Juvenile idiopathic arthritis

1. Juvenile idiopathic arthritis
Presented By:
1. Dr. Yeakub Sarker
2. Dr. Tanvir
3. Dr. Progga

2. Introduction
the most common chronic rheumatic illness in children
Synonyms are
a) juvenile rheumatoid arthritis
b) juvenile chronic arthritis

3. Etiology
 Not completely understood. 2 factors may be responsible.
1. Immunogenetic susceptibility: polymorphism in the gene encoding tumor necrosis factor TNF-
alpha, macrophage inhibitory factor(MIF), IL-6, IL-1alpha.
2. External trigger: includes
a) bacterial and viral infections like
1. Parvovirus B-19
2. Rubella
3. Epstein-Barr virus

4. b) Enhanced immune response to bacterial or mycobacterial
heat shock proteins
c) Abnormal reproductive hormone level
d) Joint trauma

5. Pathophysiology
 It is characterized by
 Synovitis of peripheral joints manifesting as soft tissue swelling
and effusion. Initially the synovial membrane is infiltrated by
many inflammatory cells eg lymphocytes, plasma cells,
macrophages and the membrane become swollen and
congested( Synovisits)

6. Subsequently the inflammatory process expands and give rise
to villous hypertrophy and hyperplasia of synovium and
secretion of synovial fluid in the joints (Effusion)
Inflammatory granulation tissue develops and that spreads
under the articular cartilage and the adjacent bones.
Finally there is fibrosis and ankylosis of the affected joints,
limitation of movement, and atrophy of periarticular muscles.

7. Diagnostic criteria of JIA
All three of the following must be met :
1. Arthritis persisting for 6 weeks or more
2. Onset of arthritis before 16 yrs of age
3. Exclusion of other causes of arthritis

8. Arthritis
Arthritis is defined as
Swelling or effusion or 2 or more of the following :
Limitations of range of motion
Tenderness or pain on motion
Increased temperature

9. Classification of JIA
Based on the number of joint involvement during the first 6
months of the disease and the presence of extra articular
manifestations 7 categories of JIA are seen among children
1. Oligoarthritis(40-50%)
Arthritis affects 1-4 joints during the 1st 6 months of disease. Two
sub categories are recognised:

10. Persistent oligoarthritis: affecting 4 or less joints throughout the
disease course
Extended oligoarthritis: affecting more than 4 joints after first 6
months of the disease course

11. 2 Polyarthritis: it is of 2 subtypes:
Rheumatoid factor negative: in 20-35%.
Affects 5 or more joints in the first 6 months of the disease.may
be symmetrical or asymmetrical. Affects small and large
joints,cervical spine, temporomandibular joint.

12. Rheumatoid factor positive : in less than 10%
Affects 5 or more joints during the first 6 months of the disease.
Aggressive symmetrical polyarthritis

13. 3. Systemic arthritis (5-15%)
Along with joint manifestation it hase prominent extra articular
systemic manifestation
Like : Non remittent fever
Rash
Lymphadenopathy
Serositis
Hepatosplenomegaly

14. 4. Enthesitis related arthritis (5-10%)
Inflammation at one or more of the entheses ( site of insertion of
tendons, ligaments or fascia fascia). The common sites of
inflammation are at insertion of planter fascia and at the insertion
of tendo Achilles into calcaneum

15. 5. Psoriatic arthritis: (5-10%)
Arthritis and psoriasis or arthritis and at least 2 of the following
Dactylitis, nail pitting and onycholysis
H/O psoriasis in a first degree relative
6. Undifferentiated: it covers the overall definition of JIA but don’t
fulfill the specific category

16. Clinical manifestations
Although variable , the usual presentations are;
Persistent pain and swelling of joints both small and large
Limping or refusal to walk or trying not to use the affected
joints( Guardians of joints)

17. Involvement of PIP joints of hands gives rise to characteristic
spnidle shaped appearance
Sometimes dysfunction noted in upper limbs, neck(torticollis)
Joint stiffness following sleeping, rest or decresed acfivity
(as morning stiffness)

18. Presence of rheumatoid nodule on the extensor surface of
elbow and over achilles tendons
Non specific symptoms such as lathergy, high fever, poor
appetite, irritability, sleep disturbances
Presence of evanescent rash
Occasionally, features of extra articular manifestations eg:
pericarditis, serositis, organomegaly, uveitis may be present

23. Characteristics of arthritis in acute rheumatic
fever and rheumatoid arthritis
Parameters Acute rheumatic fever Rheumatoid arthritis
Types of joints involved
Large joints. Involvement
spines, small joints of
hands and hip joints are
uncommon
Both large and small joints
Symmetry of involvement Asymmetrical Usually symmetrical
Onset of arthritis
Acute. Lasts for shorter
period
Insidious onset. Arthritis
remains for a longer
duration
Characteristics of arthritis
Migratory polyarthritis with
exquisite pain and
tenderness. Mono arthritis
Arthritis and joint
symptoms are more
marked early in the
morning( morning

