Next Article in Journal
Myopathology of Adult and Paediatric Mitochondrial Diseases
Next Article in Special Issue
Ultrasound for Early Detection of Joint Disease in Patients with Hemophilic Arthropathy
Previous Article in Journal
Fibrotic Hypersensitivity Pneumonitis: Key Issues in Diagnosis and Management
Previous Article in Special Issue
Hemophilia Care in the Pediatric Age
Open AccessReview

Pathophysiology of Hemophilic Arthropathy

Section of Internal Medicine, Department of Experimental and Clinical Medicine, University of Florence, 50134 Florence, Italy
Department of Geriatric Medicine, Division of Rheumatology, Azienda Ospedaliero-Universitaria Careggi 50134 Florence, Italy
Section of Anatomy and Histology, Department of Experimental and Clinical Medicine, University of Florence, 50134 Florence, Italy
Author to whom correspondence should be addressed.
J. Clin. Med. 2017, 6(7), 63;
Received: 4 March 2017 / Revised: 16 June 2017 / Accepted: 22 June 2017 / Published: 25 June 2017
(This article belongs to the Special Issue Outstanding Advances in Hemophilia Therapies)
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. View Full-Text
Keywords: hemophilia; hemarthrosis; hemophilic arthropathy; synovitis hemophilia; hemarthrosis; hemophilic arthropathy; synovitis
Show Figures

Figure 1

MDPI and ACS Style

Melchiorre, D.; Manetti, M.; Matucci-Cerinic, M. Pathophysiology of Hemophilic Arthropathy. J. Clin. Med. 2017, 6, 63.

Show more citation formats Show less citations formats
Note that from the first issue of 2016, MDPI journals use article numbers instead of page numbers. See further details here.

Article Access Map by Country/Region

Back to TopTop