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Specialty: Rheumatology
Condition: Vasculitis
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Total 90 results found since Jan 2013.
Vasculitis associated with adenosine deaminase 2 deficiency: at the crossroads between Beh çet's disease and autoinflammation. A viewpoint
Reumatismo. 2023 Sep 18;75(3). doi: 10.4081/reumatismo.2023.1578.ABSTRACTAdenosine deaminase 2 deficiency (DADA2) is a rare monogenic vasculopathy caused by loss-of-function homozygous or compound heterozygous mutations in ADA2, formerly CECR1 (cat eye syndrome chromosome region 1) gene. The DADA2 phenotype is widely heterogeneous, and patients may present with fever, weight loss, livedo reticularis/racemosa, digital ischemia, cutaneous ulceration, peripheral neuropathy, abdominal pain, bowel perforation, and portal or nephrogenic hypertension. More specific manifestations include early-onset ischemic or hemorrhagic stroke...
Source: Reumatismo - September 18, 2023 Category: Rheumatology Authors: A Colangelo F Tromby G Cafaro R Gerli E Bartoloni C Perricone Source Type: research
Case Report: Giant Cell Arteritis-Related Stroke
Thromboembolic events are major contributors to the morbidity and mortality of patients with giant cell arteritis (GCA), but little is known about how GCA may increase the risk of ischemic strokes. GCA-related stroke is described as an ischemic cerebral infarct occurring within three to four weeks of GCA diagnosis and treatment. It occurs in 3–7%... [Read More]
Source: The Rheumatologist - September 10, 2023 Category: Rheumatology Authors: Jessica Meek, MD, Anna Coppinger, DO, & Sejal Khan, DO Tags: Conditions Home Slider Vasculitis case report GCA giant cell arteritis (GCA) stroke Source Type: research
Deficiency of Adenosine Deaminase 2
Deficiency of adenosine deaminase 2 (DADA2) is a monogenic vasculitis syndrome caused by biallelic mutations in the adenosine deaminase 2 gene. The diagnosis of DADA2 is confirmed by decreased enzymatic activity of ADA2 and genetic testing. Symptoms range from cutaneous vasculitis and polyarteritis nodosa-like lesions to stroke. The vasculopathy of DADA2 can affect many organ systems, including the gastrointestinal and renal systems. Hematologic manifestations occur early with hypogammaglobulinemia, lymphopenia, pure red cell aplasia, or pancytopenia. Treatment can be challenging. Tumor necrosis factor inhibitors are helpf...
Source: Rheumatic Disease Clinics of North America - August 1, 2023 Category: Rheumatology Authors: Andrew Grim, Keila R. Veiga, Nadine Saad Source Type: research
Incidental findings on FDG-PET/CT in large vessel vasculitis
This study aims to determine the number and type of incidental findings detected on positron emission tomography (PET)/CT in a cohort of patients with large vessel vasculitis (LVV). Reports from PET/CT studies along with the medical charts of a cohort of patients with LVV from a Rheumatology clinic in Edmonton, Alberta, Canada, were retrospectively reviewed. Incidental findings from PET/CT, along with follow-up studies and their diagnosis were documented. The data was analyzed with descriptive statistics. The disease activity of 40 patients, with an average age of 65.8 years, was investigated using PET/CT. A statisticall...
Source: Clinical Rheumatology - July 17, 2023 Category: Rheumatology Source Type: research
For Your Eyes Only: 007 Tips for the Management of Cardiovascular Risk Factors in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis
J Rheumatol. 2023 Apr 15:jrheum.2023-0219. doi: 10.3899/jrheum.2023-0219. Online ahead of print.ABSTRACTPremature atherosclerosis has been observed during the course of different systemic inflammatory diseases, such as rheumatoid arthritis (RA), systemic lupus erythematosus, and antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).1-3 As a result, cardiovascular (CV) events, including myocardial infarction, stroke, heart failure, coronary artery disease, and death, occur 1.65 to 3 times more commonly in patients with AAV as compared to the general population, and the risk appears to be highest in the firs...
Source: Journal of Rheumatology - April 15, 2023 Category: Rheumatology Authors: Jan Willem Cohen Tervaert Source Type: research
