[My Articles & Information]    [Medical Links]    [E-Mail Me]    [Index] Juvenile Rheumatoid Arthritis and the Seronegative Spondyloarthropahies Definition, History, Types, Diagnosis, Operative Intervention Definition (American Rheumatism Association) JRA is a group of diseases in which there is; chronic synovial inflamation of unknown cause onset in children of under 16 years of age objective evidence of arthritis in one or more joints for 6 consecutive weeks Arthritis is defined as a swelling, limitation of motion of ajoint with some combination of heat, pain and tenderness Arthralgia is purely joint pain The seronegative spondyloarthropathies are characterized by axial skeleton arthritis, peripheral arthritis that is often assymetric in distribution, eye or mucocutaneous involvement, urethritis or dyssentry, enthesitis, absence of rheumatoid factor and antinuclear antibodies in the blood, tendancy to be familial and high asociation with HLA B-27 Back to the top History First described by George F. Still in 1897. He described several kinds of arthritis characterized by severe systemic symptoms associated with multiple joint involvement JUVENILE RHEUMATOID ARTHRITIS most common childhood rheumatic disorder Affects 200,000 children in US Classified into 3 recognizable subgroups pauciarticular onset JRA -has four or less joints involved polyarticular JRA -has five or more joints involved Back to the top Types of JRA & their onset 1. Systemic onsent illness always begins with spiking fevers (> 39.3C) 2. Pauciarticular Onset most frequently encounteed subgroup 40 - 60% of JRA peak onset 2 years girls/ boys ratio 4/1 knee, ankle and elbow are the most frequently involved joints. hip and cervical spine involvemenr are rare ESR and WBG are normal; RF rarely positive 50% have a positive ANA if chronic iridocyclitis 64% to 88% positive ANA untreated chronic inflammation of the anterior chamber of the eye leads to the formation of adhesions between the iris and the lens (posterior synechiae) causing pupillary deformation excellent prognosis; 70% no functional impairment after 15 years 15% children go on to have severe destructive joint disease subluxation of the knee can occur in children with large flexion contractures and aggressive disease iritis may be more devestating than the arthritis 3. Polyarticular Onset accounts for 30% to 40% of children with JRA maily girls (3:1) 2 age peaks; l-3years -rheumatoid factor negative early adolescents -rheumatoid factor positive -behaves like adult RA; also get nodules, joint erosins, Feltys syndrome (RA, splenomegaly and leukopenia) by definition patients have at least 5 joints involved both large and small joints with symmetric involvement is common pattern symmetric involvement of knees, wrists and ankles is the most common pattern Prognosis is good; 60% 15 year remission 25% to 50% of adolescent subgroup go on to severe destructive arthritis 4. Systemic Onset least common form affecting 20% children with JRA equal number of girls and boys onset 5 - 10 years fever is key factor daily or BD fever to > 39.3 associated with intense arthralgias and myalgias rash: pink - salmon, non-pruritic, maculopapular, small discrete spots with central clearing pericarditis, myocarditis lymphadenopathy hepatosplenomegaly abdominal pains growth retardation vasculitis iritis ESR usually high > 100mm/hr variable prognosis with 25% - 50% having severe destructive arthritis 5. Seronegative Spondyloarthropathies seronegativity is defined as the absence of ANA or RF in the blood Patients Frequently HLA B-27 Pos. Encompases ankylosing spgndylitis, reiters, reactive arthritis and the spondylorathropathies associated with psoriasis and inflammatory bowel disease boys/girls: 4/1 other systemic manifestations; fevers, malaise, weight loss and aortitis 6. Ankylosing Spondylitis painful and progressive arthritis of the SI joints and spine which mainly affects 20 - 40 year old caucasian males may present in boys over 8 with assymetric pattern of peripheral arthritis radioiogical changes in the axial skeleton are a late finding morning stiffness; symptoms worse in early morning and the middle of the night limited back motion; Ioss of normal lumbar lordosis HLA B-27 present in 4% normal white males; present in 90% of ankylosing spondylitis 7. Other Seronegative Spondyloarthritidies Reiter's Syndrome arthritis, conjunctivitis and urethritis: 3 cardinal manifestations of Reiters syndrome strong genetic basis triggered by venereal disease or dyssentry (salmonella, shigella, yersinia or campylobacter) 8. Arthritis