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Haemophilic Arthritis 588 Annals ofthe Rheumatic Diseases 1991; 50: 588-591 REVIEW Ann Rheum Dis: first published as 10.1136/ard.50.8.588 on 1 August 1991. Downloaded from Haemophilic arthritis Rajan Madhok, John York, Roger D Sturrock Haemophilic arthritis is a relatively rare arthro- 90% of severely affected patients have this pathy, but several aspects of its management complication.2 Three stages are recognised: and pathogenesis are of interest to rheuma- acute haemarthrosis, chronic synovitis, and tologists. Firstly, the number of haemophiliac degenerative arthritis. patients has increased simultaneously with Haemarthroses occur most commonly into availability of clotting factor. concentrates, and knees, elbows, and ankles. Patients are often most severely affected patients are likely to have aware of a bleed before any clinical signs. Once arthritis amenable to prevention or amelioration a haemarthrosis is established the joint is tense, by approaches familiar to rheumatologists. painful, and held in flexion. One joint often Secondly, the pathogenesis is unclear, and a becomes a focus for recurrent bleeding. further understanding may provide insights into Onset of the synovitic stage is often difficult the responses of joint tissues to injury. Thirdly, to establish. The patient presents with persis- the histopathology of haemophilic arthritis tent swelling in the absence of pain in a target represents one extreme of changes seen in joint. Swelling is due to synovial hypertrophy rheumatoid synovium, and thus haemophilic and usually refractory to clotting factor concen- arthritis may highlight common mechanisms in trate infusions. Joint aspiration may show an joint destruction. inflammatory exudate mixed with blood. Early degenerative arthritis is considered to be present if chronic synovitis persists for more Why haemophiliac patients bleed into joints than six months.3 Haemophilia describes two disorders-haemo- philia A due to factor VIlIc deficiency and haemophilia B due to factor IX deficiency. Both Pathology factors are glycoproteins, encoded by X Acute joint bleeding produces a transient acute http://ard.bmj.com/ chromosome genes that form part of the intrin- inflammatory synovitis, and resolution of these sic plasma clotting system. Specifically, factors changes is often slow.4 With repeated haem- VIIIc and IX activate factor X. The clinical arthroses synoviocyte hypertrophy and hyper- manifestations of both disorders are therefore plasia supervene, which are supported by an similar. intense neovascularisation of the synovial mem- The predisposition to musculoskeletal bleed- brane. A characteristic feature is deposition of ing in haemophilia compared with other con- iron in superficial and subsynovial layers. A on September 25, 2021 by guest. Protected copyright. genital and acquired coagulation disorders can- perivascular mononuclear cell infiltrate and not be entirely explained by a single cause. fibrosis is also present. At the joint periphery Mechanical factors are important as suggested the inflamed synovium is organised into a by the onset of haemarthrosis with weight fibrous pannus, which is locally invasive. bearing; more frequent occurrence of bleeds Cartilage loss also occurs in weightbearing into legs than arms; and the propensity of joints areas, and at such sites chondrocytes contain on the dominant side to be more severely haemosiderin. In the later stages there is com- affected. The inherent absence of tissue plete loss of cartilage and subchondral bone is thromboplastin in synovium combined with an exposed with cyst formation. This is followed impaired intrinsic coagulation pathway may be by collapse and sclerosis of the joint. another factor.' The early pathological features have been described in spontaneous haemophilia in dogs, and changes similar to haemophilic arthritis Clinical manifestations have been reproduced in experimental animals The frequency of bleeding episodes parallels with injections of autologous blood`'0 Some plasma factor concentrations.2 Severe defects aspects of haemophilic arthritis remain unclear, (<1 1U/1) are characterised by spontaneous however. Firstly, the response of joint tissues to bleeding-the most common sites being joints, intra-articular blood is incompletely under- Centre for Rheumatic muscles, and renal tract. Factor concentrations stood-changes include synovitis and sub- Diseases, University 1 in after but the component or Department of Medicine, between and 5 IU/l result bleeding synovial angiogenesis, 10 Alexandra Parade, minor trauma. In mild deficiency (5-50 IU/1), components of blood that initiate and per- Glasgow G31 2ER bleeding occurs after severe trauma or surgery. petuate synovitis are not known. Secondly, R Madhok concentrations remain stable throughout mediators participating in cartilage breakdown J York Factor R D Sturrock life, except in rare variants. are uncertain. Thirdly, the way in which iron most enters chondrocytes is not yet explained. An Correspondence to: Joint disease is the single important Dr Madhok. cause of morbidity in haemophilia and up to understanding of these mechanisms may Haemophilic arthris 589 provide insights into the pathogenesis of more arthritis, but the incidence has increased common inflammatory arthropathies, such as in patients with HIV-1 related immuno- Ann Rheum Dis: first published as 10.1136/ard.50.8.588 on 1 August 1991. Downloaded from rheumatoid arthritis. deficiency. 