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Systemic vasculitides comprise a collection of rare and heterogeneous disorders capable of impacting any organ and system, posing a considerable burden of mortality and comorbidity. As with previous annual reviews of this series, this review will offer a critical overview of the latest literature on pathogenesis, biomarkers, and treatment options in both small- and large-vessel vasculitis.
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http://dx.doi.org/10.55563/clinexprheumatol/gkve60 | DOI Listing |
Allergol Immunopathol (Madr)
September 2025
Corporación Universitaria Rafael Núñez, Ginumed, Cartagena, Colombia;
Human proteinase 3 (hPR3) is a lysosomal enzyme of the serine protease type. In autoimmune vasculitis, autoantibodies to hPR3 appear to have a role in the inception of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), where this protein is the main autoantigen. Indeed, patients with antibodies against hPR3 have more severe symptoms, relapses, and resistance to immunosuppressive therapies, supporting an important role for this autoantigen in the pathophysiology and severity of AAV.
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August 2025
Department of Internal Medicine, Local Health Unit of Santa Maria, Lisbon, PRT.
Polyarteritis nodosa (PAN) rarely affects both intracranial and mesenteric arteries. Evidence on optimal timing of revascularisation and the role of interleukin-6 blockade remains limited. A 73-year-old man with longstanding ankylosing spondylitis presented with weight loss and elevated inflammatory markers.
View Article and Find Full Text PDFMedicine (Baltimore)
September 2025
Al Mouwasat University Hospital, Damascus University, Damascus, Syria.
Rationale: Systemic sclerosis (SS) is an immune-mediated connective disease characterized by skin fibrosis, microvascular damage, and multisystem manifestations. One of the most important processes in connective tissue disorders is vasculitis. The clinical findings can differ when the disease is presented with an antineutrophil cytoplasmic antibody.
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August 2025
Norton College of Medicine, SUNY Upstate Medical University, Syracuse, USA.
Hydralazine is an antihypertensive that can induce immune-related adverse effects, such as hydralazine-induced lupus and hydralazine-induced antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitis (AAV). AAV involves necrotizing inflammation of small blood vessels, manifesting as fever, malaise, arthralgia, and myalgia, potentially leading to organ failure. Diagnosis includes clinical evaluation, serological testing for ANCA, and histopathological examination, confirmed by necrotizing granulomatous inflammation in affected tissues.
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August 2025
Internal Medicine, Hospital General Dr. Dario Fernandez Fierro, Mexico City, MEX.
Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is a rare form of pauci-immune vasculitis that primarily affects the respiratory tract and kidneys, though it can involve virtually any organ system. As a systemic vasculitis, it targets small- and medium-sized blood vessels and is associated with anti-neutrophil cytoplasmic antibodies (ANCA), particularly those directed against proteinase 3 (PR3). Due to its nonspecific symptoms and variable clinical presentation, GPA requires a high index of suspicion for timely diagnosis.
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