herein, the authors present a pictorial review of the various cranial, extracranial and orbital manifestations of GCA. An increased awareness of this entity may help with timely and accurate diagnosis, helping expedite therapy and preventing serious complications.ABBREVIATIONS: ACR= American College of Rheumatology, AION= Anterior Ischemic Optic Neuropathy, EULAR= European League Against Rheumatism, GCA= Giant Cell Arteritis, LV-GCA= Large vessel GCA, PMR= Polymyalgia Rheumatica, US= Ultrasound, VWI= Vessel Wall Imaging.
PubMed: 38906672
DOI: 10.3174/ajnr.A8388 -
Cureus May 2024We discuss a perplexing case of a 51-year-old female with a history of asthma and morbid obesity, presenting with acute bilateral vision loss of unknown etiology. The...
We discuss a perplexing case of a 51-year-old female with a history of asthma and morbid obesity, presenting with acute bilateral vision loss of unknown etiology. The patient's clinical course was marked by a constellation of symptoms, including blurry vision, eyeball pain, photophobia, headache, nausea, and dizziness, prompting a multidisciplinary approach for diagnostic evaluation. Despite a comprehensive workup and a temporal artery biopsy ruling out large vessel arteritis, the etiology of vision loss remained elusive until myelin oligodendrocyte glycoprotein (MOG) antibody testing returned positive, implicating myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). High-dose corticosteroid therapy was initiated. However, the patient had worsening visual symptoms and was started on plasmapheresis and subsequent administration of Rituximab to prevent relapses, along with a long-term steroid taper regimen. This case underscores the diagnostic challenge of optic neuritis, particularly in MOGAD. It emphasizes the importance of a thorough evaluation and multidisciplinary collaboration.
PubMed: 38903369
DOI: 10.7759/cureus.60612 -
BMJ Case Reports Jun 2024Takayasu arteritis is an inflammatory disease of unknown aetiology affecting large vessels. Medium vessel involvement is also well documented; however, neuropathy as a...
Takayasu arteritis is an inflammatory disease of unknown aetiology affecting large vessels. Medium vessel involvement is also well documented; however, neuropathy as a presenting manifestation is rare. In this case report, a young woman in her 20s presented with an 8-month history of intermittent claudication in the right upper limb progressing to rest pain with allodynia in C5-C8 distribution and painless right axillary mass. On examination, she had absent pulses in the right radial, brachial and subclavian artery with audible bruit in the right subclavian and abdominal aorta. CT angiogram showed features suggestive of Takayasu arteritis with a partially thrombosed aneurysm arising from the right axillary artery leading to compression of the right brachial plexus. This patient received treatment with methotrexate and oral corticosteroids. At 3 months follow-up, there was a reduction in the size of the aneurysm, resolution of compressive symptoms and normalisation of inflammatory markers.
Topics: Humans; Takayasu Arteritis; Female; Axillary Artery; Aneurysm; Brachial Plexus Neuropathies; Adult; Computed Tomography Angiography; Methotrexate
PubMed: 38901855
DOI: 10.1136/bcr-2024-260086 -
Eye (London, England) Jun 2024Treatment of giant cell arteritis (GCA) aims initially to prevent acute visual loss, and subsequently to optimise long-term quality of life. Initial prevention of acute... (Review)
Review
Treatment of giant cell arteritis (GCA) aims initially to prevent acute visual loss, and subsequently to optimise long-term quality of life. Initial prevention of acute visual loss in GCA is well-standardised with high-dose glucocorticoid therapy but in the longer term optimising quality of life requires tailoring of treatment to the individual. The licensing of the IL-6 receptor inhibitor tocilizumab combined with advances in vascular imaging have resulted in many changes to diagnostic and therapeutic practice. Firstly, GCA is a systemic disease that may involve multiple vascular territories and present in diverse ways. Broadening of the "spectrum" of what is called GCA has been crystallised in the 2022 GCA classification criteria. Secondly, the vascular inflammation of GCA frequently co-exists with the extracapsular musculoskeletal inflammation of the related disease, polymyalgia rheumatica (PMR). Thirdly, GCA care must often be delivered across multiple specialities and healthcare organisations requiring effective interprofessional communication. Fourthly, both GCA and PMR may follow a chronic or multiphasic disease course; long-term management must be tailored to the individual patient's needs. In this article we focus on some areas of current rheumatology practice that ophthalmologists need to be aware of, including comprehensive assessment of extra-ocular symptoms, physical signs and laboratory markers; advanced imaging techniques; and implications for multi-speciality collaboration.
PubMed: 38898105
DOI: 10.1038/s41433-024-03153-7 -
Journal of Veterinary Internal Medicine Jun 2024Traditionally, 6-month courses of prednisolone are used to treat steroid-responsive meningitis-arteritis (SRMA), but this medication is associated with adverse effects...
BACKGROUND
Traditionally, 6-month courses of prednisolone are used to treat steroid-responsive meningitis-arteritis (SRMA), but this medication is associated with adverse effects that can lead to poor quality of life.
HYPOTHESIS/OBJECTIVES
Resolution of clinical signs and rate of relapse of SRMA would not be significantly different between a 6-month prednisolone protocol and a 6-week protocol.
ANIMALS
Forty-four hospital cases from multiple referral centers in the United Kingdom (2015-2019). Twenty of 44 were treated with the 6-month protocol and 24/44 with the 6-week protocol.
METHODS
Prospective, randomized trial with 12-month follow-up. The same prednisolone protocol reinitiated in the event of relapse. Analysis of relapses with binary logistic and Poisson regression modeling.
RESULTS
All cases responded to their treatment protocol. Relapses occurred in 6/20 (30%) of the 6-month protocol and 9/24 (38%) of the 6-week protocol. There was no statistical difference in the incidence risk of at least 1 relapse between the 2 groups (odds ratio = 1.40; 95% confidence interval [CI], 0.40-4.96, P = 0.60). Among the 15 dogs that relapsed, 10/15 (67%) relapsed once, 3/15 (20%) relapsed twice, and 2/15 (13%) relapsed 3 times. No statistical difference was detected in the incidence rate ratio (IRR) of total relapse events between the 2 groups (IRR = 1.46; 95% CI, 0.61-3.48; P = 0.40).
CONCLUSIONS AND CLINICAL IMPORTANCE
"Short" 6-week prednisolone protocols could be used to treat SRMA, thereby presumably reducing the duration and severity of prednisolone's adverse effects.
PubMed: 38895927
DOI: 10.1111/jvim.17130 -
The Lancet. Rheumatology Jun 2024
PubMed: 38885664
DOI: 10.1016/S2665-9913(24)00123-1