18 Haemophilic synovitis represents one Systemic corticosteroid treatment has been extreme of the continuum of histological evaluated in dampening the intra-articular in- changes present in rheumatoid arthritis. In both flammatory response in acute haemarthrosis, rheumatoid and haemophilic arthritis it has but the benefits are short lived and because of been suggested that iron perpetuates synovitis frequent side effects their use is limited and not and mediates tissue damage.'0'2 In both rheu- advocated. " matoid and haemophilic arthritis microbleeding Although treatment for haemophilia has is well recorded, and synovial iron deposition greatly improved, the influence of clotting correlates closely with the extent of erosive factor replacement therapy on the progression disease and is associated with a poorer prog- of haemophilic arthritis is not known. Two nosis.'3 4 Differences include the relative uncontrolled studies reported that arthritis absence of a chronic inflammatory response and progresses despite adequate early treatment of the presence of iron with chondrocytes in haemarthrosis, and even patients receiving haemophilic arthritis.'5 A possible sequence of prophylactic infusions develop episodes of events in haemophilic arthritis may be that synovitis.20 21 To determine further the intra-articular bleeding initially provokes a non- influence of clotting factor on the outcome of specific inflammatory response, and macro- arthritis in haemophilia an international co- phages accumulate around synovial iron operative study has been started. An interim deposits, release monokines, and stimulate analysis at four years showed that progression production of latent collagenases and pros- seems to be directly related to the frequency of tanoids from synovium. With repeated haem- joint bleeds. Treatment with dose regimens of arthroses the synovium proliferates, and the greater than 1000 units/kg a year confers some continued leak of red blood cells from the advantage in progression, but more than 2000 hypertrophied, vascular synovium produces a units/kg a year offer no additional advantage. cycle of synovitis--bleeding--synovitis. The The early use of clotting factor concentrates, increased iron load results in further synovio- therefore, seems to delay rather than prevent cyte hyperplasia, pannus formation, and macro- haemophilic arthritis. A small subgroup of phage accumulation. patients was identified in whom no joint disease The cartilage loss is multifactorial, initiated was found. These patients had used signifi- by sequestered polymorphonuclear cells and the cantly more clotting factor concentrate and had increased amounts of plasmin in the inflamed been receiving prophylactic treatment more synovium," and perpetuated by the synovial often than those who had progressive disease response, mediating damage directly in areas (Aledort, personal communication). where pannus forms.'0 In areas free from On the other hand, the lifelong dependence pannus the deposition of iron within chrondro- on blood products has placed patients with http://ard.bmj.com/ cytes may play an important part.'0 15 haemophilia at increased risk of blood borne viral infections. Hepatitis due to hepatitis B and the non A, non B agents is invariably found in Treatment regularly treated haemophiliac patients, and The aims in treating haemophilic arthritis are chronic liver disease is an increasingly common similar to those of any inflammatory arthro- cause of morbidity.22 23 HIV-1 induced pathy: symptom relief, prevention of the pro- immunodeficiency is now the most common on September 25, 2021 by guest. Protected copyright. gression of the joint damage, and maintenance cause of death in patients with severe haemo- of function. In haemophilic arthritis these goals philia.24 The risk of infections with these agents can be achieved by early treatment of haem- in the future has been significantly reduced, arthrosis and by limiting the effects of chronic however, with newer methods of fractionating synovitis. In the subject with degenerative and sterilising clotting factor concentrates.
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