Background: Anti-neutrophil cytoplasm antibodies in necrotizing vasculitides need to be distinguished from ANCAs in other inflammatory conditions to avoid clinical misinterpretation.
Objectives: To help clinicians and laboratory scientists recognize and utilize vasculitis-related ANCAs as an aid in diagnostic workup and patient follow-up, and be aware that ANCAs with different characteristics are commonly found in other chronic inflammatory conditions that persistently engage neutrophils in the inflammatory process.
Methods: Indirect immunofluorescence and enzyme immunoassay methods were used to detect ANCAs with both known and unknown neutrophil autoantigenic targets.
Results: Primary necrotizing small vessel vasculitides such as Wegener's granulomatosis, Churg-Strauss syndrome, microscopic polyangiitis, and renal-limited rapidly progressive necrotizing glomerulonephritis target either the serine protease proteinase 3 or myeloperoxidase in azurophilic granules. In ulcerative colitis and rheumatoid arthritis, we found multiple ANCA targets contained in azurophilic and specific granules, the cytosol and the nucleus, whereas PR3 and MPO were not, or only weakly, recognized.
Conclusions: ANCAs typically found in active SVV are demonstrable both by indirect immunofluorescence and antigen-specific enzyme immunoassay, and strong reactivity to either PR3 or MPO is characteristic. Strong ANCA with MPO reactivity is also found in some patients with drug-induced syndromes (lupus, vasculitis). Intermediate to strong perinuclear ANCAs are found in a substantial proportion of patients with UC (40-60%) and RA (30-70%), but in these conditions the ANCAs have many antigen targets that are only weakly recognized.
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Indian J Nephrol
July 2024
Department of Nephrology, Christian Medical College, Vellore, Tamil Nadu, India.
The simultaneous occurrence of vasculitic glomerulonephritis and membranous nephropathy is unusual. We report two cases that presented to our outpatient department with rapidly progressive renal failure. On evaluation, in one patient, anti-myeloperoxidase (MPO) titers were high, and renal biopsy was suggestive of concurrent necrotizing and diffuse crescentic anti-MPO anti-neutrophil cytoplasmic antigen-associated glomerulonephritis with the circumferential cellular crescent formation and membranous glomerulopathy.
View Article and Find Full Text PDFReumatologia
December 2024
Department of Rheumatology and Immunology, Jagiellonian University Medical College, Krakow, Poland.
Introduction: Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by eosinophilic granulomatous vasculitis. Typical symptoms include late-onset bronchial asthma and blood and tissue eosinophilia. In addition to these characteristic symptoms, EGPA can affect important organs such as the skin, kidneys, heart, sinuses, gastrointestinal tract, and nervous system.
View Article and Find Full Text PDFAME Case Rep
October 2024
Center for Asbestos-Related Diseases, Toyama Rosai Hospital, Toyama, Japan.
Background: The underlying pathophysiology of some occupational diseases such as silicosis involves autoantibodies. An autoantibody, antineutrophil cytoplasmic antibody (ANCA), has been recently reported and is known to be elevated in diseases such as vasculitis; therefore, the disease is currently known as ANCA-associated vasculitis. The risk of ANCA-associated vasculitis is known to be 25 times higher in patients with silicosis than in those without any occupational disease.
View Article and Find Full Text PDFNefrologia (Engl Ed)
January 2025
Unidad de Enfermedades Sistémicas Autoinmunes, Departamento de Medicina Interna, Hospital Universitario Vall d'Hebron, Barcelona, Spain.
Anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis is characterised by small vessel necrotising inflammatory vasculitis. Prior to immunosupressant therapy availability it usually led to a fatal outcome. Current treatment has changed ANCA-associated vasculitis into a condition with a significant response rate, although with a not negligible relapse occurrence and cumulative organ lesions, mostly due to drug-related toxicities.
View Article and Find Full Text PDFBMJ Case Rep
January 2025
Department of Nephrology, Barts Health NHS Trust, London, UK.
Anti-neutrophil cytoplasmic antibody-associated vasculitides (AAV) represent a heterogeneous multisystem group of disorders typified by necrotising inflammation of smaller blood vessels, classically yielding a pauci-immune, crescentic glomerulonephritis. Without prompt treatment, there is a significant risk of irreversible damage and ensuing renal impairment.Diagnosis is often challenging, exacerbated by the disorder's often vague and insidious presentation.
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