jia ,arthriitis

1. ARTHRITIS
JUVENILE IDIOPATHIC
ARTHRITIS
Dr. Rajesh kumar

2. Arthralgia :Pain in the joint, No inflammation
Arthritis:
Intra articular swelling
Or
Presence of swelling of joint
Or
Presence of any two of the following
Joint warmth
Tenderness
Redness
Limitation of range of movement

3. Articular vs
Non articular

4. Inflammatory VS Non inflammatory

5. Noninflammatory Non articular Pain
Growing pains:
• A benign condition,
• it is classically seen at 3–12 years of age,
• peaking between 3 and 5 years of age.
• pain is usually bilateral and localized to shin, calves, thighs and
popliteal
• Decreased pain threshold, vascular changes and stress
• Reassurance and symptomatic treatment with oil massage and
paracetamol

6. Pain amplication syndromes :
• It is more common in preteens and teenagers.
• is usually a significant stressor in the social milieu of the patient.
treatment would involve acknowledging to the patient and the care
givers the fact that the patient denitely has pain and would involve
physiotherapy and behavioral therapy.
• disorder can be chronic and only in resistant cases, are drugs like
antidepressants used.
• It is classically a disease in which there are many symptoms and not
too many signs on examination.
• patients come with pain symptoms in anatomically distinct regions
with some gastrointestinal and genitourinary symptoms

7. Periarticular Inflammation Orthopedic conditions Fractures :
in periarticular region might rarely simulate arthritis.
Osteomyelitis in the metaphyseal region might give rise to symptoms
which can mimic arthritis and sometimes an extension into the joint
can cause septic arthritis. Ere is an entity known as chronic recurrent
multifocal osteomyelitis (CRMO) which is auto inammatory disease of
the bone and is seen in the metaphysis of the bone .

8. ARTICULAR INFLAMMATION/ARTHRITIS :
• acute if the duration of the disease is less than 3 weeks
• subacute if it is between 3 and 6 weeks
• chronic if it is more than 6 weeks.
Acute Arthritis :
• Septic arthritis
• Reactive arthritis

9. Septic arthritis
• A 3-year-old boy was brought with history of fever associated with
right knee joint swelling and pain for the past 3 days.
• knee was so tender that the child refused anybody permission to
even come near it. CBC revealed mild anemia and leukocytosis.
• ESR was elevated. synovial fluid analysis revealed cell count of
1,00,000/mm3 with neutrophilic predominance.
• Gram stain of synovial fluid showed Gram positive cocci. Is is the
typical case of septic arthritis Treatment is antibiotics for at least 4–6
weeks.

10. • Reactive arthritis A 10-year-old boy was brought with history of bacillary
diarrhea 3 weeks back and had been treated with oral antibiotics.
• He had recovered completely from the bout when he started having painful
swelling of right knee with limitation of movement since past one week.
• Fever was not very high and CBC showed mild leukocytosis with mildly
elevated ESR. Is is a typical case of reactive arthritis.
• It is found to be more common in individuals who are HLA-B27 positive.
• Treatment is usually with NSAIDs. When reactive arthritis becomes chronic,
differentiation from JIA might be difficult.

11. Chronic Arthritis:
• Infection A chronic infection like tuberculosis of joint can sometimes
mimic JIA.
• clue would be that it tends to be a monoarthritis in a child with a
possible TB contact with strongly positive Mantoux test.
• Synovial fluid analysis will show increased leukocyte count with
lymphocytic predominance.

12. • children less than 16 years with arthritis persisting beyond 6 weeks.
• Enthesitis related arthritis It is a form of arthritis which tends to aect older
children and adolescents with predominant lower limb arthritis and a
tendency to aect the axial skeleton and
• Ere is absence of autoimmune antibodies like ANA and rheumatoid factor
and presence of HLA-B27 in majority of these patients.
• It is called as Juvenile ankylosing spondylitis if there is involvement of
sacroiliac joint as confirmed by imaging (X-ray/MRI).
• Psoriatic arthritis e presence of arthritis of at least 6 weeks in a child less
than 16 years of age with psoriasis or if psoriasis is absent, presence of at
least two of the following three criteria: dactylitis; nail pitting; and
psoriasis in a first degree relative with absence of following: HLA-B27,
rheumatoid factor, features of systemic onset JIA and family history of HLA-
B27 associated diseases
• Chronic arthritis can also be associated with disorders including SLE,
juvenile dermatomyositis, vasculitis, and periodic fever syndromes.

