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Juvenile Arthritis
1
JIA
• Commonest cause of chronic arthritis in childhood.
• Onset of arthritis before 16th birthday which lasts for atleast 6
weeks and has no known cause.
• Prevalence 1 to 4 per 1000 children.
2
JA classification (Old)
ACR criteria :
• Juvenile arthritis is called as Juvenile Rheumatoid
Arthritis(JRA)
• Duration > 6 wks
• Types : Systemic onset
Oligoarticular(<= 4joints)
Polyarticular(>4 joints)
3
JA classification (Old)
EULAR criteria :
• Juvenile arthritis is called as Juvenile Chronic Arthritis(JCA)
• Duration > 12 wks
• Also included Spondyloarthropathy(jA S, jPs A, IBD associated)
• RA factor positive polyarticular arthritis was called as JRA
• Types : Systemic onset
Oligoarticular
Polyarticular
Juvenile Ankylosing Spondylitis
Juvenile Psoriatic Arthritis
Arthritis associated with IBDs
4
ILAR classification
• Juvenile arthritis is called as Juvenile Idiopathic Arthritis(JIA)
• Duration > 6 wks
• Most widely used classification worldwide
• Types : Systemic onset
Oligoarticular
Polyarticular, RF +ve
Polyarticular, RF –ve
Psoriatic Arthritis
Enthesitis Related Arthritis
Undifferentiated Arthritis
5
ILAR classification types
Systemic Arthritis
• Quotidian fever for atleast 2 weeks
• Evanescent non fixed erythematous rash
• Arthritis
• In the absence of arthritis, presence of fever and rash plus any
two of
Generalised lymphadenopathy
Hepatosplenomegaly
Serositis
6
ILAR classification types
Polyarticular RF –ve :
• Arthritis >5 joints during first 6 months
Polyarticular RF +ve :
• Arthritis >5 joints during first 6 months with positive RF on at least 2
occasions 12 weeks apart.
Oligoarticular :
• Arthritis 1-4 joints during first 6 months
Extended Oligoarticular :
• Affecting more than 4 joints after 6 months of disease
7
ILAR classification types
Enthesitis Related Arthritis :
• Arthritis and Enthesitis or
• Arthritis with at least 2 of,
Sacroiliac joint tenderness
Inflammatory spinal pain
HLA B27, onset of arthritis in a male over 6 years of age
Anterior uveitis
Family history of anterior uveitis/ Spondyloarthropathy/ IBD
8
ILAR classification types
Psoriatic Arthritis :
• Psoriasis and Arthritis or
• Arthritis and family history of psoriasis and presence of
Dactylitis or
Nail abnormalities (pitting, onycholysis)
Undifferentiated Arthritis :
• Arthritis that doesnt fits in any of the above categories or
with features of two or more categories together.
9
Oligo ERA PA RF -ve PA RF +ve Systemic
Frequency
World %
30 – 60 3 -11
(36% in
India - mc)
10 -30 5 - 10 10 - 15
Sex ratio F>M M>F F>M F>M F=M
Uveitis 20%
chronic
25%
acute ant
RF +
ANA 60% 20% 20%
HLA B27 70%
Complicatio
ns
Blindness
Leg length
discrepancy
Spondyloath
ropathy
Joint
destruction
Amyloidosis
MAS
Prognosis Good Good Fair Poor Fair
10
Differential Diagnosis
• Infections – ARF, osteomyelitis, TB
• Malignancy – Leukaemias
• Other CTDs – SLE, juvenile dermatomyositis
11
Investigations
• CBC – anaemia, leucocutosis, thrombocytosis
• Elevated ESR, CRP
• RA factor
• ANA – presents of ANA increases the risk of chronic uveitis
and warrants 3 monthly eye checkup.
• Elevated ferritin and low fibrinogen – MAS
• Elevated ASO titre can be seen 20-25% of JIA patients.
12
Investigations
• Radiographs – r/o other causes
• High resolution USG and MRI – early detection of synovitis
and erosions
• Synovial fluid analysis and Biopsy – in persistent monoarthritis
to exclude chronic infection
13
Treatment
Physical and occupational therapy :
• Splints, exercises, modified footwear
Medical therapy :
• NSAIDs
• DMARDs – HCQs, Methotrexate, Sulfasalzine
• Steroids – Oral, IV, intraarticular
• Biologicals – TNF antagonists
14
Treatment of Enthesitis Related JIA
• Mild disease - < 4 peripheral joint involvement without critical
joint like hip joint
- NSAIDs and intraarticular steroids
• Severe disease – hip joint involvement
- Methotrexate
- Sulfasalazine
- TNF blockers – if no improvement in 3 months.
