In patients with connective tissue disease, vascular injury induced by primary or secondary vasculitis syndromes can lead to organ dysfunction due to the loss of nutrient supply from the blood. Such vasculitis syndromes can be refractory to treatment and fatal. The nomenclature and the definition of vasculitis syndromes have recently been revised, and clinical practice guidelines for diseases associated with vasculitis syndrome are evolving. The present review provides an overview of vasculitis syndromes from the viewpoint of diagnosis and treatment.
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http://dx.doi.org/10.1002/jgf2.4 | DOI Listing |
J Neurol
January 2025
Department of Neuro-Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Background: Neurologic symptoms seen in patients receiving immune checkpoint inhibitors (ICI) may not be entirely caused by immunotoxicity. We aim to highlight these confounding conditions through clinical cases to encourage early recognition and management.
Methods: We describe a series of seven cases from our institution that were treated with ICI and presented with Neurologic symptoms and were diagnosed with superimposed conditions beyond immunotoxicity.
Background: When Behçet's disease is complicated with gastrointestinal ulcers, it is referred to as intestinal Behçet's disease (BD). Clinically uncommon, this condition can involve the entire gastrointestinal tract, often presenting diagnostic challenges in differentiation from Crohn's disease.
Methods: In this case, atypical BD was diagnosed through endoscopic examination, whereas latent tuberculosis infection (LBTI) was confirmed via T-SPOT and PPD tests.
Turk J Pediatr
December 2024
Department of Pediatric Rheumatology, Umraniye Training and Research Hospital, University of Health Sciences, İstanbul, Türkiye.
Background: Polyarteritis nodosa (PAN) is a rare and serious form of systemic necrotizing vasculitis that predominantly affects medium and small-sized arteries, with central nervous system involvement being particularly uncommon. Treatment strategies are tailored according to the extent and severity of the disease. While conventional therapy includes glucocorticoids and conventional disease-modifying-rheumatic drugs (cDMARDs), biologic agents may be critical for severe and refractory cases.
View Article and Find Full Text PDFOrphanet J Rare Dis
January 2025
Department of Rheumatology and Immunology, The First Affiliated Hospital, Fujian Medical University, Fuzhou, 350005, Fujian, China.
Background: Intestinal Behçet's syndrome (IBS) has high morbidity and mortality rates with serious complications. However, there are few specific biomarkers for IBS. The purposes of this study were to investigate the distinctive metabolic changes in plasma samples between IBS patients and healthy people, active IBS and inactive IBS patients, and to identify candidate metabolic biomarkers which would be useful for diagnosing and predicting IBS.
View Article and Find Full Text PDFActa Dermatovenerol Croat
November 2024
Khalid Al Aboud King Faisal Hospital P.O Box 5440, Makkah, Saudi Arabia;
parts of the world (1,2). CL is characterized by significant clinical variability. An ulcerated nodule on the exposed parts of the body (corresponding to the parasite inoculation site by the vector insect) is the classic presentation.
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