Juvenile ra

1. Still’s Disease Synonym: Juvenile RA, Juvenile Chronic Polyarthritis, Juvenile Idiopathic Arthritis

2. definition Juvenile Rheumatoid Arthritis (JRA) is defined as a chronic condition causing joint inflammation for at least 6 weeks in a child 16 years of age or younger . June 11, 2012 Ratan M.P.T., (Ortho & Sports) 2

3. introduction JRA is a term used to describe a common type of arthritis in children . JIA is a subset of arthritis seen in childhood, which may be transient and self-limited or chronic. It differs significantly from arthritis commonly seen in adults (OA,   RA), and other types of arthritis in childhood which are chronic conditions  (e.g.  psoriatic arthritis  and   AS ). It is an autoimmune disorder resulting in joint pain & swelling . June 11, 2012 Ratan M.P.T., (Ortho & Sports) 3

4. Introduction cont… It is an inflammatory condition occurs during childhood or adolescence & affects one or more joints, although it can also affect other organ systems (particularly the eyes). It tends to affect major joints rather than smaller joints of hands & feet as with primary chronic adult form. Atlantoaxial subluxation can be a concomitant problem. The course of the disease is very variable & the prognosis is good ( particularly if only a small number of joints are involved) in 80% of cases. June 11, 2012 Ratan M.P.T., (Ortho & Sports) 4

5. Historical background, occurrence The disease was described by G. F. Still in 1897. The incidence of JRA is approx. 3–5 new cases per 100,000 children under 15 years of age. JRA usually occurs before age 16 & symptoms may start as early as 6 months old. Substantial geographical differences exist, with illness occurring more frequently in northern countries. The male to female ratio is 1:2.5. June 11, 2012 Ratan M.P.T., (Ortho & Sports) 5

6. Etiology It is UNKNOWN but immunological , genetic, climatic, infectious & psychological factors are propose etiological factors . Immune system: Some children with JRA, particularly severe forms, show anomalies of the immune system e.g . antinuclear antibodies or hypogamma-globulinemia . Autoantibodies, abnormal antigen-antibody complexes & other anomalies detectable in the lab also occur. June 11, 2012 Ratan M.P.T., (Ortho & Sports) 6

7. Etiology cont… Genetic & Climate components : T here is a North-South differential in the frequency of the disease which is associated with climate. The condition is also widespread in those hot countries like New Zealand, Australia. Common in UK with its damp, cold climate Infection:. Microorganisms such as Chlamydia trachomatis, Yersinia enterocolitica and Mycoplasma fermentans have also been discussed as the possible cause of JRA. A bacteria-specific, synovial cellular immune response has been observed. June 11, 2012 Ratan M.P.T., (Ortho & Sports) 7

8. Etiology cont … Psychological factors: It play a role in the manifestation of disease , as children have often reported as being in stressful situation prior to its onset. Children with JRA also tend to be rather reserved and seem to have difficulty in expressing their problems & conflicts . June 11, 2012 Ratan M.P.T., (Ortho & Sports) 8

9. Pathology JRA is generalized disorder of connective tissue affecting – Articular structure & Extra articular structures June 11, 2012 Ratan M.P.T., (Ortho & Sports) 9

10. Articular Changes Stage I: Inflammation of the synovial membrence spreads to articular cartilage & other soft tissues. Limitation of joint movt with pain & muscle spasm June 11, 2012 Ratan M.P.T., (Ortho & Sports) 10

11. Articular Changes cont… Stage II : Granulation tissue formation within synovial membrence & spread to periarticular tissue. Cartilage disintegration & joint filled with granulation Thickening of joint capsule, tendon (with sheaths) & impaired joint movt permanently. June 11, 2012 Ratan M.P.T., (Ortho & Sports) 11

12. Articular Changes cont … Stage III : Granulation tissue converted into fibrous tissue with adhesion formation between tendon, joint capsule & articular surface. Articular surface cover partly by cartilage & partly by fibrous tissue. June 11, 2012 Ratan M.P.T., (Ortho & Sports) 12

13. Articular Changes cont… Stage IV: Permanent joint damage and deformity Disability June 11, 2012 Ratan M.P.T., (Ortho & Sports) 13

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15. Extra articular changes Nodule formation: In the pressure area & may be subcutaneous or intracutaneous. They may present in organs such as lung & heart. Vascular changes: It constitute inflammation of all size arteries. The lumen of small vessels can become obliteration. June 11, 2012 Ratan M.P.T., (Ortho & Sports) 15

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17. Oligoarticular (or pauciarticular ) JIA Oligoarticular is used with JIA terminology, and pauciarticular is used with JRA terminology. It affects 4 or fewer joints in first 6 months of illness.  O ligoarticular JIA – Often   ANA  positive, when compared to other types of JIA . Accounts for about 50% of JIA cases . Usually involves the large joints such as the knees, ankles & elbows but smaller joints (such as the fingers and toes) may also be affected. June 11, 2012 Ratan M.P.T., (Ortho & Sports) 17

