Juvenile ra


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Juvenile ra

  1. 1. 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 22, 2012 Ratan M.P.T., (Ortho & Sports) 2
  2. 2. 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 22, 2012 Ratan M.P.T., (Ortho & Sports) 3
  3. 3. 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 June 22, 2012 Ratan M.P.T., (Ortho & Sports) 4 number of joints are involved) in 80% of cases.
  4. 4. 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.June 22, 2012 Ratan M.P.T., (Ortho & Sports) 5 The male to female ratio is 1:2.5.
  5. 5. 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 22, 2012 Ratan M.P.T., (Ortho & Sports) 6
  6. 6. etiology cont… Genetic & Climate components: – There 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 immuneJune 22, 2012 Ratan M.P.T., (Ortho & Sports) 7 response has been observed.
  7. 7. 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 22, 2012 Ratan M.P.T., (Ortho & Sports) 8
  8. 8. PathologyJRA is generalized disorder of connective tissue affecting – – Articular structure & – Extra articular structuresJune 22, 2012 Ratan M.P.T., (Ortho & Sports) 9
  9. 9. Articular ChangesStage I: – Inflammation of the synovial membrence spreads to articular cartilage & other soft tissues. – Limitation of joint movt with pain & muscle spasmJune 22, 2012 Ratan M.P.T., (Ortho & Sports) 10
  10. 10. 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 22, 2012 Ratan M.P.T., (Ortho & Sports) 11
  11. 11. 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 22, 2012 Ratan M.P.T., (Ortho & Sports) 12
  12. 12. articular changes cont…Stage IV: Permanent joint damage and deformity DisabilityJune 22, 2012 Ratan M.P.T., (Ortho & Sports) 13
  13. 13. June 22, 2012 Ratan M.P.T., (Ortho & Sports) 14
  14. 14. 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 becomeJune 22, 2012 Ratan M.P.T., (Ortho & Sports) 15 obliteration.
  15. 15. Oligoarticular JRA JRA Polyarticular JRACLASSIFICATION Systemic JRAJune 22, 2012 Ratan M.P.T., (Ortho & Sports) 16
  16. 16. Oligoarticular (orpauciarticular) 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. Oligoarticular 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 22, 2012 Ratan M.P.T., (Ortho & Sports) 17
  17. 17.  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 22, 2012 Ratan M.P.T., (Ortho & Sports) 18
  18. 18.  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 22, 2012 Ratan M.P.T., (Ortho & Sports) 19
  19. 19. 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 22, 2012 Ratan M.P.T., (Ortho & Sports) 20
  20. 20.  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 22, 2012 Ratan M.P.T., (Ortho & Sports) 21
  21. 21. 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 spontaneousJune 22, 2012 Ratan M.P.T., (Ortho & Sports) 22 rapid return to baseline (vs. Septic Arthritis of
  22. 22.  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 22, 2012 Ratan M.P.T., (Ortho & Sports) 23
  23. 23.  Systemic JIA may have internal organ involvement: – Hepatosplenomegaly, Lymphadenopathy, Hepatitis, T enosynovitis, etc. A polymorphism in macrophage migration inhibitory factor has been associated with this condition. It is sometimes called "adolescent-onset Stills disease", to distinguish it from adult-onset Stills disease. However, thereRatan some evidence that the two 24June 22, 2012 is M.P.T., (Ortho & Sports)
  24. 24. 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 – – OftenJune 22, 2012 in girls, in conjunction with psoriasis, although Ratan M.P.T., (Ortho & Sports) 25
  25. 25. Summary of Symptoms Joint swelling Pain Stiffness Limping Limited movement Slow movement Fever Skin rashJune 22, 2012 Ratan M.P.T., (Ortho & Sports) 26
  26. 26. 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 & Sports)June 22, 2012 Ratan M.P.T., (Ortho 27
  27. 27. 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 22, 2012 Ratan M.P.T., (Ortho & Sports) 28
  28. 28. 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 22, 2012 Ratan M.P.T., (Ortho & Sports) 29