13. JUVENILE IDIOPATHIC ARTHRITIS

14. • JIA is the most common rheumatic disease in children and one of the
more common chronic illnesses of childhood.
• The etiology and pathogenesis of JIA are largely unknown, and the
genetic component is complex, making clear distinction among
various subtypes difficult.
• It was previously called Juvenile Rheumatoid Arthritis by the
American College of Rheumatology (ACR).
• Juvenile Chronic Arthritis, is a term coined by (ELAR) European League
Against Rheumatism.

15. Definition:
(ILAR) defines JIA as
1.Arthritis in > 1 joint which is defined as
1.Swelling or
2.Effusion or
3.Presence of 2 or more of the following signs
1.Limitation of range of motion
2.Pain or tenderness in motion
3.Increased local temperature
2.Age of onset, before 16 years
3.Duration - Present for at least 6 weeks
4.Exclusion of other forms of arthritis in children

16. Types of JIA
Systemic-onset JIA
Oligoarticular JlA
Polyarthritis (Rheumatoid Factor-negative)
Polyarthritis (Rheumatoid Factor-positive)
Psoriatic arthritis
Enthesitis-related arthritis
Undifferentiated arthritis

17. Table 1 - Classification criteria for various types of JIA
Category Definition Exclusion
Systemic onset
Arthritis in 1 joint with, or preceded by a fever of at least 2 wk in duration that is
documented to be daily (quotidian) for at least 3 days with 1 of the following.
1. Evanescent (nonfixed) erythematous rash.
2. Generalized lymph node enlargement.
3. Hepatomegaly or splenomegaly or both.
4. Serositis.
a) Psoriasis or a history of psoriasis in the patient or a 1st-degree relative.
b) Arthritis in an HLA-B27 - positive boy beginning after the 6
birthday.
c) Ankylosing spondylitis, enthesitis-related arthritis, sacroiliitis with inflammatory
bowel disease, Reiter
syndrome, or acute anterior uveitis, or a history of
one of these disorders in an Ist-degree relative.
d) Presence of IgM RF on at least 2 occasions at least
3 months apart.
Oligoarticular
JIA
Arthritis affecting 1-4 joints during
the 16 months of disease. Two
subcategories are recognized
1. Persistent oligoarthritis
affecting ≤ 4 joints throughout the
disease course.
2. Extended oligoarthritis
affecting > 4 joints after the 1st 6
month of disease
a, b, c, d (above)
plus
e) Presence of systemic JIA in the patient.
Polyarthritis
(RF-negative)
Arthritis affecting ≥ 5 joints during the 1st 6 months of disease; a test for RF is
negative.
a, b, c,d, e
Polyarthritis
(RF-positive)
Arthritis affecting ≥5 joints during the 1st 6 months of disease; ≥2 tests for RF at
least 3 months apart during the Ist 6 months of disease are positive.
a, b, c, e
Psoriatic arthritis
Arthritis and psoriasis, or arthritis and at least 2 of the following
1. Dactylitis.
2. Nail pitting and onycholysis.
3. Psoriasis is an Ist-degree relative
b, c, d, e
Enthesitis-related arthritis
Arthritis and enthesitis, or arthritis or enthesitis with at least 2 of the
following
1. Presence of or a history of sacroiliac joint tenderness or inflammatory
lumbosacral pain or both.
2. Presence of HLA-B27 antigen.
3. The onset of arthritis in a male> 6 yr old.
4. Acute (symptomatic) anterior uveitis.
5. History of ankylosing spondylitis, enthesitis-related arthritis,
sacroiliitis with IBD, Reiter syndrome, or acute anterior uveitis in a
I st-degree relative.
Undifferentiated
arthritis
Arthritis that fulfills criteria in no category or that fits in 2 of the above categories.