18. The hip is not affected unlike polyarticular JIA. It is usually not symmetrical Length discrepancy & muscles atrophy often happens which leads to asymmetric growth and risk of flexion contracture. Early childhood onset are at risk for developing a chronic iridocyclitis or an anterior uveitis (inflammation of the eye). June 11, 2012 Ratan M.P.T., (Ortho & Sports) 18

19. This condition often goes unnoticed; therefore these children should be closely monitored by an ophthalmologist. If ANA+, patient need routine eye exam every 3 months. If ANA- and older than 7 years old, can have eye exam every 6 months. Late childhood onset are at risk for sacrolitis and spondyloarthropahty. June 11, 2012 Ratan M.P.T., (Ortho & Sports) 19

20. Polyarticular JRA Affecting 5 or more joints in first 6 months of disease. This subtype can include the neck and jaw as well as the small joints usually affected. It is more common in girls than in boys. Usually the smaller joints are affected in polyarticular JIA, such as the fingers and hands, although weight-bearing joints such as the knees, hips, and ankles may also be affected. June 11, 2012 Ratan M.P.T., (Ortho & Sports) 20

21. The joints affected are usually symmetrical Children with polyarticular JIA are also at risk for developing chronic iridocyclitis or uveitis and should also be monitored by an ophthalmologist. Rh factor may be positive in polyarticular JIA and is rarely positive in children with systemic JIA . June 11, 2012 Ratan M.P.T., (Ortho & Sports) 21

22. Systemic JRA It is also known as "systemic onset JRA”. Characterized by arthritis,  fever  & a salmon pink  rash . It affects males and females equally It generally involves both large & small joints. Systemic JIA can be challenging to diagnose because the fever and rash come and go. Fever – Can occur at the same time every day or twice a day Often in late afternoon or evening with spontaneous rapid return to baseline (vs. Septic Arthritis of continuous fever) June 11, 2012 Ratan M.P.T., (Ortho & Sports) 22

23. The rash – Often occurs with fever. It is a discrete, salmon-pink macules of different sizes. It migrates to different location on skin, rarely persists in one location more than one hour. The rash commonly seen on trunk and proximal extremities or over pressure areas . June 11, 2012 Ratan M.P.T., (Ortho & Sports) 23

24. Systemic JIA may have  internal organ  involvement : Hepatosplenomegaly , Lymphadenopathy, Hepatitis, Tenosynovitis, etc. A polymorphism in  macrophage migration inhibitory factor  has been associated with this condition . It is sometimes called "adolescent-onset Still's disease", to distinguish it from  adult-onset Still's disease . However , there is some evidence that the two conditions are closely related . June 11, 2012 Ratan M.P.T., (Ortho & Sports) 24

25. Other types OF JRA Some doctors include two other, less common forms: Enthesitis -related arthritis & Psoriatic JIA. Enthesitis – It is an inflammation of the insertion points of the tendons. This form occurs most often in boys older than girls, characteristically causes back pain, and is linked to  ankylosing spondylitis  and  inflammatory bowel disease . Psoriatic JIA – O ften in girls, in conjunction with  psoriasis , although joint problems may precede the skin manifestations by several years . June 11, 2012 Ratan M.P.T., (Ortho & Sports) 25

26. Summary of Symptoms Joint swelling Pain Stiffness Limping Limited movement Slow movement Fever Skin rash June 11, 2012 Ratan M.P.T., (Ortho & Sports) 26

27. Complications Wearing away or destruction of joints Slow rate of growth Uneven growth of an arm or leg Loss of vision or decreased vision from chronic uveitis (may be severe, even before arthritis is not very severe) Anaemia Swelling around the heart (pericarditis) Chronic pain Poor school attendance June 11, 2012 Ratan M.P.T., (Ortho & Sports) 27

28. Investigations Acute phase reactants (APRs) Erythrocyte sedimentation rate (ESR) C-reactive protein (CRP) Full blood count (FBC) Rheumatoid factor (RF) Antinuclear antibody (ANA) Urea & electrolytes (U&E) Liver function tests (LFT) June 11, 2012 Ratan M.P.T., (Ortho & Sports) 28

29. Investigations cont… Uric acid/ Synovial fluid analysis Urinalysis Bone marrow examination Thyroid function (TSH, T3,T4) Hepatic enzymes (SGOT, SGPT, alkaline phosphatase) Muscle enzyme (CPK,) June 11, 2012 Ratan M.P.T., (Ortho & Sports) 29

