Vasculitis poses a great diagnostic, investigative and therapeutic challenge to the treating physician. The classification of vasculitides itself still eludes universal acceptance. Comprehensive management comprises establishing the diagnosis of true vasculitis after ruling out vasculitis mimics, finding the etiology if feasible, assessing the caliber of the vessels involved, deciphering the pathological process of vessel damage, investigating for the existence and extent of systemic involvement and finally planning the therapy in the background of co-morbidities. Successful management also entails regular monitoring to foresee complications arising from the disease process itself as well as complications of immunosuppressive treatment. Although steroids remain first line drug, biologics are emerging as popular agents in the treatment of immune-mediated vasculitis. Triphasic treatment is the best plan of action comprising induction, maintenance of remission and treatment of relapses.
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http://dx.doi.org/10.4103/idoj.IDOJ_248_18 | DOI Listing |
Pediatr Rheumatol Online J
December 2024
Section of Rheumatology, Department of Pediatrics, Alberta Children's Hospital, University of Calgary, Calgary, Canada.
Background: Primary small vessel CNS vasculitis (sv-cPACNS) is a challenging inflammatory brain disease in children. Brain biopsy is mandatory to confirm the diagnosis. This study aims to develop and validate a histological scoring tool for diagnosing small vessel CNS vasculitis.
View Article and Find Full Text PDFArthritis Res Ther
December 2024
Department of Rheumatology, Hospital Universitario de Bellvitge. Bellvitge Biomedical Research Institute (IDIBELL), Barcelona, Spain.
Objective: To investigate differences in arterial involvement patterns on F-FDG PET-CT between predominant cranial and isolated extracranial phenotypes of giant cell arteritis (GCA).
Methods: A retrospective review of F-FDG PET-CT findings was conducted on 140 patients with confirmed GCA. The patients were divided into two groups: the cranial group, which presented craniofacial ischemic symptoms either at diagnosis or during follow-up, and the isolated extracranial group which never exhibited such manifestations.
Autoimmun Rev
December 2024
Institute of Pathology, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia. Electronic address:
Giant cell arteritis (GCA) is a primary systemic vasculitis affecting the elderly, characterized by a granulomatous vessel wall inflammation of large- and medium-sized arteries. The immunopathology of GCA is complex, involving both the innate and adaptive arms of the immune system, where a maladaptive inflammatory-driven vascular repair process ultimately results in vessel wall thickening, intramural vascular smooth muscle cell proliferation, neovascularization and vessel lumen occlusion, which can lead to serious ischemic complications such as visual loss and ischemic stroke. Over the past decade, microRNA (miRNA) dysregulation has been highlighted as an important contributing factor underlying the pathogenesis of GCA.
View Article and Find Full Text PDFPurpose: This study investigates the capabilities of ultrasonography (US) in determing the stage of orbital inflammation in patients with granulomatosis with polyangiitis (GPA).
Material And Methods: The study included 24 patients (8 men and 16 women) with diffuse orbital tissue involvement in GPA. Group 1 (active stage) included nine patients, while group 2 (inactive stage) consisted of 18 patients.
Vestn Otorinolaringol
December 2024
Pavlov Ryazan State Medical University, Ryazan, Russia.
The article presents an analysis of 2 clinical cases of granulomatosis with polyangiitis from the practice of an otorhinolaryngologist. In the first case, the pathology manifested with lesions of the middle ear and nose. In the second case, in addition to manifestations from the nose and the ear, there was a lesion of the larynx.
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