Chronic Arthritis in Haemophilic Patients

What is haemophilic arthritis?

It is a common bleeding manifestation that occurs either spontaneously or from a mild trauma, but the haemarthroses becomes more spontaneous with time and repeated bleeding. The knees, elbows, ankles, hips and shoulders are the most common joints involved.

What are the causes of the disorder?

Haemophilia is a genetic (X-linked) recessive disease that causes abnormal bleeding. Bleeding occurs because the plasma in the person's blood has too little of a protein that helps the blood to clot.

Haemophilic arthropathy develops either spontaneously or from minor trauma that first occurs before the age of two and continues into adolescence. It is common for the same joint to be involved repeatedly. In adults, haemarthroses is not very common but a chronic inflammation of the synovium of the affected joint leads to the development of haemophilic arthropathy.

Haemophilic arthritis develops in three stages:

1. Acute haemarthrosis
2. Chronic synovitis
3. Degenerative arthritis

Following an incident of haemarthritis, when the synovium lining a joint is unable to absorb sufficient blood, recurrent bleeding into the joint results in the presence of blood breakdown products to a level that the synovial membrane cannot remove. It is believed that iron, a major constituent of red blood cells in the blood, plays a major role in inflaming the synovium. The breakdown product of haemoglobin promotes the production of pro-inflammatory compounds like cytokines and tumour necrosis factor (TNF), along with an increased vascularity and hypertrophy of the synovium.

The inflamed tissue becomes impinged between the surfaces of the joint and the inflammatory compounds further interfere with the normal maintenance of the articular cartilage (exposure of cartilage to blood leads to prolonged cartilage damage). These processes together result in a degenerative arthritis that progress towards joint destruction.

What one needs to know about symptoms or signs?

Signs and symptoms of haemophilic arthropathy include:

1. Swelling in joint
2. Joint pain
3. Joint fibrosis
4. Joint fusion
5. Erosion of joint cartilage
6. Bleeding into the joint spaces

Which specialist should be consulted in case of signs and symptoms?

Persons experiencing symptoms similar to haemophilic arthritis must consult an orthopaedic surgeon.

What are the screening tests and investigations done to confirm or rule out the disorder?

Diagnosis of haemophilic arthritis is made through:

1. Physical examination- The doctor examines the joints, gait, range of motion, muscle tone, level if disability, pain and swelling to assess the condition.
2. Imaging tests- Soft-tissue changes are detected through magnetic resonance imaging (MRI), which can detect joint effusion and synovial hypertrophy. A simpler, more convenient and radiation-free method for diagnosing haemophilic arthritis is contrast ultrasonography.

What treatment modalities are available for management of the disorder?

Treatment and management of haemophilic arthritis is done through early prevention and treatment of joint bleeds before the degenerative disease sets in. Treatment options include:

1. Replacing clotting factor- The level of clotting factor must be high to stop bleeding and prevent recurrence. Factor VIII or IX are replaced to prevent or delay joint destruction.
2. Analgesics - These medicines provide relief from pain that occurs due to bleeding into the joint. However, certain aspirin-containing drugs or other non-steroidal anti-inflammatory drugs can aggravate bleeding, which is why paracetamol or mild analgesics are given.
3. Anti-inflammatory medication - Once the acute haemarthrosis is contained, synovitis needs to be treated with intra-articular corticosteroid injections.
4. Rest, ice, compression and elevation (RICE) - Minor haemarthroses may not require immobilisation. However, rest, compression, ice treatment and elevation of affected joint helps combat pain in most patients. Ice packs can be applied every 4-6 hours for 20 minutes. The joint can be supported through slings, splints or pressure bandages. However, immobilization must occur only for as much time as is necessary, as long-term rest can lead to muscle atrophy.
5. Physical therapy - Physiotherapy is important to preserve movement and function in the affected joint, reduce swelling and pain, and maintain muscle strength. Physiotherapy can be started as soon as the patient can bear the exercise routine.
6. Joint aspiration - This helps to reduce the load of blood after a joint bleed, relieve pain and spasm, and speed up rehabilitation. The affected joint must be immobilised for an hour after the procedure.
7. Surgical treatment - Patients with severe joint impairment may require surgical intervention or when conservative therapies fail. Options include:

Synovectomy - This procedure does not remove the cause of synovitis but it helps prevent the progression of haemophilic arthropathy and development of end-stage arthropathy. Surgical synovectomy may be performed as an open procedure or through arthroscopy. Non-surgical synovectomies are minimally invasive procedures that may involve percutaneous injection of radioisotopes

(Radiosynovectomy) or chemical agents (chemical synovectomy) to generate fibrosis of the hypertrophied synovium. Radiosynovectomy is a quicker method of inactivating the synovium before the onset of irreversible joint destruction.

Joint debridement - This is a minimally invasive surgical procedure that removes synovitis and loose cartilage from the joint. The procedure can be done arthroscopically in young patients to prevent or delay the requirement of joint arthroplasty. The procedure extends the functional life of the joint.

Arthroplasty (Joint replacement surgery)- This is performed when the joint pain is intense enough to impact the quality of life. Most commonly replaced joints are the knees and hips.

Fusion (Arthrodesis) - This surgery involves the removal of the painful joint and fusion of the bones.

How can the disorder be prevented from happening or recurring?

The best method of preventing haemophilic arthritis in haemophilia patients is prophylaxis treatment with factor concentrates from an early age. This helps to reduce the risk of joint bleeds and arthropathy.

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