Hemophilic Arthritis

Hemarthrosis symptoms vary with the severity of the hemorrhage. A larger hemorrhage initiates an acute inflammatory reaction in the joint, which becomes swollen, warm, very tender, and painful to move. Fever and leukocytosis are common associated findings. When joint hemorrhage and synovitis are mild, the arthritis may resolve completely in a few days; if they are severe, the joint inflammation may persist for several months. Excessive iron deposition in synovial lining cells from repeated hemarthroses results in a chronic arthritis that is characterized by villous proliferation of the synovium with few lymphocytes and significant fibrosis. Invasive pannus may develop, leading to cartilage destruction and erosion, creating a secondary osteoarthritis with permanent joint destruction and disability. Extensive bleeding into the muscles around the affected joint may cause hematomas that compress adjacent nerves or blood vessels, or both, further restricting joint motion.

Radiographic Findings. Soft tissue shadows indicate acute hemorrhage into the joint. After repeated hemarthroses, joint radiographs reveal cartilage thinning, narrowing of joint space, rough subchondral bone, marginal spurs, bone cysts, and a thick joint capsule. These same findings are seen in the older patient with osteoarthritis. Radiographic findings unique to hemophilic arthritis are soft tissue densities of hemosiderin deposits, hypertrophy of epiphyses adjacent to the affected joint, enlargement of the radial head, flattening of the articular surface (“squaring”) of the patella, slipped capital femoral epiphysis, and, sometimes, deformity or even destruction of the femoral head.

Treatment. Prophylactic clotting factor replacement therapy can decrease the frequency of hemarthroses and help prevent hemophilic arthropathy. Recombinant factor VIII therapy is administered prophylactically to children who have severe hemophilia (factor levels often < 1% of normal). This will reduce the rate of spontaneous bleeding in these patients. Desmopressin, a synthetic analog of vasopressin, can transiently stimulate increase production of factor VIII and can be used in patients with milder forms of the disease. Patients should make every effort to prevent joint trauma. Children and adults receiving prophylactic therapy may participate in sports with judicious supervision and precautions. High-contact sports should be avoided. Prompt treatment of acute hemarthrosis helps to minimize structural damage that can cause chronic joint disability. On demand factor replacement should be given. The affected joint should be immobilized immediately and ice packs and analgesic drugs used to reduce pain. NSAIDs that inhibit platelets are contraindicated.

After administration of a coagulation factor, blood from the distended joint can be aspirated to relieve pain and reduce articular damage. After the bleeding and synovitis have subsided, an active physical therapy program is started to restore full joint motion. Intra-articular injections of glucocorticosteroids may reduce joint pain and stiffness. Surgical synovectomy is useful to treat proliferative synovitis and joint damage but has significant morbidity. Arthroscopic synovectomy has fewer complications. Chemical synovectomy (intra-articular injection of osmic acid or other sclerosing agent) and radiation synovectomy (intra-articular injections of yttrium-90 or other agent) demonstrate short-term efficacy. Total joint arthroplasty should be considered for advanced joint disease.

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Jul 3, 2016 | Posted by in MUSCULOSKELETAL MEDICINE | Comments Off on Hemophilic Arthritis
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