1,721,068 research outputs found

    Giant cell arteritis presenting as a stroke in the internal carotid artery territory: a case-based review

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    Giant cell arteritis (GCA) is a large-vessel vasculitis, typically affecting the aorta and its branches. The involvement of vertebral and internal carotid arteries occurs in a limited number of cases, and stroke as a presenting symptom of GCA is extremely unusual: this subset of the disease has a poor prognosis and rarely responds to immunosuppression.We report the case of a 70-year-old woman, who presented to the Emergency Department for ischemic stroke, which appeared to be the first and only symptom of GCA. The prompt administration of steroids and tocilizumab (TCZ) led to clinical and radiological resolution, with no residual disability at 6-month follow-up.Our case-based review, highlighting the rarity of a large vessel vasculitis presenting only with a cerebrovascular accident, provides new evidence for the efficacy of TCZ even in more unusual varieties of GCA: in these cases, TCZ should be immediately prescribed, in order to prevent mortality and severe long-term morbidity

    Atypical Posterior Reversible Encephalopathy Syndrome (PRES) in a Patient with Polymyalgia Rheumatica and Giant Cell Arteritis

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    : Posterior reversible encephalopathy syndrome (PRES) is a potentially life-threatening condition, composed of focal neurologic symptoms and peculiar magnetic resonance imaging (MRI) findings suggestive for cerebral vasogenic edema. PRES has been predominantly associated with severe hypertension, but a concomitant inflammatory state, common in vasculitis, can contribute to worsening cerebral vasogenic edema towards cytotoxic edema, and it should be promptly treated with glucocorticoids (GC). Atypical cases of PRES should be suspected in cases of focal neurologic symptoms, associated with severe hypertension, and systemic inflammation. We report the first description of a patient with polymyalgia rheumatica and giant cell arteritis who developed PRES after GC discontinuation for arthroscopic surgery

    Impact of non-immunosuppressive medical therapy on disease progression and complications of Takayasu arteritis: A narrative review

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    Takayasu's arteritis is a rare large vessel vasculitis typically affecting young Asian women. It causes inflammation of the aorta and its major branches, leading to stenosis and aneurysmal dilations, and increasing cardiovascular morbidity due to accelerated atherosclerosis. Although glucocorticoids are effective for acute disease control and preliminary data on immunosuppressive drugs are promising, standardized treatment protocols are lacking. The use of prophylactic treatments with antihypertensives, antiplatelets, anticoagulants, and lipid-lowering drugs to prevent thrombotic and ischemic complications remains debated. This study reviews the evidence on the effectiveness of non-immunosuppressive medical therapy in Takayasu's arteritis. A search of the PubMed database identified eleven studies involving 204 patients. Antiplatelets: data on 68 patients were mixed, in fact low-dose aspirin did not prevent major cardiovascular events in 36 patients, but higher doses reduced ischemic complications in 24 patients. Anticoagulants: no data on new oral anticoagulants were available, and vitamin K antagonists in 9 patients did not alter cardiovascular complications. Antihypertensives: ACE-inhibitors controlled blood pressure in patients with renovascular hypertension but increased the risk of acute renal function decline, while β-blockers reduced the symptoms and the progression of myocardial hypertrophy in patients with heart failure and aortic regurgitation. Statins: data from two cohorts showed that while statins reduced the recurrence rate of arteritis in 30 patients, they did not affect recurrence rates or cardiovascular complications in 13 patients. Overall, current evidence, although not definitive, supports the use of non-immunosuppressive medical treatments to prevent long-term complications and damage in Takayasu's arteritis, considering the disease's pathophysiological mechanisms and increased cardiovascular risk. Further research is strongly encouraged

    Safety and Efficacy of Long-Term Tocilizumab in a Cohort of Patients with Giant Cell Arteritis: An Italian Monocentric Retrospective Study

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    Objective: Tocilizumab (TCZ) is the only biologic drug approved for the treatment of giant cell arteritis (GCA), having clinical trials and real-life studies proved its efficacy and safety. However, the optimal duration of the treatment has yet to be determined, being its early interruption associated with an increased risk of relapse. Conversely, prolonged schemes of therapy may rise safety concerns. The aim of the study was to evaluate the incidence of adverse events (AEs) and remission/relapse rate in a cohort of GCA patients treated with TCZ and an accelerated steroid tapering scheme, followed for 24 months. Methods: We retrospectively included patients referring to our clinic from January 2019 to November 2021 who were diagnosed with GCA and started subcutaneous TCZ treatment (162 mg/week). They also received up to 62,5 mg of prednisone (PDN), tapered following an accelerated six-month scheme. Results: We collected 38 patients, with a mean age of 76,4 years, treated with TCZ for an average of 22,3 months. AEs occurred in 11 (29%) subjects, and only one serious AE was reported; 7 (18%) patients permanently discontinued TCZ. At the end of the follow-up, all the patients continuing treatment showed clinical remission, with a PDN dosage <5mg. We registered 3 (8%) minor relapses under TCZ, after an average of 15 months. Conclusion: Our data support the evidence of a safe and effective long-term use of TCZ in GCA patients, especially when combined with moderate GCs doses for the shortest possible duration

    Biologic drugs in the treatment of polyarteritis nodosa and deficit of adenosine deaminase 2: a narrative review

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    Polyarteritis nodosa (PAN) is a medium vessels vasculitis variously involving different organs and systems, sometimes with an aggressive course, leading to death or disability in a significant number of cases. First-line treatment usually relies on steroids and classical immunosuppressants, but a growing number of case reports and small case series shows the potential role of biologic drugs, mostly anti-tumor necrosis factor (TNF)-alpha agents, in inducing and maintaining remission in patients affected by PAN. Similarly, the recently described auto-inflammatory disease named deficit of adenosine deaminase 2 (DADA2), considered by several experts as a more precocious and aggressive variant of PAN, seems to respond to a prompt treatment with TNF-alpha inhibitors. The aim of this review is to collect all existing evidences about the use of biologic drugs in PAN and DADA2. Fifty-one articles published during the last 15 years were retrieved, including 58 and 76 patients affected by PAN and DADA2, respectively, and treated with biologic drugs. The majority of subjects was treated with TNF-alpha inhibitors, whose effectiveness was reported in the treatment of such difficult-to-manage diseases, particularly in DADA2. Among the other biologic drugs, Tocilizumab was successfully employed in some subjects affected by PAN who did not respond to TNF-alpha inhibitors, while Rituximab did not give substantial benefits neither in PAN nor in DADA2. Only few data exist about the role of Janus-kinase inhibitors and anti-IL1 agents.This study provides the first comprehensive assessment of biologic agents in both PAN and DADA2, with encouraging results especially in the context of TNF-alpha inhibitors. Nevertheless, due to the lack of prospective, randomized, case control studies, further efforts should be made in order to fully elucidate the role of these drugs in such rare and life-threatening conditions
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