Hemophilic arthropathy (HA) [1] [2] [54] [55]

Osteoarthritis (OA) [56] [57] [58]

Rheumatoid arthritis (RA) [59] [60] [61] [62] [63]

Shared features with HA

-

Ÿ Cartilage degeneration and osteophyte formation

Ÿ Synovitis, cartilage degradation, and bone resorption

Presentation

Ÿ Painful acute onset

Ÿ Joint swelling and damage

Ÿ Gradual joint swelling and enlargement

Ÿ Gradual painful joint swelling and damage

Pathophysiology

Ÿ Bleeding directly from synovial lining into the joint

Ÿ Mix of risk factors, including age, sex, injury, wear and tear, obesity, genetics

Ÿ Biochemical processes involving synovium, cartilage, and bone

Ÿ Genetic basis

Ÿ Chronic autoimmune disorder

Ÿ May also include systemic and extra-articular manifestations

Major processes

Ÿ Hemarthrosis-induced, widespread synovial inflammation

Ÿ Acute inflammatory mediators and iron-induced oxygen metabolites cause chondrocyte apoptosis and hemosiderin deposition

Ÿ Iron increases pro-inflammatory cytokines that increase pannus in synovium

Ÿ Widespread cartilage destruction and bone changes

Ÿ Synovial inflammation, mostly in regions adjacent to cartilage and bone damage

Ÿ Activated synovium releases cytokines that accelerate destruction of adjacent cartilage

Ÿ Autoimmune-mediated synovitis causes pannus (through angiogenesis), and leads to bone destruction

Ÿ Enzymes from synoviocytes and chondrocytes destroy cartilage

Age of onset

Ÿ First joint bleed usually occurs in infants (age < 2 years) in most severe cases (varies with severity and quality of prophylaxis)

Ÿ Present in 50% of people > 65 years of age with chronic knee pain

Ÿ Present in 3% - 4% of people 65 - 74 years of age, with prevalence of onset decreasing with age

Degree of damage

at equivalent age

Ÿ Greater than with OA or RA

Ÿ Less than with HA

Ÿ Less than with HA

Severity depends on:

Ÿ Number of hemarthroses

Ÿ Cumulative biomechanical stresses

Ÿ Access to medical care, factor infusion concentrate and physical therapy

Ÿ Age

Ÿ Time since onset

Ÿ Risk factors (see above)

Ÿ Age at diagnosis

Ÿ Access to disease-modifying drugs

Ÿ Smoking status

Ÿ Disease duration

Ÿ Joint erosions