30. Differential diagnosis Joint effusions occur in a range of diseases e.g . hemophilia or suppurative arthritis , but also in other rare conditions such as enthesopathic arthritis , leukemia , systemic lupus erythematodes and rheumatic fever . A traumatic cause must also be ruled out. As tumor -like lesions, pigmented villonodular synovitis and synovial chondromatosis can also produce chronic effusions. June 11, 2012 Ratan M.P.T., (Ortho & Sports) 30

31. Diagnosis Diagnosis of JIA is difficult because joint pain in children can be from many other causes. There is no single test that can confirm the diagnosis and most physicians use a combination of  blood tests ,  x rays  and the clinical presentation to make an initial diagnosis of JIA. The blood tests measure  antibodies   & R h factor. Unfortunately , the R h factor is not present in all children with JIA. June 11, 2012 Ratan M.P.T., (Ortho & Sports) 31

32. Diagnosis cont… X rays are obtained to ensure that the joint pain is not from a fracture ,  cancer ,  infection  or a congenital abnormality. In most cases, joint fluid is aspirated & analysed. This test often helps in making a diagnosis of JIA by ruling out other causes of joint pain. June 11, 2012 Ratan M.P.T., (Ortho & Sports) 32

33. Diagnostic Criteria of JRA Onset before age 16 years; Arthritis involving one or more joints or presence of at least two of the following findings: Limitation in ROM Tenderness or pain with joint movement Increased fever Disease persisting 6 weeks or longer June 11, 2012 Ratan M.P.T., (Ortho & Sports) 33

34. Treatment Medical Rx Physiotherapy Surgical June 11, 2012 Ratan M.P.T., (Ortho & Sports) 34

35. Medical Treatment Goal: Control symptoms, Prevent joint damage and maintain function Medications: 1. Non-steroidal Anti-Inflammatory Drugs (NSAIDS) Motrin or Advil 2 . Disease Modifying Drugs (DMARDS) Hydroxychloroquine : Plaquenil Sulfasalazine: Azulfidine Methotrexate: Rheumatrex June 11, 2012 Ratan M.P.T., (Ortho & Sports) 35

36. Treatment : Physical Measures June 11, 2012 Ratan M.P.T., (Ortho & Sports) 36

37. Aims To reduce pain & stiffness To minimize swelling To maintain or increase ROM in affected joints To maintain or increase muscle strength in affected groups To prevent deformities To rehab the child to be independent and educate parents in the management of the condition June 11, 2012 Ratan M.P.T., (Ortho & Sports) 37

38. Heat application Acute conditions – Heat application to the inflamed joint is not recommended. Chronic conditions – Thermotherapy, especially paraffin baths combined with ex, should included as an intervention to improve ROM & decrease pain & stiffness. June 11, 2012 Ratan M.P.T., (Ortho & Sports) 38

39. Therapeutic ultrasound Therapeutic US is effective for reducing joint tenderness caused by JRA. Continuous US is more effective for patients with chronic JRA. Mechanical effect of pulsed & continuous US increases skin permeability, thus decreasing inflammatory response, reducing pain & facilitating soft tissue healing. Dosage for acute condition-Initial stage 0.25 to 0.5w/cm sq. Time-2-3 minutes June 11, 2012 Ratan M.P.T., (Ortho & Sports) 39

40. Therapeutic us cont… Failure case 0.25 to 0.5w/cm sq. Time-4-5 minutes Chronic condition Maximally up to 2w/cm sq. Time-8 minutes Ultrasonic 3MHZ-Superficial tissue Ultrasonic 1MHZ-Penetrate deeply June 11, 2012 Ratan M.P.T., (Ortho & Sports) 40

41. Interferential therapy It helps in minimizing pain in JRA The electrodes needs to place carefully Skin care taken in pts with high dose steroid Used of such modalities may addicted to the patient & when experiencing multiple joint pain it would be impractical. Dosage: 90 – 100 Hz – reduce nerve accommodation 50 – 100 Hz – improve healing, blood supply & membrane permeability June 11, 2012 Ratan M.P.T., (Ortho & Sports) 41

42. To Minimize Swelling Cryotherapy with compression Elevation of the limb Active ROM exs. June 11, 2012 Ratan M.P.T., (Ortho & Sports) 42

43. Manual therapy Mobilisation Should be avoided in pain and swelling Manipulation Myofascial release Trigger point therapy Acupuncture and Massage June 11, 2012 Ratan M.P.T., (Ortho & Sports) 43

44. Exercise Target: Neck Shoulder Elbow Wrist & hand Chest Hip Knee Lower leg June 11, 2012 Ratan M.P.T., (Ortho & Sports) 44

45. Positioning & Exercise Each jt. should moved actively through full range Strengthen the extensor muscels in prone & supine position June 11, 2012 Ratan M.P.T., (Ortho & Sports) 45

