Haemophilic arthropathy

Dr Craig Hacking and Dr Andrew Dixon et al.

Haemophilic arthropathy refers to permanent joint disease occurring in hemophilia sufferers as a long-term consequence of repeated hemarthrosis. Around 50% of patients with hemophilia will develop a severe arthropathy.

Hemophilia is an X-linked recessive disease affecting males. Haemarthroses may be spontaneous or result from minor trauma and typically first occurs before the age of two and continues to occur into adolescence. It is usual for the same joint to be repeatedly involved. In adulthood, haemarthroses are uncommon. However, proliferative chronically-inflamed synovium results in the development of haemophilic arthropathy.

There is synovial hyperplasia, chronic inflammation, fibrosis, and hemosiderosis. The synovium mass erodes cartilage and subchondral bone leading to subarticular cyst formation 3.

Haemophilic arthropathy is often monoarticular or oligoarticular. Large joints are most commonly involved in the following order of frequency 2:

  1. knee
  2. elbow
  3. ankle
  4. hip
  5. shoulder
  • joint effusion is seen in the setting of hemarthrosis
  • periarticular osteoporosis: from hyperemia
  • epiphyseal enlargement with associated gracile diaphysis: from hyperemia (appearances can be similar to juvenile rheumatoid arthritis and paralysis)
  • secondary degenerative disease: symmetrical loss of joint cartilage involving all compartments equally with periarticular erosions and subchondral cysts, osteophytes and sclerosis
  • knee 3
  • elbow 2
    • enlarged radial head
    • widened trochlear notch
  • ankle 1
    • talar tilt: relative undergrowth of the lateral side of the tibial epiphysis leads to a pronated foot
  • good for detection of early disease
  • thickened synovium with low signal due to hemosiderin susceptibility effect: siderotic synovitis
  • enhancing synovium due to synovitis
  • joint effusion
  • cartilage loss and erosions can be well seen
  • bone scan
    • sensitive for detecting areas of disease over the entire skeleton
    • follow-up scans can monitor treatment response
  • radiosynoviorthesis
    • radioisotopes can be injected therapeutically into a joint to decrease bleeding and synovitis
    • Rhenium186 is emerging as the preferred isotope over Phosphorus32 and Yttrium90 particularly in medium-sized joints 5

Early factor VIII or IX replacement may prevent or delay joint destruction. Radiosynoviorthesis has been shown to be effective in reducing bleeding and effusion in selected cases. Surgical arthrodesis 6 or joint replacement 7 can be an effective treatment for the end-stage disease.

Consider:

  • mono or oligoarticular involving large joints
  • joint effusion: may show fluid-fluid levels related to the blood products
  • chronic haemarthroses and hyperemia leading to growth deformities  (e.g. epiphyseal enlargement, widened intercondylar notch of the knee )
  • chronic periarticular erosions and subchondral cysts
Arthritides
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Article information

rID: 9545
Synonyms or Alternate Spellings:
  • Hemophilic arthropathy

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Cases and figures

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  • Case 2
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  • Case 3: left side
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  • Case 6
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  •  Case 7
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  • Case 8: elbow haemophilic arthropathy
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