Haemophilic Arthritis | An Introduction

Introduction:

Haemephilia include a group of disease affecting males but transmitted by female and characterized by prolonged coagulation and a life long tendency to excessive bleeding.

Types:

1. Haomophilia A (factor VIII deficiency)

2. Haemophilia B (Christmas disease factor IX deficiency)

3. Von willebrallds disease: There is abnormality of platelet function.

Grades of factor VIII deficiency:

Grade I: < 1 % severe bleeding

Grade II: < 5% gross bleeding with minor trauma

Grade III: < 5 -25% severe bleeding after trauma or surgery

Grade IV: < 25 -50% bleeding after major trauma or surgery

Rheumatological manifestations:

1. Haemaphilic arthropathy

2. Intramuscular or soft tissue haemorrhege (may cause pseudotumor or compartment syndrome)

3. Septic arthritis.

a. Haemophilic arthropathy:

· Acute hemarthrosis: It usually starts when child begins to walk characterized by pain, increased temperature swelling and restricted motion. Severity and recurrences is dependent on factor levels (if there are 75% normal, there is a lesser tendency towards haemarrhage).

· Sub acute haemophilic arthropathy: It usually follows repeated episodes of joint bleeding characterized by features suggestive of chronic synovitis, It is accompanied with muscle laxity and ligament laxity. Polyarticular involvement is uncommon.

· Chronic haemophilic arthropathy: The main feature are of joint deformity, fibrous ankylosis and osteophyte over growth.

Extra skeletal manifestations:

1. Sub cutaneous bleeding with tendency to bruise easily.

2. Mucous membrane and internal bleeding

· Haematuria

· Epistaxis

· Bleeding into brain or spinal cord

· Mouth, gums, lips and tongue bleeding.

Diagnosis:

1. Family history: Especially suggesting a sex linked disorders.

2. Preliminary cougulation testing: Tests include APTT, PT, thrombin clotting time (TCT) and bleeding time. In haemophilia A & B, APTT is prolonged, if bleeding time is prolonged one should consider diagnosis of Von willebrands disease.

3. Specific factor essay: To differentiate between haemophilia A & B.

Radiological staging of haemophilic arthritis:

Stage I: Soft tissue swelling

Stage II:

• Soft tissue swelling

• Osteopenia of epiphyses

• Joint space maintained

• Subchondral cysts are present

• Squaring of the patella

• Intercondylar notch femur and trochlear notch of ulna widened

Stage III:

• Disorganization of joint

• ? Ankylosis

• Large subchondral cysts

Management of haemophilia - A:

1. Local haemostatic agents: Such as thrombin or gelfoam if bleeding site is accessible.

2. Factor VIII replacement:

a. Cryoprecipitate: it is stored frozen

b. Freeze dried factor VIII concentrates -the advantages being ability to store in domestic refrigerator and higher purity.

3. Non blood products such as synthetic vasopressin may be used in mild to moderate cases.

4. General care: It includes

a. Prophylactic immunization

b. Correction of anaemia if present

c. To avoid bleeding including drugs such as aspirin.

5. Treatment of acute haemarthritis:

a. Cold application

b. Analgesics

c. Joint immobilization

d. Replacement of coagulation factor

e. Gradual physiotherapy

6. Treatment of chronic arthropathy:

a. Prophylactic factor replacement to prevent recurrences of bleeding.

b. Rest

c. Gradual physiotherapy

d. Intraarticular gluco corticoids to reduce symptoms and recurrent hemathrosis.

e. Synoviectomy for chronic synovitis unresponsive to conservative therapy.

f. For recent contractures: Plaster splinting, dynamic traction, exercises.

g. Postsubluxation of tibia: dynamic traction

h. Painful unstable joints: Orthotic splintage

i. Supracondylar osteotomy for severe FFD knee

j. TA lengthening for TA contractures

k. Total joint replacement in cases with advanced joint destruction.

l. Arthrodesis in severe destructive arthritis

m. ORIF for fracture nonunion.

Arthritis With Leukemia:

Acute monoarticular mimicking acute rheumatic fever may occur, particularly in acute monocytic leukemia.