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 Factor XII Factor IX Factor XI Factor VIII activated on activated on foregin platelet Surface surface Extrinsic pathway Tissue factor VII (tissue thromboplastin)

Common pathway

Factor X Factor V PF3 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.

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 Arthritis With Leukemia: Acute monoarticular mimicking acute rheumatic fever may occur, particularly in acute monocytic leukemia.