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Background/aims: This study applied the 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology (ACR/EULAR) criteria for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) to patients with Behçet's disease (BD) to investigate the proportion and clinical implications of the reclassification to the overlap syndrome of BD and AAV (OS-BD-AAV).

Methods: We included 280 BD patients presenting with ANCA positivity but without medical conditions mimicking AAV at diagnosis. Demographic data, items from the 2014 revised International Criteria for BD and 2022 American College of Rheumatology and European Alliance of Associations for Rheumatology criteria for AAV, ANCA positivity, and laboratory results were recorded as clinical data at diagnosis.

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Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are autoimmune inflammatory small-vessel disorders with potentially life-threatening organ manifestations. Recent disease definitions and classification criteria allow distinction between granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), and non-granulomatous microscopic polyangiitis (MPA). The discovery of ANCA-autoantibodies directed against proteolytic enzymes of neutrophil granules-has enabled earlier diagnosis of AAV and paved the way to stage-adapted treatments.

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Article Synopsis
  • Adenosine deaminase 2 (DADA2) deficiency is an autoinflammatory disease caused by mutations in the ADA2 gene, leading to various symptoms and potential complications like renal subcapsular hematoma.
  • An 18-year-old female with a history of optic neuritis experienced severe flank pain and inflammatory symptoms, and imaging confirmed bilateral renal subcapsular hematomas, leading to the consideration of DADA2 based on her symptoms and family history.
  • The patient was treated successfully with immunosuppressives, resulting in significant improvement and reduction of the hematomas, highlighting the importance of early diagnosis and treatment in managing DADA2 effectively.
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Granulomatosis with polyangiitis is a rare vasculitis characterized by necrotizing granulomatous inflammation affecting small blood vessels. It often presents with pulmonary masses and nodules, mimicking malignancy in imaging studies. Distinguishing granulomatosis with polyangiitis from cancer is crucial due to differing treatment approaches and prognoses.

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Background: Hepatitis B virus (HBV) infection affects millions worldwide, predominantly targeting the liver, leading to conditions like cirrhosis and hepatocellular cancer. However, HBV can also cause extrahepatic complications, including autoimmune-mediated vasculitis. Small vessel vasculitis, often associated with antineutrophil cytoplasmic antibodies (ANCA), can occur in chronic infections like HBV.

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