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Pathophysiology of Hemophilic Arthropathy
Spontaneous joint bleeding and repeated hemarthroses lead to hemophilic arthropathy—a debilitating disease with a significant negative impact on mobility and quality of life. Iron, cytokines, and angiogenic growth factors play a pivotal role in the onset of the inflammatory process that involves the...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2017
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5532571/ https://www.ncbi.nlm.nih.gov/pubmed/28672826 http://dx.doi.org/10.3390/jcm6070063 |
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author | Melchiorre, Daniela Manetti, Mirko Matucci-Cerinic, Marco |
author_facet | Melchiorre, Daniela Manetti, Mirko Matucci-Cerinic, Marco |
author_sort | Melchiorre, Daniela |
collection | PubMed |
description | Spontaneous joint bleeding and repeated hemarthroses lead to hemophilic arthropathy—a debilitating disease with a significant negative impact on mobility and quality of life. Iron, cytokines, and angiogenic growth factors play a pivotal role in the onset of the inflammatory process that involves the synovial tissue, articular cartilage, and subchondral bone, with early damages and molecular changes determining the perpetuation of a chronic inflammatory condition. Synovitis is one of the earliest complications of hemarthrosis, and is characterized by synovial hypertrophy, migration of inflammatory cells, and a high degree of neo-angiogenesis with subsequent bleeding. The pathogenic mechanisms and molecular pathways by which blood in the joint cavity causes articular cartilage and subchondral bone destruction have yet to be fully elucidated. Both cytokines and matrix metalloproteinases and hydroxyl radicals may induce chondrocyte apoptosis. Members of the tumor necrosis factor receptor superfamily (such as the molecular triad: osteoprotegerin—OPG; receptor activator of nuclear factor κB—RANK; RANK ligand—RANKL) seem instead to play a major role in the inflammatory process. These pathogenic processes interact with each other and ultimately lead to a fibrotic joint and the disabling condition characteristic of hemophilic arthropathy. |
format | Online Article Text |
id | pubmed-5532571 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2017 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-55325712017-08-08 Pathophysiology of Hemophilic Arthropathy Melchiorre, Daniela Manetti, Mirko Matucci-Cerinic, Marco J Clin Med Review Spontaneous joint bleeding and repeated hemarthroses lead to hemophilic arthropathy—a debilitating disease with a significant negative impact on mobility and quality of life. Iron, cytokines, and angiogenic growth factors play a pivotal role in the onset of the inflammatory process that involves the synovial tissue, articular cartilage, and subchondral bone, with early damages and molecular changes determining the perpetuation of a chronic inflammatory condition. Synovitis is one of the earliest complications of hemarthrosis, and is characterized by synovial hypertrophy, migration of inflammatory cells, and a high degree of neo-angiogenesis with subsequent bleeding. The pathogenic mechanisms and molecular pathways by which blood in the joint cavity causes articular cartilage and subchondral bone destruction have yet to be fully elucidated. Both cytokines and matrix metalloproteinases and hydroxyl radicals may induce chondrocyte apoptosis. Members of the tumor necrosis factor receptor superfamily (such as the molecular triad: osteoprotegerin—OPG; receptor activator of nuclear factor κB—RANK; RANK ligand—RANKL) seem instead to play a major role in the inflammatory process. These pathogenic processes interact with each other and ultimately lead to a fibrotic joint and the disabling condition characteristic of hemophilic arthropathy. MDPI 2017-06-25 /pmc/articles/PMC5532571/ /pubmed/28672826 http://dx.doi.org/10.3390/jcm6070063 Text en © 2017 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Review Melchiorre, Daniela Manetti, Mirko Matucci-Cerinic, Marco Pathophysiology of Hemophilic Arthropathy |
title | Pathophysiology of Hemophilic Arthropathy |
title_full | Pathophysiology of Hemophilic Arthropathy |
title_fullStr | Pathophysiology of Hemophilic Arthropathy |
title_full_unstemmed | Pathophysiology of Hemophilic Arthropathy |
title_short | Pathophysiology of Hemophilic Arthropathy |
title_sort | pathophysiology of hemophilic arthropathy |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5532571/ https://www.ncbi.nlm.nih.gov/pubmed/28672826 http://dx.doi.org/10.3390/jcm6070063 |
work_keys_str_mv | AT melchiorredaniela pathophysiologyofhemophilicarthropathy AT manettimirko pathophysiologyofhemophilicarthropathy AT matuccicerinicmarco pathophysiologyofhemophilicarthropathy |