Blood-Induced Joint Damage in Hemophilia

 

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ABSTRACT

Hemophilia is a X chromosome-linked disease characterized by an increased tendency to hemorrhage. Because of recurrent hemarthrosis, specific changes occur in synovium and cartilage. This process is called hemophilic arthropathy. The pathogenetic mechanisms involved are not precisely known. Current concepts, which are based on experimental in vitro studies and clinical experience, hold that the synovium becomes catabolically active because of the exposure to blood components and as a result induces cartilage destruction. A considerable number of reports concerning blood-induced joint damage suggest that synovial changes have a leading role in the development of the joint damage and therefore precede the changes in cartilage. However, there are also observations that question whether this is the only and initiating mechanism of joint damage in hemophilia. These observers hold that intra-articular blood has a direct harmful effect on cartilage before synovial changes and suggest that joint damage may occur before synovial inflammation is evident. Primarily, there may be damage of articular cartilage with synovitis as a consequence. Some studies show that synovitis is involved but that it is not the only mechanism in the joint damage caused by intra-articular bleeding. These findings do not contradict the current concept of blood-induced cartilage damage in which synovial changes are thought to play an important role. Several pathological processes are possibly involved, some of them occurring in parallel and others sequentially. Possibly, intra-articular blood first has a direct effect on cartilage, and then it affects the synovium. Thus, both processes occur in parallel, and although they influence each other they probably do not depend on each other. This concept resembles degenerative joint damage as found in osteoarthritis.