• Acute uveitis – Topical steroids
15
THANK YOU
16

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Juvenile Idiopathic Arthritis

  • 2. JIA • Commonest cause of chronic arthritis in childhood. • Onset of arthritis before 16th birthday which lasts for atleast 6 weeks and has no known cause. • Prevalence 1 to 4 per 1000 children. 2
  • 3. JA classification (Old) ACR criteria : • Juvenile arthritis is called as Juvenile Rheumatoid Arthritis(JRA) • Duration > 6 wks • Types : Systemic onset Oligoarticular(<= 4joints) Polyarticular(>4 joints) 3
  • 4. JA classification (Old) EULAR criteria : • Juvenile arthritis is called as Juvenile Chronic Arthritis(JCA) • Duration > 12 wks • Also included Spondyloarthropathy(jA S, jPs A, IBD associated) • RA factor positive polyarticular arthritis was called as JRA • Types : Systemic onset Oligoarticular Polyarticular Juvenile Ankylosing Spondylitis Juvenile Psoriatic Arthritis Arthritis associated with IBDs 4
  • 5. ILAR classification • Juvenile arthritis is called as Juvenile Idiopathic Arthritis(JIA) • Duration > 6 wks • Most widely used classification worldwide • Types : Systemic onset Oligoarticular Polyarticular, RF +ve Polyarticular, RF –ve Psoriatic Arthritis Enthesitis Related Arthritis Undifferentiated Arthritis 5
  • 6. ILAR classification types Systemic Arthritis • Quotidian fever for atleast 2 weeks • Evanescent non fixed erythematous rash • Arthritis • In the absence of arthritis, presence of fever and rash plus any two of Generalised lymphadenopathy Hepatosplenomegaly Serositis 6
  • 7. ILAR classification types Polyarticular RF –ve : • Arthritis >5 joints during first 6 months Polyarticular RF +ve : • Arthritis >5 joints during first 6 months with positive RF on at least 2 occasions 12 weeks apart. Oligoarticular : • Arthritis 1-4 joints during first 6 months Extended Oligoarticular : • Affecting more than 4 joints after 6 months of disease 7
  • 8. ILAR classification types Enthesitis Related Arthritis : • Arthritis and Enthesitis or • Arthritis with at least 2 of, Sacroiliac joint tenderness Inflammatory spinal pain HLA B27, onset of arthritis in a male over 6 years of age Anterior uveitis Family history of anterior uveitis/ Spondyloarthropathy/ IBD 8
  • 9. ILAR classification types Psoriatic Arthritis : • Psoriasis and Arthritis or • Arthritis and family history of psoriasis and presence of Dactylitis or Nail abnormalities (pitting, onycholysis) Undifferentiated Arthritis : • Arthritis that doesnt fits in any of the above categories or with features of two or more categories together. 9
  • 10. Oligo ERA PA RF -ve PA RF +ve Systemic Frequency World % 30 – 60 3 -11 (36% in India - mc) 10 -30 5 - 10 10 - 15 Sex ratio F>M M>F F>M F>M F=M Uveitis 20% chronic 25% acute ant RF + ANA 60% 20% 20% HLA B27 70% Complicatio ns Blindness Leg length discrepancy Spondyloath ropathy Joint destruction Amyloidosis MAS Prognosis Good Good Fair Poor Fair 10
  • 11. Differential Diagnosis • Infections – ARF, osteomyelitis, TB • Malignancy – Leukaemias • Other CTDs – SLE, juvenile dermatomyositis 11
  • 12. Investigations • CBC – anaemia, leucocutosis, thrombocytosis • Elevated ESR, CRP • RA factor • ANA – presents of ANA increases the risk of chronic uveitis and warrants 3 monthly eye checkup. • Elevated ferritin and low fibrinogen – MAS • Elevated ASO titre can be seen 20-25% of JIA patients. 12
  • 13. Investigations • Radiographs – r/o other causes • High resolution USG and MRI – early detection of synovitis and erosions • Synovial fluid analysis and Biopsy – in persistent monoarthritis to exclude chronic infection 13
  • 14. Treatment Physical and occupational therapy : • Splints, exercises, modified footwear Medical therapy : • NSAIDs • DMARDs – HCQs, Methotrexate, Sulfasalzine • Steroids – Oral, IV, intraarticular • Biologicals – TNF antagonists 14
  • 15. Treatment of Enthesitis Related JIA • Mild disease - < 4 peripheral joint involvement without critical joint like hip joint - NSAIDs and intraarticular steroids • Severe disease – hip joint involvement - Methotrexate - Sulfasalazine - TNF blockers – if no improvement in 3 months. • Acute uveitis – Topical steroids 15