18. CLASSIFICATION
CATEGORY DEFINITION
SYSTEMIC
Arthritis in ≥1 joint with,or preceeded by,fever of at least 2
weeks duration that is documented to be daily (quotidian) for
at least 3 days and accompanied by ≥1 of the following :
1. Evanescent (non fixed) erythematous rash
2. Generalized lymphadenopathy
3. Hepatomegaly or splenomegaly or both
4. serositis
OLIGOARTHRITIS
Arthritis affecting 1-4 joints during the 1st 6 months of disease.
Two subcategories are recognized :
1. Persistent oligoarthritis : affecting ≤4 joints throughout
the disease course
2. Extended oligoarthritis : affecting >4 joints after the 1st 6
months of disease.

19. CLASSIFICATION contd.
CATEGORY DEFINITION
POLYARTHRITIS
( RF-NEGATIVE )
Arthritis affecting ≥5 joints during the 1st 6 months of
disease; a test for RF is negative.
POLYARTHRITIS
(RF-POSITIVE )
Arthritis affecting ≥5 joints during the 1st 6 months of
disease; ≥ 2 tests for RF at least 3 months apart during the
1st 6 months of disease are positive.
PSORIATIC ARTHRITIS
Arthritis and psoriasis, or arthritis and at least 2 of the
following :
1. Dactylitis
2. Nail pitting and onycholysis
3. Psoriasis in a 1st degree relative

20. CLASSIFICATION contd.
CATEGORY DEFINITION
ENTHESITIS RELATED ARTHRITIS Arthritis and enthesitis or arthritis or enthesitis with at
least 2 of the following :
1. Presence of or a history of sacroiliac joint tenderness
or inflammatory lumbosacral pain or both
2. Presence of HLA B 27 antigen
3. Onset of arthritis in a male >6yr old
4. Acute (symptomatic) anterior uveitis
5. History of ankylosing spondylitis, enthesitis-related
arthritis,sacroiliitis with inflammatory bowel disease,
Reiter syndrome, or acute anterior uveitis in a 1st
degree relative.
UNDIFFERENTIATED ARTHRITIS Arthritis that fulfills criteria in no category or in ≥ 2 of the
above categories.

21. Epidemiology
incidence of JIA ranges from 0.8-22.6 per
100,000/year
with prevalence ranges from 7-401 per
100,000/year
Oligoarthritis (40–50%),
Polyarthritis (25–30%)
systemic JIA (5–15%)

22. Epidemiology
Oligo and Polyarticular : girls affected more than
boys
SJIA: There is no sex predominance
The peak age at onset
for oligoarticular disease: 2-4 years
polyarthritis, with peaks at 2-4 yr and 10-14 yr.
sJIA with a peak at 1-5 yr.

23. ETIOLOGY

24. PATHOGENESIS

25. • A. All forms of JIA other than systemic-onset JIA, are
disorders of adaptive immunity autoimmune disorders.
• B. Systemic onset JIA is a disorder of innate immunity, an
autoinflammatory disorder.
• All these immunologic abnormalities cause inflammatory
synovitis, characterized pathologically by villous hypertrophy
and hyperplasia with hyperemia and edema of the synovial
tissue.
• Advanced and uncontrolled disease leads to pannus formation
and progressive erosion of articular cartilage and contiguous
bone

26. Clinical manifestations

27. General Clinical features in JIA
1. Involved joints are often swollen, warm to touch, and
painful on movement or palpation with reduced range of
motion but usually are not erythematous.
2. Morning stiffness with a limp or gelling after inactivity.
3. Easy fatigability and poor sleep quality may be
associated.
4. Arthritis in large joints, especially knees, initially
accelerates linear growth, causing the affected limb to be
longer and resulting in a discrepancy in limb lengths.
5. Continued inflammation stimulates rapid and premature
closure of the growth plate, resulting in shortened bones.

28. Oligoarticular JIA
1. Oligoarthritis involves large joints of the lower
extremities, such as the knees and ankles. Often only
a single joint is involved.
2. Involvement of the hip almost never occurs and if
present suggests a spondyloarthropathy or non-
rheumatologic cause.
3. The presence of a positive antinuclear antibody (ANA)
test result confers increased risk for asymptomatic
anterior uveitis, requiring periodic slit-lamp
examination.