REFERENCES

• 1 Van Creveld S, Hoedemaker P J, Kingma M J, Wagenvoort C A. Degeneration of joints in haemophiliacs under treatment by modern methods.  J Bone Joint Surg Br . 1971;  53 296-302
  PubMedGoogle Scholar
• 2 Madhok R, Bennett D, Sturrock R D, Forbes C D. Mechanisms of joint damage in an experimental model of haemophilic arthritis.  Arthritis Rheum . 1988;  31 1148-1155
  PubMedGoogle Scholar
• 3 Stein H, Duthie R B. The pathogenesis of chronic haemophilic arthropathy.  J Bone Joint Surg Br . 1981;  63B 601-609
  PubMedGoogle Scholar
• 4 Madhok R, York J, Sturrock R D. Haemophilic arthritis.  Ann Rheum Dis . 1991;  50 588-591
  PubMedGoogle Scholar
• 5 Hoaglund F T. Experimental hemarthrosis. The response of canine knees to injections of autologous blood.  J Bone Joint Surg Am . 1967;  49 285-298
  PubMedGoogle Scholar
• 6 Zeman D H, Roberts E D, Shoji H, Miwa T. Experimental haemarthrosis in rhesus monkeys: morphometric, biochemical and metabolic analyses.  J Comp Pathol . 1991;  104 129-139
  PubMedGoogle Scholar
• 7 Wolf C R, Mankin H J. The effect of experimental hemarthrosis on articular cartilage of rabbit knee joints.  J Bone Joint Surg . 1965;  47A 1203-1210
  PubMedGoogle Scholar
• 8 Roy S, Ghadially F N. Pathology of experimental haemarthrosis.  Ann Rheum Dis . 1966;  25 402-415
  PubMedGoogle Scholar
• 9 Mainardi C L, Levine P H, Werb Z, Harris E D. Proliferative synovitis in hemophilia: biochemical and morphologic observations.  Arthritis Rheum . 1978;  21 137-144
  CrossrefPubMedGoogle Scholar
• 10 Pelletier J-P, Martel-Pelletier J, Ghandur-Mnaymneh L, Howell D S, Frederick Woessner J. Role of synovial membrane inflammation in cartilage matrix breakdown in the Pond-Nuki model of osteoarthritis.  Arthritis Rheum . 1985;  28 554-561
  PubMedGoogle Scholar
• 11 Abrahams T G, Pavlov H, Bansal M, Bullough P. Concentric joint space narrowing of the hip associated with hemosiderotic synovitis (HS) including pigmented villonodular synovitis (PVNS).  Skeletal Radiol . 1988;  17 37-45
  CrossrefPubMedGoogle Scholar
• 12 France M P, Gupta S K. Non-haemophilic hemosiderotic synovitis of the shoulder. A case report.  Clin Orthop . 1991;  262 132-136
  PubMedGoogle Scholar
• 13 Morris C J, Blake D R, Wainwright A C, Steven M M. Relationship between iron deposits and tissue damage in the synovium: an ultra structural study.  Ann Rheum Dis . 1986;  45 21-26
  PubMedGoogle Scholar
• 14 Blake D R, Gallagher P J, Potter A R, Bell M J, Bacon P A. The effect of synovial iron on the progression of rheumatoid disease. A histologic assessment of patients with early rheumatoid synovitis.  Arthritis Rheum . 1984;  27 495-501
  PubMedGoogle Scholar
• 15 Roosendaal G, Vianen M E, Wenting M JG. et al . Iron deposits and catabolic properties of synovial tissue from patients with haemophilia.  J Bone Joint Surg Br . 1998;  80-B 540-545
  PubMedGoogle Scholar
• 16 Roosendaal G, Van Rinsum C A, Vianen M E. et al . Haemophilic arthropathy resembles degenerative rather than inflammatory joint disease.  Histopathology . 1999;  34 144-153
  CrossrefPubMedGoogle Scholar
• 17 Roosendaal G, Vianen M E, van den Berg M H, Lafeber F P, Bijlsma J W. Cartilage damage as a result of hemarthrosis in a human in vitro model.  J Rheumatol . 1997;  24 1350-1354
  PubMedGoogle Scholar
• 18 Roosendaal G, Vianen M E, Marx J JM. et al . Blood induced joint damage: a human in vitro study.  Arthritis Rheum . 1999;  42 1025-1032
  CrossrefPubMedGoogle Scholar
• 19 Lafeber F P, Vander Kraan M P, van Roy L J, Huber Bruning O, Bijlsma J W. Articular cartilage explant culture; an appropriate in vitro system to compare osteoarthritic and normal human cartilage.  Connect Tissue Res . 1993;  29 287-299
  PubMedGoogle Scholar
• 20 Niibayashi H, Shimizu K, Suzuki K. et al . Proteoglycan degradation in hemarthrosis. Intraarticular, autologous blood injection in rat knees.  Acta Orthop Scand . 1995;  66 73-79
  PubMedGoogle Scholar
• 21 Bates E J, Lowther D A, Handley C J. Oxygen free-radicals mediate an inhibition of proteoglycan synthesis in cultured articular cartilage.  Ann Rheum Dis . 1984;  43 462-469
  PubMedGoogle Scholar
• 22 Burkhardt H, Schwingel M, Menninger H, Macartney H W, Tschesche H. Oxygen radicals as effectors of cartilage destruction. Direct degradative effect on matrix components and indirect action via activation of latent collagenase from polymorphonuclear leukocytes.  Arthritis Rheum . 1986;  29 379-387
  PubMedGoogle Scholar
• 23 Roosendaal G, TeKoppele J M, Vianen M E. et al . Blood induced joint damage: a canine in vivo study.  Arthritis Rheum . 1999;  42 1033-1039
  CrossrefPubMedGoogle Scholar
• 24 Roosendaal G, TeKoppele J M, Vianen M E. et al . Articular cartilage is more susceptible to blood-induced cartilage damage at young age than old age.  J Rheumatol . 2000;  27 1740-1744
  PubMedGoogle Scholar
• 25 Convery F R, Woo S L, Akeson W H. et al . Experimental hemarthrosis in the knee of the mature canine.  Arthritis Rheum . 1976;  19 59-67
  CrossrefPubMedGoogle Scholar
• 26 Parsons J R, Zingler B M, McKeon J J. Mechanical and histological studies of acute joint hemorrhage.  Orthopedics . 1987;  10 1019-1026
  PubMedGoogle Scholar