Hemophilic arthropathy is caused by repetitive bleeding into joints (hemarthroses) in patients with hemophilia. It most commonly affects the knees, elbows, ankles and shoulders of young males. Over time, the bleeding leads to progressive joint damage and degeneration. Treatment involves controlling bleeds with factor replacement, analgesics, and sometimes surgery like joint replacement for severe cases. The prognosis depends on the severity of the underlying factor deficiency and presence of inhibitors against replacement factors.

1. HEMOPHILIC ARTHROPATHY
DR AKHIL JOSE

2. • systemic arthropathy
• most commonly caused by hemophilia
• characterized by repetitive hemarthroses and
progressive joint disease.
• Diagnosis - recurrent atraumatic hemarthroses in a
patient with a bleeding disorder such as hemophilia A,
hemophilia B or von Willibrand's disease.
• Treatment -compressive dressings, analgesics, and
bleeding factor administration.
• Joint arthroplasty is indicated in cases of severe end-
stage degenerative joint disease.

3. EPIDEMIOLOGY
• young males
• affects patients between 3-15 years old
• knee is most commonly affected
• elbow, ankle, shoulder and spine are also
involved

4. Pathophysiology
Root bleeding disorder may be
A)hemophilia A
• X-linked recessive
• decrease factor VIII
B)hemophilia B - Christmas disease
• X-linked recessive
• decreased factor IX
C)von Willebrand's disease
• rare cause of joint bleeds
• more commonly mucosal bleeding
• autosomal dominant
• abnormal factor VIII with platelet dysfunction

11. PATHOANATOMY
• persistent minor trauma
• synovitis -> enzyme based cartilage
destruction -> joint deformity

14. ASS CONDITIONS
• hemarthrosis
• intramuscular hematoma (pseudotumor)
• nerve compression(femoral nerve palsy -iliacus
hematomas)
• leg length discrepancy(epiphyseal overgrowth)
• Fractures( generalized osteopenia)
• medical conditions and comorbidities-HIV( 90% in
hemophiliacs)

15. CLASSIFICATION
•Arnold-Hilgartner Staging
•Stage 1 •Shows swelling of the soft tissues
•Stage 2 •Shows osteoporotic changes
•Stage 3
•Shows development of
subchondral cysts
•Joint is grossly intact
•Stage 4
•Shows cartilage loss with
narrowing of the joint
•Stage 5
•Demonstrates severe arthritis of
affected joint

18. CLINICAL PRESENTATION
• painful range of motion of joints
• hemarthrosis
• M.C –KNEE JOINT
• Acute- painful and tense joint effusion
• Subacute-occurs after two prior bleeds
• Chronic- contractures or arthritis
• Paresthesias- L4 distribution( iliacus
hematoma -femoral nerve)

26. IMAGING
KNEE
• squaring of patella and femoral condyles (Jordan's sign)
• ballooning of distal femur
• widening of intercondylar notch
• joint space narrowing
• patella appear long and thin on lateral
ANKLE AND ELBOW
• joint arthritis
• epiphyseal overgrowth
• generalized osteopenia
• fractures

27. • Plain radiograph
• 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
– widened intercondylar notch
– squared inferior margin of the patella
– bulbous femoral condyles
– flattened condylar surfaces
– changes can be classified through the Arnold-Hilgartner classification 4
• 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

31. MRI
can be used to identify early
degeneratve joint disease
Ultrasound
often helpful to follow intramuscular
hematomas

32. • Labs
• Screening tests-platelet count,BT,PT,APTT
• screening for factor VIII inhibitors (including IgG
antibodies)
• indicated prior to surgery as presence will negate
effects of factor treatment
• Prefered test-platelet aggregometry
• Histology-hypertrophy and hyperplastic changes
to the synovium

33. • Nuclear medicine
• bone scintigraphy
– 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

35. D/D s
• Septic arthritis
• concomitant infection should be ruled out by
physical exam and joint aspiration

36. • Differential diagnosis
• juvenile rheumatoid arthritis (plain
radiograph)
• paralysis
• tenosynovial giant cell tumor (MRI)
• synovial osteochondromatosis (MRI

37. • TREATMENT
• Nonoperative
• compressive dressings, analgesics, short term
immobilization followed by rehabilitation
• indications
• joint pain
• modalities
• steroids for to help reduce inflammation
• splints and braces
• physical therapy to prevent contracture development

42. • factor administration
• indications
• vigorous physical therapy
• increase factor VIII to 20%
• acute hematomas (including intramuscular hematomas)
• increase factor VIII to 30%
• acute hemarthrosis and soft tissue surgery
• increase factor VIII to 40-50%
• skeletal surgery
• increase factor VIII to 100% for first week following surgery then maintain at > 50% for second week
following surgery
• modalities
• home transfusion therapy
• has reduced the severity of arthropathies
• desmopressin
• indications
• mild or moderate hemophillia A

50. Operative
• synovectomy
• indications
• recurrent hemarthroses recalcitrant to medical management
• techniques
• increase factor VIII to 40-50%
• outcomes
• decreases incidence of recurrent hemarthroses
• limits pain and swelling
• synoviorthesis
• indications
• chronic hemophiliac synovitis that is recalcitrant to medical management
• technique
• destruction of synovial tissue with intra-articular injection of radioactive agent
• colloidal phosphorus-32 chromic phosphate

53. • total joint arthroplasties
• indications
• end stage arthropathy
• perioperative care
• increase factor VIII to 100% for first week postoperatively
then maintain at > 50% for second week postoperatively
• arthrodesis
• indications
• arthropathy of the ankle
• perioperative care
• increase factor VIII to 100% for first week postoperatively
then maintain at > 50% for second week postoperatively

54. PROGNOSIS
• Prognostic variables
• degree of factor deficiency
• determines severity of disease
• mild: 5-25%
• moderate: 1-5%
• severe: 0-1%
• presence of factor VIII inhibitors (including IgG antibodies)
• IgG antibody inhibits response of therapeutic factor
treatment (monocolonal recombinant factor VIII)
• found in 5-25% of hemophiliac patients
• is a relative contraindication for surgical interventions
• should be screened for preoperativel

60. jra