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Juvenile Idiopathic Arthritis

Juvenile Idiopathic Arthritis
International league against rheumatism(ILAR )Classification of Juvenile Idiopathic ArthritisOligo-arthritis :Persisting arthritis : of 1 – 4 joints in first 6 months of the disease Extending arthritis : restricted to 1 – 4 joints in first 6 months that subsequently develops into polyarthritis Young

Young

Classification of Juvenile Idiopathic Arthritis
2)Polyarthritis Rh –ve arthritis of >4 joints in first 6 months Rh + ve arthritis restricted to 1 -4 joints in first 6 months + positive serum Rh test 3 months apart3)Psoriatic arthritis Arthritis + psoriasis or arthritis + family history of psoriasis and either dactilylitis or nail pitting as onycholysis Young

Older-adolescent

Older girls and boys equally

Classification of Juvenile Idiopathic Arthritis

4)Enthesitis – related arthritis :Arthritis + enthesitis or Arthritis + 2 of :SI j. tenderness, inflammatory spinal pain , HLA B27 , anterior uveitis , family history of uveitis , spondarthritis or IBD5) systemic arthritis :Arthritis + fever >2 weeks , evanescent skin rash 6) Other arthritis Patients who fit no category or more than one category older boys

Under 2 yrs f= m


Oligoarthritis
Most common form targeting lower limb joint , mainly knee joint , prognosis is excellent , 30 % progress to severe polyarthritisANA an HLA – DR 5 are presented in 50 % and those children at high risk of asymptomatic chronic anterior uveitis

Polyarthritis

30 -40 % of JIP , 90 % seronegative for Rf , it is commonly affect the cervical spine , this lead to early cervical fusion and under – development of the mandible which give stiff neck , receding chin and dental problems . The prognosis is less favorable than oligoarthritis , 2/3 have residual problems into adult life and 10 % have severe joint damage

Polyarthritis

10 % Rh +ve , occur in older (>8) girl, follow an aggressive coarse with erosion , joint damage , nodules and vasculitis and showed high association with HLA DR4

Psoriatic arthritis

Presence of skin plaquesEnthesitis related arthritis Affect older children especially boys75 % are HLA – B27 positivethe prognosis is worse than for adult

Systemic arthritis

It is least common , equally affect male and female Mostly < 2 year Lymphadenopathy , hepatosplenomegally , pleuri- pericarditis and high fever Intermittent fever with evanescent faint pink macular rash are helpful for diagnosis

Prognosis

Remission within 6 months Half cases had recurrent episodes 30 % develop chronic polyarthritis and lead to secondary amyloidosis

Complication

Uveitis : is asymptomatic and potentially blinding , often bilateral , detected by slit lamp , greatest risk with early onset ( <6 years ) oligoarthritis in young girls who ANA +ve Growth disturbance accelerated or retarded epiphyseal growth ( long leg or short limbs and fingers ) or early fusion of epiphyses ( short limbs , micrognathia ) Loss of schooling and family disruption


Management
EducationRegular exercisePhysical therapy , to reduce deformities and maintain muscle strength , avoid development of flexion deformities especially hip and kneesDrug therapyNSAIDs : as naproxin , ibuprofen , tolmetin , and piroxicam avoid aspirin because of risk of Rey’s syndrome

Management

Intra articular steroid injection ( long life than adult ) DMARDs : as MTX , if fail to respond to NSAIDs , HCQ , Cyclosporine A may be considered if MTX alone is insufficient , sulfasalazine Gold , penicillamine HCQ have no significant benefit

Management

Long – term corticosteroids are used Severe systemic disease Chronic uveitis not responding to local therapy Active joint diseaseChlorambucil is used in children with secondary amyloidosis

Management

Surgery Soft tissue release Osteotomies Total hip arthroplasty




رفعت المحاضرة من قبل: Mostafa Altae
المشاهدات: لقد قام 15 عضواً و 158 زائراً بقراءة هذه المحاضرة








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