with Innsmmatory Dowel Disease 7% to 21% children with IBD develop transient penpheral arthritis or spondylitis 9. Psoriatic Arthritis occurs in 7% of patients with psoriasis usually DIP jt involvement Enthesopathy (pain at a ligament or tendon insertion) seen frequently in children with seronegative arthritis 10. Aetiology largely unknown genetic infleunce familial clustering HLA B-27 assocn. many theories of infection rubella, varicella, EBV all cultured bunelia burgdorferi causes Lyme disease (named after Lyme in USA) 11. Pathology underlying pathology that of inflammation synovial hypertrophy -synovium hyperaemic and oedematous -plasma cell and lymphocyte infiltration -secretion of fluid into joint with leukocytes 5000 to 50,000 -villi hypertrophy grow in from cartilage margins pannus formation with cartilage erosions, subchondral collapse fibrous anklyosis arthrodesis inflammatory hyperaemia may cause overgrowth of adjacent growth plates 12. Radiographic changes in the cervical spine (7); anterior erosion of the odontoid ant-post erosion of the odontoid (apple-core peg) subluxation of C1 on C2 focal soft tissue calcification anterior to the arch of C1 ankylosis of the apophyseal joints longtitudinal and circumferential growth abnormalities subaxial subluxations, usually ankylosed above and below 13. In Anklyosing Spondylitis clinically detectable anklyosis ascending from SI jt upwards vertebrae appear squared off on the lateral longtitudinal ligaments and annulus calcified: bamboo spine. Back to the top Differential diagnosis of childhood arthritis and arthralgia Leukemia child systemically ill: affects young, usually monoarticular presentation bone pain is common Sarcoid non-tender boggy synovium with no limitation of ROM mav have fevers, rash and iritis pulmonary disease, erythema nodosum, abdominal pain, liver enlargement, perihilar adenopathy Acute Rheumatic Fever autoimmune complication 2 - 4 weeks after a streptococcal infection mainly children 5 -15 years of age use MODIFIED JONES CRITERIA FOR DIAGNOSIS OF RHEUMATIC FEVER - joints are exquisitely red, hot and tender - arthritis migrates from joint to joint over a period of hours - rapid response to aspirin almost diagnostic Pigmented Villonodular Synovitis slow growing B9, locally invasive tumor of the synovium usually monoarticular and most frequently involves the knee most have hemorrhagic ,dark brown synoviai fluid biopsy is diagnostic Lyme Disease tick borne spirocheatal infection (Burellia Burgdorferi) rash, constitutional Sx, neurologic symptoms, carditis, acute or chronic arthritis Henoch-Schonlein Purpura small vessel vasculitis involving skin, joints, gut and kinneys boys more than girls rash from waist down course lasts 6 weeks; relapses are common SLE mainly affects teenage girls marked constitutional symptoms butterfly rash, major organ disease arthritis responds quickly to steroids Foriegn Body Synovitis Investigations Arthrocentesis inflammatory fluid with WCC 2000 - 50 000 range, polymorphs predominate Synovial biopsy rule out PVNS goals of medical management pain relief prevention of deformity maintenance of function requires team approach with; drugtherapy intraarticular injections occupational therapy and splinting selective orthopaedic surgery Medical therapy; first line NSAIDS - Aspirin well tolerated by children; GIT upset or tinnitus common SE second line drugs SARDS (slowly accumulating remittive drugs) - used when first line therapy not controlling pain - include salicylazoslfapyridine, hydorxychloroquinone oral gold and D-penicillamine - relatively toxic - may require several months to evaluate third line therapy cytotoxic agents methothrexate Intrarticular Steroids useful in child with a limited number of joints affected which have failed medical treatment can give up to 1mg/kg dose no more than 3 injections per joint per year or 5-6 total injections Physical and occupational therapy aim to improve and maintain strength and range of motion while protecting joints night splinting of wrist, hand, knee. ankle may help prevent flexion contractures shoe inserts for enlistheses and shoe raises for LLD (to stop long, leg getting FFD) Back to the top Operative Intervention 70% children enter remission without significant permanent joint damage aims: prevent deformity, loss of movement and pain special considerations; hyperaemia and dissuse and intraarticular steroids lead to osteoporosis smaller weaker bones (standard components not fit; need custom prostheses) consider activity of disease at time of surgery; perform better if in remission success of procedure and painful rehabilitation requires maturity in the patient -many say 6 is the lower limit of surgery. 