46. Shoulder- Girdle exercise with breathing exs . will keep the shoulder & costovertebral jt. mobile Elbow- Full flexion of this joint is important for maintenance of activities of daily living. AROM ex are recommended Holding arms at full extension (sitting & standing ) Use of night splints (especially when flexion contracture begins to develop ) Extensor muscle strengthening ex . June 11, 2012 Ratan M.P.T., (Ortho & Sports) 46

47. Wrist- Loss of extension and ulnar deviation at the radiocarpal joints are often the first limitations noted. A night resting splint is recommended in addition to the active extension exercises. Fingers- Terminal flexion and extension are limited AROM & PROM exs , preserving muscle power with squeezing a sponge and not allowing excess load on the loose joints, are recommended. June 11, 2012 Ratan M.P.T., (Ortho & Sports) 47

48. Hip- P rimarily extension & IR are limited. To prevent these pathologies, it is recommended that patients sleep in the prone position 2 times per day for 30-min durations Sleep in the prone position at night, and stretch and strengthen the extensor muscles. June 11, 2012 Ratan M.P.T., (Ortho & Sports) 48

49. Knee- Extension & flexion limitations are often observed. Night splints should be applied in case flexion contractures begin to develop. Recommended activities include Swimming , Ascending & descending stairs Kicking a ball. June 11, 2012 Ratan M.P.T., (Ortho & Sports) 49

50. Ankle- I n neutral position for heel strike & orderly walking pattern. Wearing appropriate shoes & slightly raising the heels relieve pain and provide a comfortable walking environment Foot- Small, wide feet with high arches, due to premature closure of tarsal and metatarsal joints. This may limit pronation & supination of the mid-foot. Plantar fascia can tighten & metatarsal adduction can be observed. June 11, 2012 Ratan M.P.T., (Ortho & Sports) 50

51. To retain flexibility, active & passive ROM exs Picking up marbles from the carpet to strengthen intrinsic foot muscles Using an arch support in the shoes are recommended. Shoes with thick soles and ankle supports are recommended for these patients. June 11, 2012 Ratan M.P.T., (Ortho & Sports) 51

52. Hydrotherapy All active exs. should be done in full range in Hydrotherapy pool Due to buoyancy providing weight relief, reeducation of walking can be given Passive stretching of tight structures is less painful in pool . Games and activites can encourage children to move stiffer jts.without their realizing it June 11, 2012 Ratan M.P.T., (Ortho & Sports) 52

53. Gait Training Walking is started in Hydrotherapy pool where pain relief and increas jt.mobility allows improvement in gait pattern Hip and Knee extension is encouraged during stance phase together with the push-off and heel strike at the beginning and end of the swing phase A walking aid may be necessary if child is limping Body weight supported treadmill is helpful in gait training June 11, 2012 Ratan M.P.T., (Ortho & Sports) 53

54. Surgery Rarely used in the early course of disease Indications: Relieve pain Release joint contractures Replace a damaged joint June 11, 2012 Ratan M.P.T., (Ortho & Sports) 54

55. Prognosis The prognosis depends on prompt recognition & Rx. With proper therapy, some children do improve with time and lead normal lives. However , severe cases which are not treated promptly can lead to poor growth & worsening of joint function . The greater the number of joints affected, the more severe the disease and the less likely that the symptoms will eventually go into total remission . June 11, 2012 Ratan M.P.T., (Ortho & Sports) 55

56. Prognosis cont… Finally, it is important for both the child and family member to be educated about the disorder. The more educated the person, the better the care you can receive. Chronic JIA is no longer the dreaded disease where one remains home bound. Many children with JIA have gone on to play professional  sports  and have a variety of successful careers. June 11, 2012 Ratan M.P.T., (Ortho & Sports) 56

57. Frequency In the U.S. 10-20 cases per 100,000 children Pauciarticular and polyarticular disease occur more frequently in girls Both sexes are affected with equal frequency in systemic-onset disease Internationally Occurs more frequently in certain populations (e.g., Native Americans) from areas like British Columbia and Norway June 11, 2012 Ratan M.P.T., (Ortho & Sports) 57

58. Mortality Less than 1% Often associated with the evolution of disease to manifestations of other rheumatic diseases June 11, 2012 Ratan M.P.T., (Ortho & Sports) 58

59. Morbidity Morbidity: Relates to adverse effects of medications, particularly NSAIDS Abdominal pain due to gastritis or ulcer disease, hepatotoxicity, renal toxicity Psychological Morbidity: Situational depression Problems functioning in school June 11, 2012 Ratan M.P.T., (Ortho & Sports) 59

60. Is There a Bright Side? There are numerous resources for parents: Websites Support systems for children Ongoing research to improve quality of life 5 -year-old Katie Tortorice leads an active, healthy life despite having JRA. June 11, 2012 Ratan M.P.T., (Ortho & Sports) 60