Pathological mechanism of joint destruction in haemophilic arthropathy
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MINI REVIEW ARTICLE
Pathological mechanism of joint destruction in haemophilic arthropathy Haijia Zhu1 · Yilin Meng1 · Peijian Tong2 · Shanxing Zhang2 Received: 25 September 2020 / Accepted: 30 November 2020 © The Author(s), under exclusive licence to Springer Nature B.V. part of Springer Nature 2020
Abstract Haemophilic arthropathy (HA), caused by intra-articular haemorrhage, is one of the most common complications in patients with haemophilia. Factor replacement therapy provides missing coagulation factors to prevent children with haemophilia from joint bleeding and decreases their risk for HA. However, haemophilia patients in developing countries are still suffering from HA due to insufficient replacement therapy. Symptoms such as pain and activity limitations caused by HA seriously affect the functional abilities and quality of life of patients with HA, causing a high disability rate in the haemophilia cohort. The pathological mechanism of HA is complicated because the whole pathological mainly involves hypertrophic synovitis, osteopenia, cartilage and bone destruction, and these pathological changes occur in parallel and interact with each other. Inflammation plays an important role in the whole complex pathological process, and iron, cytokines, growth factors and other factors are involved. This review summarizes the pathological mechanism of HA to provide background for clinical and basic research. Keywords Haemophilic arthropathy · Synovitis · Cartilage lesions · Bone destruction
Introduction Haemophilia is an inherited haemorrhagic disorder caused by the deficiency of specific coagulation factors (F VII, IX, etc.) [1]. More than half a million people worldwide are suffering from haemophilia [2], and most also suffer from complications of the disease; however, the mortality rate decreases for patients receiving treatment, such as prophylactic coagulation factor injection and blood transfusion [1, 2]. Haemophilic arthropathy (HA) is one of the most common complications due to severe haemophilia (coagulation factor VII or IX activity
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