Haemotologic Joint Diseases:
Bleeding into joint.
Due to- or increased risk in- haemophilia (see below); trauma (eg injury, fracture, post-operative, post-joint aspiration); connective tissue disorders (eg Marfan’s syndrome); neoplasm (eg pigmented villonodular synovitis), scurvy, sickle cell disease, septic arthritis, Charcot’s neuroarthropathy, warfarin therapy
Rest joint, aspiration, ice, analgesia
• common feature of haemophilia – more common in knee, ankle and elbow
• haemorrhage induces a synovial proliferation, chronic inflammation release of degradative proteinases joint damage. Recurrent bleeding irreversible damage
• Stage 1 – acute haemoarthrosis in child when begin to walk – pain, tenderness, warmth, limited range of motion, distended joint capsule. Often have prodromal symptoms of stiffness and warmth in the joint.
• Stage 2 – subacute or chronic arthritis – repeated haemorrhage self perpetuating chronic proliferative synovitis; haemosiderin accumulates in joint tissues; cartilage is degraded – joint is chronically swollen, warm but not as painful as acute – range of motion is decreased; crepitus present; muscles atrophied may have significant disability
• Stage 3 – ‘end-stage arthropathy’ - chronic destructive arthropathy with joint instability, fibrous ankylosis, osteophytic overgrowth
• develop secondary osteoarthritis (from cartilage degradation) and increased risk for septic arthritis (consider infection if haemoarthrosis fails to respond after factor replacement)
X-ray – in acute stage only see soft tissue swelling and effusions. Later radiodense effusions (due to haemosiderin accumulating in synovial membrane), osteoporosis, bone erosions, joint space narrowing (from denudation of cartilage)
Treatment – medical management of haemophilia – need prompt replacement of deficient factor; ice packs; joint immobilisation in extended position (within tolerance); joint aspirations; analgesics; patient education on strategies for prevention of trauma.
Long term – exercises/braces to improve joint stability, muscle strengthening, synovectomy (if synovitis unresponsive to pharmacological approaches). Joint arthroplasty if end stage.
Functional foot orthoses have been should to be very effective in reducing the ankle pain associated with haemophilic arthropathy
Sickle cell anaemia:
• may cause acute bone infarction/necrosis (in long bones – often multiple sites and sometimes symmetrical – present with bony tenderness and fever) and arthritis (polyarticular involvement of hands and feet common – bilateral and symmetrical – usually sudden onset)
• increased risk for gout (40% have hyperuricaemia), haemoarthrosis, septic arthritis, osteomyelitis, rhabdomyolysis
• dactylitis, due to vascular occlusions in bones of hands and feet acute painful, non-pitting swelling of hands and feet – called hand-foot syndrome
Management of osteonecrosis often unsatisfactory (due to microvascular occlusion from sickle cells). Need immediate non-weightbearing to prevent collapse of affected bone.