29. Polyarticular JIA
1. RF positive polyarticular disease resembles the characteristic
symmetric presentation of adult rheumatoid arthritis.
2. Rheumatoid nodules on the extensor surfaces of the elbows
and over the Achilles tendons are associated with a more
severe course and almost exclusively occur in RF-positive
individuals.
3. Chronic Temporomandibular joint (TMJ) disease results in
micrognathia.
4. Cervical spine involvement, manifesting as decreased neck
extension, occurs with a risk of atlantoaxial subluxation and
neurologic sequelae.
5. Hip disease may be subtle, with findings of a decreased or
painful range of motion on the exam.

31. systemic-onset JIA
1.Systemic JIA is characterized by arthritis,
fever, and prominent visceral involvement,
including hepatosplenomegaly,
lymphadenopathy, and serositis.
2.Quotidian fever. The fever is often present in
the evening.
3.Characteristic faint, erythematous, macular
rash. Koebner phenomenon is often present.
Heat, such as a warm bath towel, can evoke
rash.
4. Arthritis is classically polyarticular, may be
very destructive, and includes the hip, cervical
spine, and TMJ.

32. Diagnosis
• JIA is a clinical diagnosis without any diagnostic laboratory tests.
• The meticulous clinical exclusion of other diseases and many mimics
is therefore essential.
• Laboratory studies, including tests for ANA and RF, are only
supportive or prognostic, and their results may be normal in patients
with JIA

33. Laboratory findings & investigations
Hemogram :
• anemia of chronic disease with elevated WBC and platelet
counts and microcytic anemia on PS
• low WBC count and low platelet count, keep suspicion about
Macrophage Activation Syndrome (MAS)
• Elevated ESR and C-reactive protein (CRP)

34. Laboratory findings & investigations
• Serology
Elevated ANA titers - Can be seen in 40-85% of children with
oligoarticular or polyarticular JIA. These are rarely seen in SoJIA.
ANA can be associated with an increased risk of chronic uveitis in JIA.
Rheumatic Factor - 5-10% of patients with polyarticular JIA are
seropositive for RF.
Anti-cyclic citrullinated peptide (CCP) antibody - Like RF, it is also a
marker of more aggressive disease.
HLA-B27 - is positive in enthesitis-related form.

35. Radiology Investigations
Xray
Continued active disease
may lead to early
radiographic changes of
arthritis such as
1.Soft tissue swelling
2.Periarticular osteoporosis
and
3.Periosteal new-bone
apposition
4.Subchondral erosions
5.Loss of cartilage

36. MRI
MR is more sensitive than X-rays to early changes.
It is also the most sensitive radiologic indicator of disease activity.
1.Synovial hypertrophy
2.Define soft tissue swelling
3.Demonstrate excellent detail of articular cartilage
4.Anatomy of overall joint integrity.
6.Fusion

37. • Investigations for extra-articular manifestations
1.Cardiac examination and ECHO for pericarditis
2.Ocular examination for Uveitis
3.Chest examination for pleuritis

38. Management
• Children with JIA should be managed with a multidisciplinary
approach and need individualized treatment plans.
• The management is tailored according to
oDisease subtype
oSeverity
oPresence of poor prognostic indicators
oResponse to medications

39. 1.Pharmacologic management
2.Psychosocial factors, including counseling for patients and parents
3.School performance, such as academic counseling, school-life
adjustments, and physical education adjustments.
4.Nutrition, particularly to address anemia and generalized
osteoporosis
5.Physical therapy, to relieve pain and address range of motion,
muscle strengthening, activities of daily living, and conditioning
exercises.
6.Occupational therapy, including joint protection, a program to
relieve pain, range of motion, and attention to activities of daily living.
Management :

40. The primary goals of medical therapy are
1.To eliminate active disease
2.To normalize joint function
3.To preserve normal growth
4.To prevent long-term joint damage

