Erdheim-Chester disease is an unusual syndrome characterized by non-Langerhans cell histiocytosis which can mimic rheumatologic diseases. We report a case of Erdheim-Chester masquerading as giant cell arteritis, which...Erdheim-Chester disease is an unusual syndrome characterized by non-Langerhans cell histiocytosis which can mimic rheumatologic diseases. We report a case of Erdheim-Chester masquerading as giant cell arteritis, which was success-fully managed with anakinra as anti IL-1 therapy.展开更多
We report a rare case of association of temporal arteritis with recurrent central corneal ulcer. A 91-year-old male with a remote history of clinically diagnosed giant cell arteritis (GCA) and penetrating keratoplasty...We report a rare case of association of temporal arteritis with recurrent central corneal ulcer. A 91-year-old male with a remote history of clinically diagnosed giant cell arteritis (GCA) and penetrating keratoplasty (PK) for corneal edema in 1990’s in the left eye presented with irritation and severe dry eye. He progressively developed central corneal ulcer regardless of treatment with aggressive lubrication and patching. The clinical course, blood tests and temporal artery biopsy results yielded the diagnosis of recurrent GCA. The clinical course improved after starting oral steroids. The case report illustrates the importance of ruling out GCA in patients with recurrent corneal ulcers and history of GCA. It also highlights the significance of shared understanding between primary care physicians and specialists to handle common and chronic diseases to make efficient diagnoses and plan treatment regimens. This is the first plausible case of reported GCA association with central corneal ulcer in the United States.展开更多
Objective: To determine the effectiveness of antiplatelet and/or anticoagulant therapy (AP/AC) at reducing ischemic events in patients with Large Vessel Vasculitis (LVV). Methods: We performed a random effects meta-an...Objective: To determine the effectiveness of antiplatelet and/or anticoagulant therapy (AP/AC) at reducing ischemic events in patients with Large Vessel Vasculitis (LVV). Methods: We performed a random effects meta-analysis of studies examining antiplatelet and/or anticoagulant therapy (AP/AC) and ischemic events in Takayasu’s Arteritis (TAK) or Giant Cell Arteritis (GCA). Severe ischemic events were defined as stroke, ischemic ocular manifestations and claudication symptoms. Any ischemic event included jaw claudication in addition to the above manifestations. Results: Seven studies met inclusion criteria: 1 TAK and 6 GCA. The majority of patients (>80%) were treated with ASA and treatment was initiated prior to diagnosis of LVV. Risk of severe and any ischemic event in patients with LVV treated with AP/AC versus no treatment was not significantly different (OR 0.570, 95% CI 0.243, 1.340 and OR 0.594, 95% CI 0.248, 1.421, respectively). For studies with follow-up data (26-76 months), AP/AC was protective for severe ischemic events (OR 0.18, 95% CI 0.04, 0.83). Findings were similar when excluding studies that did not account for potential confounders, such as cardiovascular risk factors. Conclusion: At follow-up, antiplatelet therapy significantly decreases ischemic events in patients with LVV. However, in most cases of GCA, the treatment was initiated prior to the diagnosis of vasculitis. The benefit of initiating anti-platelet therapy at the time of GCA diagnosis remains unclear.展开更多
Glucocorticoids remain the cornerstone of medicaltherapy in giant cell arteritis(GCA) and should be started immediately to prevent severe consequences of the disease, such as blindness. However, glucocorticoid therapy...Glucocorticoids remain the cornerstone of medicaltherapy in giant cell arteritis(GCA) and should be started immediately to prevent severe consequences of the disease, such as blindness. However, glucocorticoid therapy leads to significant toxicity in over 80% of the patients. Various steroid-sparing agents have been tried, but robust scientific evidence of their efficacy and safety is still lacking. Tocilizumab, a monoclonal IL-6 receptor blocker, has shown promising results in a number of case series and is now being tested in a multi-centre randomized controlled trial. Other targeted treatments, such as the use of abatacept, are also now under investigation in GCA. The need for surgical treatment is rare and should ideally be performed in a quiescent phase of the disease. Not all patients follow the same course, but there are no valid biomarkers to assess therapy response. Monitoring of disease progress still relies on assessing clinical features and measuring inflammatory markers(C-reactive protein and erythrocyte sedimentation rate). Imaging techniques(e.g., ultrasound) are clearly important screening tools for aortic aneurysms and assessing patients with largevessel involvement, but may also have an important role as biomarkers of disease activity over time or in response to therapy. Although GCA is the most common form of primary vasculitis, the optimal strategies for treatment and monitoring remain uncertain.展开更多
文摘Erdheim-Chester disease is an unusual syndrome characterized by non-Langerhans cell histiocytosis which can mimic rheumatologic diseases. We report a case of Erdheim-Chester masquerading as giant cell arteritis, which was success-fully managed with anakinra as anti IL-1 therapy.