1. Synovectomy role ofsynovectomy is controversial rationale to remove the diseased synovium and slow or stop the destructive process recent studies show pathological tissue regenerates (although in lesser quantities) most orthopods report good short term results in JRA results are better in large than small joints recurrence is reported in all series no decrease injoint-space narrowing or osteoporosis criteria for synovectomy. - good preoperative range of movement - minimal radiographic destruction - little more than proliferative synovium seen at surgery Arthritis Foundation Comittee on Evaluation of Synovectomy sponsored by the Americal Rheumatism Association reported 5 year results; -little long-term value in the prevention of recurrence of disease activity, the prevention or progression of articular damage recommend adequate trial of medical therapy first -6 months theraputic NSAIDS, or SARDs with splinting and PT -age over 6 to cope with rehabilitation no definite evidence complete synovectomy is better than partial done under torniquet control post operative early ROM arthroscopic synovectomy decreased morbidity over open procedure 2. Soft Tissue Release decreased arc of motion places unphysiological stresses on joint increased energy of locomotion goals of soft tissue release to put joint into a more functional position increase arc of motion (inc. function and articular cartilage nourishment) prerequisites - in most joints (except the hip) need radiographic evidence of good joint space - studyes show widening of articular cartilage - joint better aligned for later surgical procedures i.e. TJA require aggressive post-operative PT and splinting release of hip and knee most successful 3. Osteotomy painless severe contracture in a non-functional position with mod to severe evidence of joint incongruity periarticular extension osteotomv of knee of benefit intertrochanteric hip osteotomy not as effective; unable to immobilize these patients in a spica 4. Arthrodesis for isolated severe joint destruction with deformity and pain from other cause; infection, trauma not appropriate for hip/knee in JRA as multiple joint involvement 5. Total Joint Arthroplasty recommended if severe hip and knee destruction all early Rx aimed at delaying the time of insertion of implant main problems of loosening and infection delay of TJA recommended not until skeletal maturity 50% cases require custom made prosthetic components due to size do hips before knees in JRA due to possible to rehab hip in presence of FFD knee Joint specific Orthopaedic Treatment HIP flexion contracture followed by internal rotation and adduction contracture if onset after 10 years tend to progress to fibrous ankylosis with protrusio soft tissue release indicated in severe FFD of hip regardless of state of articular cartilage limited open adductor and psoas tenotomy (through Ludloff- approach) intertrochanteric osteotomy contraindicated as tends to increase stiffness arthrodesis not consideration as patients have multiple joints involved TJA; best after skeletal maturity KNEE most frequently involved joint in children with JRA - deformities; flexion contracture, restricted ROM valgus deformity posterior subluxation of the tibia easily accessible; intraarticular steroids physical therapy; positioning, splinting should be used early to prevent FFDs > 15' 15' - 25' serial casting of benefit synovectomy of limited use; - does not increase range of motion soft tissue release - indicated for FFD > 25' - prerequisite is for good joint space - requires extensive release (including PCL if posterior subluxation of tibia) - Supracondylar osteotomy if bony deformity of joint incongruity or significant subluxation also ideal correction for genu valgum FOOT AND ANKLE problems usually amenable to footwear, braces, orthoses hindfoot - ankle and subtalar joints involved - talipes equinus forefoot - claw toes - hallux valgus - metatarsalgia CERVICAL SPINE approx 60% involvement in-JRA neurologic involvement less common in JRA than adult RA as basilar invagination is rare in JRA all children should have flexion/extension Xrays posterior fIusion CI-C2 is indicated for instability with progressive neurological signs HAND AND WRIST OT and splinting make the greatest difference surgical procedures are rarely indicated tendon ruptures uncommon in JRA wrist may be amenable to splinting in a functional position; arthrodesis other option Back to the top