Antineutrophil cytoplasmic antibodies are classical serological markers of small-vessels vasculitis. However, they have been described in many other pathological situations. The aim of this study was to determine through our experience, the main antineutrophil cytoplasmic antibodies-associated diseases and to investigate antigen targets of these antibodies. Forty complete observations of antineutrophil cytoplasmic antibodies (ANCA) positive patients either by indirect immunofluorescence or by enzyme immunoassay were analysed. Only five (12.5%) patients have small-vessels vasculitis. Among these, antineutrophil cytoplasmic antibodies were detected only by Elisa in one patient and they were exclusively directed against bactericidal permeability increasing protein in another one. Our study confirms the presence of antineutrophil cytoplasmic antibodies in different diseases. It demonstrates that antineutrophil cytoplasmic antibodies should be investigated by Elisa when indirect immunofluorescence is negative. In small-vessels vasculitis, Proteinase 3 and myeloperoxidase are mainly but not exclusively the antigenic targets of antineutrophil cytoplasmic antibodies.
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http://dx.doi.org/10.1016/j.patbio.2008.07.019 | DOI Listing |
Cureus
December 2024
Department of Orthopaedics, Tokyo Metropolitan Bokutoh Hospital, Tokyo, JPN.
Hypertrophic pachymeningitis (HP) is a rare inflammatory disease that causes the thickening of the dura mater. Its etiology is mainly classified as idiopathic or secondary, and autoimmune disease is one of the main causes of secondary HP. Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis and IgG4-related disease are common among autoimmune diseases.
View Article and Find Full Text PDFMol Neurodegener
January 2025
College of Life Sciences and Oceanography, Brain Disease and Big Data Research Institute, Shenzhen University, Shenzhen, 518060, Guangdong, China.
Background: Astrocytes, the most abundant glial cell type in the brain, will convert into the reactive state in response to proteotoxic stress such as tau accumulation, a characteristic feature of Alzheimer's disease (AD) and other tauopathies. The formation of reactive astrocytes is partially attributed to the disruption of autophagy lysosomal signaling, and inhibiting of some histone deacetylases (HDACs) has been demonstrated to reduce the molecular and functional characteristics of reactive astrocytes. However, the precise role of autophagy lysosomal signaling in astrocytes that regulates tau pathology remains unclear.
View Article and Find Full Text PDFDrug Des Devel Ther
January 2025
Center of Expertise for Lupus-, Vasculitis- and Complement-Mediated Systemic Diseases (Luvacs), Department of Internal Medicine - Nephrology Section, Leiden University Medical Center, Leiden, the Netherlands.
Recently, avacopan has been approved for the treatment of ANCA-associated vasculitis (AAV). Avacopan is an inhibitor of the C5a-receptor, which plays an important role in chemotaxis and the amplification loop of inflammation in AAV. In the most recent, international guidelines avacopan is recommended as steroid-sparing agents for the management of AAV.
View Article and Find Full Text PDFKorean J Neurotrauma
December 2024
Department of Neurosurgery, Kyungpook National University Hospital, Kyungpook National University School of Medicine, Daegu, Korea.
This case report describes a rare presentation of antineutrophil cytoplasmic antibodies-associated hypertrophic pachymeningitis in a 49-year-old woman with cranial and spinal lesions. Initial management comprised decompressive laminectomy and steroid therapy, with the subsequent addition of immunosuppressant therapy following symptom recurrence. After a ten-year follow-up, significant symptom improvement and return of motor function were noted.
View Article and Find Full Text PDFTypical renal involvement of antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is pauci-immune glomerulonephritis that presents clinically as rapidly progressive renal failure (RPRF). Here, we report an unusual presentation of myeloperoxidase (MPO)-specific ANCA with isolated involvement of the tubulointerstitium in the form of peritubular capillaritis as the sole lesion without any involvement of the glomerulus. A 52-year-old woman with no previous comorbidities presented with nonspecific symptoms such as fatigue, dysuria, and nausea for two months.
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