41. Commonly used drugs in the present-day treatment of JIA
Anti-inflammatory drugs
NSAIDs
1.Ibuprofen
2.Naproxen
3.Indomethacin
4.Meloxicam
corticosteroids:
Prednisolone
Corticosteroid joint injections
1. Triamcenolone hexacetonide
2. Triamcenolone acetonide
3. Methyl prednisolone
Conventional
DMARD
1.Methotrexate
2.Sulfasalazine
3.Leflunomide
4.Cyclosporin
Biological DMARD
1.Adalimumab
2.Etanercept
3.lnfliximab
4.Abatacept
5.Toclizumab
6.Rituximab
7.Anakinra

42. SUMMARY OF RECOMMENDATIONS FOR
MEDICATION SAFETY MONITORING

43. JUVENILE ARTHRITIS DISEASE ACTIVITY
SCORE (JADAS)
• Composite disease activity score for JIA which includes ;
1) Physician global assessment of disease activity, measured on
a 10-cm visual analog scale (VAS) where 0 = no activity and
10 = maximum activity
2) Parent/patient global assessment of well-being, measured on
a 10-cm VAS where 0 = very well and 10 = very poor.
3) Count of joints with active disease
4) Erythrocyte sedimentation rate (ESR)

44. Low Disease Activity
(must satisfy all)
Moderate Disease
Activity (does not
satisfy criteria for low
or high activity)
High Disease Activity
(must satisfy at least
3)
1 or fewer active joints 1 or more features
greater than low
disease activity level
AND fewer than 3
features of high
disease activity
2 or more active joints
ESR or C-reactive protein
level normal
ESR or CRP level
greater than twice
upper limit of normal
Physician global
assessment of overall
disease activity < 3 of 10
Physician global
assessment of overall
disease activity ≥ 7 of
10
Patient/parent global
assessment of overall
well-being < 2 of 10
Patient/parent global
assessment of overall
well-being ≥ 4 of 10
DISEASE ACTIVITY oligoarticular
Low Disease Activity
(must satisfy all)
Moderate Disease
Activity (does not satisfy
criteria for low or high
activity)
High Disease Activity
(must satisfy at least 3)
4 or fewer active joints ≥1 feature than low
disease activity level AND
< 3 features of high
disease activity
8 or more active joints
ESR or CRP normal ESR or CRP more than
twice of normal
Physician global
assessment of overall
disease activity < 4 of 10
Physician global
assessment of overall
disease activity >7 of 10
Patient/parent global
assessment of overall
well-being < 2 of 10
Patient/parent global
assessment of overall
well-being ≥5 of 10
DISEASE ACTIVITY POLYARTHRITIS

45. SUMMARY OF RECOMMENDATIONS FOR
MEDICATION SAFETY MONITORING

46. Treatment algorithm for oligoarticular

47. Treatment algorithm for polyarthritis

48. Treatment algorithm sJIA

49. Treatment guidance for Enthesitis
1. NSAID treatment is strongly recommended over no
treatment with an NSAID.
2. Use of TNFi is conditionally recommended over methotrexate
or sulfasalazine in active enthesitis despite treatment with
NSAIDs.
3. Bridging therapy with a limited course of oral glucocorticoids
(<3 months) during initiation or escalation of therapy.
4. Bridging therapy may be more useful in cases with high
disease activity, limited mobility, and/or significant symptoms.

50. Management
• Management of JIA must include periodic slit-lamp ophthalmologic
examinations to monitor for asymptomatic uveitis
• initial management may include mydriatics and corticosteroids used
topically, systemically, or through periocular injection.
• DMARDs allow for a decrease in exposure to steroids, and methotrexate
and TNF-α inhibitors (adalimumab and infliximab) are effective in treating
severe uveitis.
• Dietary evaluation and counseling to ensure appropriate calcium, vitamin
D, protein, and caloric intake are important for children with JIA.
• Physical therapy and occupational therapy are invaluable adjuncts to any
treatment program.

55. PROGNOSIS
• Studies analyzing management of JIA in the pre TNF alpha antagonists era
indicate that up to 50% patients of JIA have active disease persisting into
early adulthood.
• sJIA is most difficult to control in terms of both articular inflammation and
systemic manifestations.
• Data from India suggest that after a median follow-up of 10 years,up to 60%
patients have active disease.

56. THANK YOU