Physiopathology of haemophilic arthropathy
Authors: LAFEBER, F. P. J. G.1; MIOSSEC, P.2; VALENTINO, L. A.3
Source: Haemophilia, Volume 14, Supplement 4, July 2008 , pp. 3-9(7)
Publisher: Blackwell Publishing
Abstract:
Summary.
Haemophilic arthropathy, which shares some clinical and biological
injury characteristics with rheumatoid arthritis, is characterized by
two main features: chronic proliferative synovitis and cartilage
destruction.
It is the consequence of repeated extravasation of blood into joint
cavities, but its exact pathogenesis, particularly with regard to
early changes in the joint, is still incompletely understood.
This review presents recent findings obtained in experiments performed
in vitro and using animal models, which have improved our knowledge of
the pathogenesis of haemophilic arthropathy. These experimental
studies show that haemophilic arthropathy is a multifactorial event in
which the deposit of iron in the joints appears to exert a central
role.
First, iron may promote the apoptosis of chondrocytes by catalysing
the formation of oxygen metabolites; this may explain the fact that
intra-articular blood exerts a directly harmful effect on cartilage
before, and independent of synovial changes.
Secondly, iron may also act on the synovial membrane by favouring its
proliferation through the induction of proto-oncogenes involved in
cellular proliferation and stimulation of inflammatory cytokines as
well as abrogation of apoptosis.
These two processes, one degenerative and cartilage-mediated, the
other inflammatory and synovium-mediated could occur in parallel or
sequentially.
Overall, it may be expected that these experimental results will yield
new therapeutic strategies capable of effectively preventing the
occurrence of this still serious and common complication in patients
with severe haemophilia.
Keywords: arthropathy; blood; cartilage; haemophilia; joint;
synovitis
Document Type: Research article
DOI: 10.1111/j.1365-2516.2008.01732.x
Affiliations: 1: Department of Rheumatology and Clinical Immunology,
University Medical Center Utrecht, Utrecht, The Netherlands 2:
Department of Immunology and Rheumatology, Hôpital Edouard Herriot,
Lyon, France 3: Hemophilia and Thrombophilia Center, Rush University
Medical Center, Chicago, IL, USA
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