文摘We report a rare case of association of temporal arteritis with recurrent central corneal ulcer. A 91-year-old male with a remote history of clinically diagnosed giant cell arteritis (GCA) and penetrating keratoplasty (PK) for corneal edema in 1990’s in the left eye presented with irritation and severe dry eye. He progressively developed central corneal ulcer regardless of treatment with aggressive lubrication and patching. The clinical course, blood tests and temporal artery biopsy results yielded the diagnosis of recurrent GCA. The clinical course improved after starting oral steroids. The case report illustrates the importance of ruling out GCA in patients with recurrent corneal ulcers and history of GCA. It also highlights the significance of shared understanding between primary care physicians and specialists to handle common and chronic diseases to make efficient diagnoses and plan treatment regimens. This is the first plausible case of reported GCA association with central corneal ulcer in the United States.
文摘Objective: To determine the effectiveness of antiplatelet and/or anticoagulant therapy (AP/AC) at reducing ischemic events in patients with Large Vessel Vasculitis (LVV). Methods: We performed a random effects meta-analysis of studies examining antiplatelet and/or anticoagulant therapy (AP/AC) and ischemic events in Takayasu’s Arteritis (TAK) or Giant Cell Arteritis (GCA). Severe ischemic events were defined as stroke, ischemic ocular manifestations and claudication symptoms. Any ischemic event included jaw claudication in addition to the above manifestations. Results: Seven studies met inclusion criteria: 1 TAK and 6 GCA. The majority of patients (>80%) were treated with ASA and treatment was initiated prior to diagnosis of LVV. Risk of severe and any ischemic event in patients with LVV treated with AP/AC versus no treatment was not significantly different (OR 0.570, 95% CI 0.243, 1.340 and OR 0.594, 95% CI 0.248, 1.421, respectively). For studies with follow-up data (26-76 months), AP/AC was protective for severe ischemic events (OR 0.18, 95% CI 0.04, 0.83). Findings were similar when excluding studies that did not account for potential confounders, such as cardiovascular risk factors. Conclusion: At follow-up, antiplatelet therapy significantly decreases ischemic events in patients with LVV. However, in most cases of GCA, the treatment was initiated prior to the diagnosis of vasculitis. The benefit of initiating anti-platelet therapy at the time of GCA diagnosis remains unclear.
文摘Glucocorticoids remain the cornerstone of medicaltherapy in giant cell arteritis(GCA) and should be started immediately to prevent severe consequences of the disease, such as blindness. However, glucocorticoid therapy leads to significant toxicity in over 80% of the patients. Various steroid-sparing agents have been tried, but robust scientific evidence of their efficacy and safety is still lacking. Tocilizumab, a monoclonal IL-6 receptor blocker, has shown promising results in a number of case series and is now being tested in a multi-centre randomized controlled trial. Other targeted treatments, such as the use of abatacept, are also now under investigation in GCA. The need for surgical treatment is rare and should ideally be performed in a quiescent phase of the disease. Not all patients follow the same course, but there are no valid biomarkers to assess therapy response. Monitoring of disease progress still relies on assessing clinical features and measuring inflammatory markers(C-reactive protein and erythrocyte sedimentation rate). Imaging techniques(e.g., ultrasound) are clearly important screening tools for aortic aneurysms and assessing patients with largevessel involvement, but may also have an important role as biomarkers of disease activity over time or in response to therapy. Although GCA is the most common form of primary vasculitis, the optimal strategies for treatment and monitoring remain uncertain.
