Granulomatosis with polyangiitis (GPA) is a rare auto-immune ANCA-associated small-vessel vasculitis characterized by necrotizing granulomatous inflammation, primarily affecting the sinuses, respiratory tract, and kidneys. Early diagnosis and treatment are crucial for improving patient outcomes and preventing rapidly progressive renal failure. We present a case of a 66-year-old woman presenting to the emergency department (ED) with a two-week history of productive cough, nausea, fevers, and headache. Observations and routine blood tests were unremarkable, so the patient was treated with antibiotics for a chest infection. Headache became a particularly prominent feature, along with jaw claudication and temporal tenderness. Treatment for another vasculitis, giant cell arteritis (GCA), was started and then stopped after ultrasound scanning showed no signs of GCA. After repeated admissions to the hospital, she was eventually diagnosed with GPA after the auto-immune screening was sent, which was positive for cANCA (anti-PR3). This case highlights a high degree of diagnostic uncertainty due to multiple investigations revealing the involvement of different organs, but these were treated separately. A more holistic approach to multi-system pathology, as well as considering other causes of fever and raised inflammatory markers, would have spared the patient from many unnecessary and invasive investigations. The patient eventually made a rapid recovery after the initiation of appropriate immunosuppressant therapy.
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http://dx.doi.org/10.7759/cureus.78502 | DOI Listing |
Rheumatology (Oxford)
March 2025
Nephrology and Dialysis Unit, Meyer Children's Hospital IRCCS, Florence, Italy.
Hypereosinophilic syndrome (HES) and eosinophilic granulomatosis with polyangiitis (EGPA) both present with hypereosinophilia and organ damage induced by eosinophils. EGPA is also characterized by vasculitis and is associated with ANCA. Yet, discriminating HES from EGPA may be difficult in clinical practice as biomarkers to reliably differentiate between HES and EGPA are still lacking.
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March 2025
Vasculitis and Lupus Clinic, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK.
This session presented emerging realworld evidence on novel glucocorticoid (GC) sparing therapies for ANCA-associated vasculitis (AAV). It covered the first-in-class oral C5a receptor antagonist avacopan for severe granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), and antiinterleukin-5 therapies in the management of eosinophilic granulomatosis with polyangiitis (EGPA). Avacopan was approved by the US Food and Drug Administration (FDA) and European Medicines Agency (EMA) for the treatment of AAV in 2021, following the phase 3 ADVOCAT E trial comparing oral avacopan to an oral GC taper for GPA and MPA.
View Article and Find Full Text PDFRheumatology (Oxford)
March 2025
Rheumatic and Immunologic Diseases, Cleveland Clinic, Cleveland, OH, USA.
Systemic vasculitis can present with different manifestations, some of which require unique approaches. This session at the 21st International Vasculitis Workshop, examined six studies focused on "Management of Special Situations". In ANCA-associated vasculitis (AAV), two institutions reported on their experience with subglottic stenosis (SGS) from the standpoint of histologic features and management compared to patients with idiopathic SGS.
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March 2025
Department of Internal Medicine IV, Nephrology and Hypertension, Medical University Innsbruck, Innsbruck, Austria.
The steps leading to key pathogenetic aspect of ANCA-associated vasculitis, such as lung nodules in granulomatosis with polyangiitis (GPA), onset of glomerulonephritis (GN) in experimental myeloperoxidase (MPO)-ANCA models and the implications of carrying staphylococcal superantigens, such as toxic shock syndrome toxin-1 (tsst-1), in GPA remain to be elucidated. The session 'Cellular and molecular mechanisms of disease (II)' was devoted to close some of these knowledge gaps and underlined that research in ANCA-associated vasculitis leads to more granular understanding of these complex diseases. Here, we present an overview of this session at a glance.
View Article and Find Full Text PDFMod Rheumatol
March 2025
Health Economics and Payer Evidence, AstraZeneca, Cambridge, UK.
Objective: To conduct a systematic literature review and meta-analysis for the incidence, prevalence and mortality of eosinophilic granulomatosis with polyangiitis (EGPA) and describe the clinical burden of EGPA.
Methods: Searches were conducted in Ovid MEDLINE and Ovid Embase from January 2019 to June 2023 to identify relevant studies. Data were extracted, and the quality was assessed.
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