24. Parameters Acute rheumatic fever Rheumatoid arthritis
Response to aspirin Dramatic Not such
Chance of deformity
Typically no deforming
disease
High
Preceding history
Sore throat 2-4 weeks prior
to the onset of joint pain
Absent
Involvement of other
organs
Heart(Caeditis/valvular
lesions), basal
ganglia(Chorea)
Uveitis, lymphadenopathy,
hepatosplenomegaly

25. Diagnosis
Investigarions Results
Complete blood counts Hb% Low, TC&DC ( Neutrophilic
leucocytosis)
Platelets(Thrombocytosis)
PBF Non specific
Acute phase reactants( ESR, CRP, Ferritin) Raised
Anti nuclear antibody Positive in 40-50% cases
Rheumatoid factor Positive in only 5% of patients
Anti cyclic citrullinated peptide (Anti
CCP)antibody
It is a highly specific serological marker
early diagnosis of rheumatoid arthritis
Urinalysis May reveal proteinuria(50-60%) and/or

26.  Radiology and imaging:
Investigations Results
Xray of affected joints Soft tissue swelling, Periarticular
osteoporosis, periostitis, subchondral bony
erosion, loss of cartilage, narrowing of
joint space
Ultrasonography of affected joints Joint effusion
MRI of affected joints More sensitive than X Ray to detect earlt
changes

27. Stages of radiological progession in JIA
Stages Radiological changes
I Only soft tissue swelling
II Periarticular osteoporosis,
Increased joint space(Effusion)
III Loss of articular cartilage
(Decreased joint space)
IV Erosion
V Subluxation and ankylosis

28. Synovial fluid analysis and synovial biopsy
Arthroscopy and arthrography
Others
Slit lamp examination of the eyes to detect uveitis
Urine R/M/E to exclude SLE

29. Complications of JIA
Joint contracture
Anaemia
Pericarditis
Myocarditis
Glomerulonephritis
Chronic ant. Uveitis
Growth disturbance
amyloidosis

30. Aims of treatment of JIA
Relieve the joint symptoms
Suppression of acrive
disease process & Prevent
the lrogression of the
disease
Conservation of joint
function
Look for any systemic
complication and take
measures if present

31. Principle of treatment
Start with
Monotherapy(Aspirin) :
Continue for 2-3 weeks.
If not improved add another
NSAID: Ibuprofen/Naproxen.
Continue for another 3
weeks
If still no improvement:
Switch to DMARDs
If there is development of
systemic symptoms in spite
of development of joint
symptoms DMARDs can be
initiated.

32. Treatment of JIA
Multidisciplinary approach needed involving paediatrician,
rheumatologist, physiotherapist, ophthalmologist
Treatment option and duration varies according to the
subtypes and from patient to patient

33. Drugs
 First line: Analgesic, NSAIDs
 Second line: DMARDs
 Thirdline: Steroids
 NSAIDs: treatment should be started with NSAIDs and unless adversity noted. Duration should be 4-6 weeks to
allow sufficient time to assess clinical response.

34. NSAIDs Dose
Naproxen 15mg/kg/day PO bid, max.
1gm/day
Or, Ibuprofen 40mg/kg/day PO tid, max
4gm/day
Or, Meloxicam 0.125mg/kg/day PO once daily,
max 15 mg/day
Commonly used NSAIDs are:

35. Sometimes along with NSAIDs other treatment options are
added depending upon response to drugs, types of arthritis,
associated co-morbidities and also stages of disease.
Disease modifying anti rheumatic drugs(DMARDs): these drugs
retard progression of the disease. They are considered for any
form of arthritist hat is not controlled by NSAIDs.

36. Commonly used DMARDs
Name dose
Methotraxate 10 mg/m2/week PO or SC
sulfasalazine
leflunamide

37.  Corticosteroids : Used as an adjunct or bridging therapy
Systemic Intra articular
Prednisolone Triamcinolone
Methyl prednisolone hexacetonide

38. Role of Steroids in JIA
Local
Intra articular: Relieve the
joint symptoms and pain
In case of acute or chronic
uveitis: reduce inflammation
Systemic
To relieve the systemic
manifestation

39. Biological agent:
 Tocilizumab
 Etanercept
 Infliximab
 Adalimumab
 Abatacept
 Rituximab
 anakinra

40. Physiotherapy
It preserves range of motion of the joints and muscular
strength amd protects joint integrity.
All rotatory joint movements are advocated to prevent disuse
atrophy
Firstly passive physiotherapy to be continued untill pain
subside. Then active physiotherapy.

42. Advice
Patient should be consulted not to gain weight.
Dietary modification is to be promoted like avoiding junk food
and high calorie food
Patient is adviced to go for swimming as there is movements
of many joints occur simultaneously

43. Follow up
 To see improvement in activities of daily life and early detection of any
complications like joint contractures, muscle wasting etc.
 Periodic slit lamp ophthalmologic examinations to monitor asymptomatic
uveitis
 To see prognosis of the disease:
CBC
PBF
ESR, CRP
Radiological evaluation
Functional assessment

44. Thank you