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Background: Granulomatosis with polyangiitis (GPA) is a rare autoimmune vasculitis affecting small and medium-sized vessels, commonly involving the respiratory tract and kidneys. Salivary gland involvement, particularly bilateral parotitis, is an uncommon presentation of GPA.

Case Report: We report the case of a 38-year-old Asian male who presented with left ear pain and parotid swelling after a water park visit.

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Objectives: To clarify the differences in clinical phenotypes, therapeutic patterns, and outcomes of patients with granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) across geographic regions using a multinational cohort.

Methods: Data were collected from patients with newly diagnosed or relapsing GPA or MPA in Europe, Japan, and the United States (US) from January to July 2020. The composite outcome of kidney failure and/or death within 52 weeks after treatment was evaluated, and the hazard ratios across the regions were estimated using the Cox proportional hazard model.

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A 16-year-old male with newly diagnosed granulomatosis with polyangiitis presented to the emergency room with chest pain. He was found to have a myocardial infarction involving the right coronary artery and the left circumflex artery. He underwent mechanical thrombectomy and stent placement without significant sequelae.

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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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Granulomatosis with polyangiitis (GPA) is a subtype of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) that commonly requires aggressive immunosuppression to achieve remission. We present a case of a young Malay lady with recurrent episodes of ANCA-positive nodular anterior scleritis who responded poorly to topical and systemic corticosteroids and relapsed while on methotrexate. A year later, she had epistaxis, and a sino-nasal biopsy confirmed granulomatous vasculitis.

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