About The Author

Dr Manoj R. kandoi is the founder president of “Institute of Arthritis Care & Prevention”

an NGO involved in the field of patient education regarding arthritis. Besides providing

literature to patient & conducting symposiums, the institute is also engaged in creating

patients “Self Help Group” at every district level. The institute also conducts a certificate

course for healthcare professionals & provide fellowship to experts in the field of

arthritis.

The author has many publications to his credit in various journals. He has also written a

book “ The Basics Of Arthritis” for healthcare professionals.

The author can be contacted at:

Dr manoj R. kandoi

C-202/203 Navare Arcade

Shiv Mandir Road, Opposite Dena Bank

Shiv mandir Road, Opposite Dena bank

Shivaji Chawk, Ambarnath(E) Dist: Thane Pin:421501

State: Maharashtra Ph: (0251)2602404 Country: India

Membership Application forms of the IACR for patients & healthcare professionals

can be obtained from.

Institute of Arthritis Care & Prevention

C/o Ashirwad Hospital

Almas mension, SVP Road, New Colony,

Ambarnath(W) Pin:421501 Dist: Thane

State: Maharashtra Country: India

Ph: (0251) 2681457 Fax: (0251)2680020

Mobile ;9822031683

Email: drkandoi@yahoo.co.in

Preface:

Studies have shown that people who are well informed & participate actively in

their own care experience less pain & make fewer visits to the doctor than do other

people with arthritis. Unfortunately in India & many third world countries we do not

have patient education & arthritis self management programs as well as support groups.

This is an attempt to give a brief account of various arthritis, their prevention & self

management methods which can serve as useful guide to the patients of arthritis.

It would be gratifying if the sufferers of the disease knew most of what is given in the

book.

Acknowledgement\

I am thankful to Dr (Mrs) Sangita Kandoi for her immense help in proofreading & for her

invaluable suggestions. The help rendered by Nisha Jaiswal is probably unrivalled.

Thanks also to vidya, praveen, rizwana and parvati for their continous support

throughout the making of the book. The author is grateful to his family for the constant

inspiration they offered. The author alone is responsible for the shortcoming in this piece

of work. He welcomes suggestions for improvement from the readers.

Haemophilic Arthritis

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.

Process of blood caugulation:

Intrinsic pathway Extrinsic pathway

Factor XII Factor IX Tissue factor VII

Factor XI Factor VIII (tissue thromboplastin)

activated on activated on

foregin platelet

Surface surface Factor X

Factor V

PF3

Common pathway Ca

Prothrombin

Thrombin

Fibrinogen Fibrin

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

Pathophysiology of haemophilic arthropathy:

Haemorrhage in the joint (Spontaneous or following trauma)

Blood remain fluid in the joint due to lack of caugulation

Plasma absorbed RBC phagocytosis

Iron deposition in synovial

Tissue and articular cartilage

Chronic inflammation with

Synovial proliferation and

Pannus formation

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.

Rheumatologic manifestations of sickle cell anaemia: 1. Bone infarction

2. Septic arthritis

3. Osteomyelitis

4. Hemarthrosis

5. Hyperuricemia and gout

6. Vascular necrosis of bone

7. Hand foot syndrome

8. Muscle necrosis