  29. 29. 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 22, 2012 Ratan M.P.T., (Ortho & Sports) 30
  30. 30. 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 & Rh factor. Unfortunately, the Rh factor is not present in all children with JIA.June 22, 2012 Ratan M.P.T., (Ortho & Sports) 31
  31. 31. 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 22, 2012 Ratan M.P.T., (Ortho & Sports) 32
  32. 32. 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 longerJune 22, 2012 Ratan M.P.T., (Ortho & Sports) 33
  33. 33. TreatmentMedical RxPhysiotherapySurgicalJune 22, 2012 Ratan M.P.T., (Ortho & Sports) 34
  34. 34. 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: RheumatrexJune 22, 2012 Ratan M.P.T., (Ortho & Sports) 35
  35. 35. Treatment:Physical MeasuresJune 22, 2012 Ratan M.P.T., (Ortho & Sports) 36
  36. 36. 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 conditionJune 22, 2012 Ratan M.P.T., (Ortho & Sports) 37
  37. 37. 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 22, 2012 Ratan M.P.T., (Ortho & Sports) 38
  38. 38. 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. •June 22, 2012 Time-2-3 minutes Ratan M.P.T., (Ortho & Sports) 39
  39. 39. 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 deeplyJune 22, 2012 Ratan M.P.T., (Ortho & Sports) 40
  40. 40. 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 permeabilityJune 22, 2012 Ratan M.P.T., (Ortho & Sports) 41
  41. 41. To Minimize SwellingCryotherapy with compressionElevation of the limbActive ROM exs.June 22, 2012 Ratan M.P.T., (Ortho & Sports) 42
  42. 42. Manual therapyMobilisation – Should be avoided in pain and swellingManipulationMyofascial releaseTrigger point therapyAcupuncture andMassageJune 22, 2012 Ratan M.P.T., (Ortho & Sports) 43
  43. 43. Exercise Target: – Neck – Shoulder – Elbow – Wrist & hand – Chest – Hip – Knee – Lower legJune 22, 2012 Ratan M.P.T., (Ortho & Sports) 44
  44. 44. Positioning & Exercise Each jt. should moved actively through full range Strengthen the extensor muscels in prone & supine positionJune 22, 2012 Ratan M.P.T., (Ortho & Sports) 45
  45. 45.  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 22, 2012 Ratan M.P.T., (Ortho & Sports) 46
  46. 46.  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 looseM.P.T., (Orthoare recommended.June 22, 2012 Ratan joints, & Sports) 47
  47. 47. Hip- – Primarily 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 22, 2012 Ratan M.P.T., (Ortho & Sports) 48
  48. 48. 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 22, 2012 Ratan M.P.T., (Ortho & Sports) 49
  49. 49.  Ankle- – In 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. Ratan M.P.T., (Ortho & Sports) June 22, 2012 50
  50. 50. – 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 22, 2012 Ratan M.P.T., (Ortho & Sports) 51
  51. 51. 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 itJune 22, 2012 Ratan M.P.T., (Ortho & Sports) 52
  52. 52. 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 in53June 22, 2012 Ratan M.P.T., (Ortho & Sports)
  53. 53. SurgeryRarely used in the early course of diseaseIndications: – Relieve pain – Release joint contractures – Replace a damaged jointJune 22, 2012 Ratan M.P.T., (Ortho & Sports) 54
  54. 54. 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 22, 2012 Ratan M.P.T., (Ortho & Sports) 55
  55. 55. 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 22, 2012 Ratan M.P.T., (Ortho & Sports) 56
  56. 56. 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 NorwayJune 22, 2012 Ratan M.P.T., (Ortho & Sports) 57
  57. 57. Mortality Less than 1% Often associated with the evolution of disease to manifestations of other rheumatic diseasesJune 22, 2012 Ratan M.P.T., (Ortho & Sports) 58
  58. 58. 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 schoolJune 22, 2012 Ratan M.P.T., (Ortho & Sports) 59
  59. 59. 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 havingJune 22, 2012 Ratan M.P.T., (Ortho & Sports) JRA. 60