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Information for Doctors

Juvenile Arthritis
Introduction
Causes of acute onset Juvenile arthritis:
Causes of chronic arthritis in children:
Juvenile rheumatoid arthritis:
  Classification:
Differentiating features of three types of JRA:
Extraarticular manifestations of systemic onset JRA
Diagnostic criteria for JRA
Differentiating features between systemic JRA and SLE:
Treatment:

Introduction:

Juvenile arthritis may be acute or chronic in nature. Under the EULAR criteria, arthritis is considered chronic if it is at least 3 months in duration.

Causes of acute onset Juvenile arthritis:

1.      Infections:

a.       Bacterial:

·        staphylococcus

·        Streptococcus

·        Tuberculous

      b.   Viral

  • Rubella

  • HIV

  • Mumps

  • Epstein barf viruses

      c.   Others

  • Mycoplasma

2.   Reactive

a.       Rheumatic fever

b.      Postvaccination

c.       Hepatitis B

d.      Lyme disease

e.       Postsreptococcal arthritis

f.        Transient synovitis

 

3.   Neoplastic

  1. Leukemia and other malignancies

  2. Hemolytic anaemia

  3. Neuro blastoma

  4. Familial mediterrean fever

  5. Metastasis

4.   Vasculitis

 

5.   Juvenile rheumatoid arthritis

 

6.   Hemophilia

Causes of chronic arthritis in children:

1. Idiopathic childhood arthritis (Juvenile rheumatoid arthritis):

    It may be

a.       Oligoarticular onset JRA

b.      Polyarticular onset JRA

c.       Systemic - onset JRA

2. Connective tissue disorder

a.       SLE

b.      Dermatomyositis

c.       Scleroderma

3. Vasculitides

a.       Kawasaki disease involving smaIl joints

b.      Henoch schonlein purpura

c.       Polyarteritis nodosa

4.  Spondyloarthropathy

a.       Juvenile spondyloathropathy

b.      Ankylosing spondylitis

c.       Reiter's syndrome

d.      Psoriatic arthritis

e.       Inflammatory bowel disease

5.   Others:

a.       Marfan syndrome

b.      Ehlers danlos syndrome

c.       Cystic fibrosis

d.      Hemolytic anaemia

e.       Sarcoidosis

f.        Villonodular synovitis

g.       Mucopolysaccharidoses

h.       Familial hypertrophic synovitis

More commonly monoarticular / oligoarticular

Differentiating features between organic and functional joint pains:
Refer to table : Click here

Juvenile rheumatoid arthritis:

JRA usually starts between 1 and 4 years of age and near by 9 and 14 years of age.

Classification:

Depending upon the number of joints involved during the first 6 months after disease onset (not the number of joints involved when the child is first seen by doctor) JRA is classified into following subtypes:

A.     Polyarticular: Multiple joints, small or large are involved in a symmetrical fashion, more common in female. It can be subclassified into following types

a.       RA-positive polyarticular: Usually late childhood onset, RA nodule is common. It is associated with poorer prognosis.

b.      RA-negative polyarthritis: Better prognosis, may present in early or late childhood.

B.  Pauciarticular: One or few (<5)

      Large joints, more commonly asymmetrically involved. They are further divided in to 2 subgroups:

  1. Early childhood (between 2-5years): Female more commonly involved, ANA is frequently positive, there is incidence of chronic iritis. Mild arthritis with usually good prognosis
  2. Late childhood (> 6 years): More often in males, more often HLA B-27 positive, ANA negative, some may have spondyloarthropathy like adults.
  1. Systematic onset: It is characterized by polyarthalgia, polyarthritis with systemic manifestations such as high fevers, evenescent rash etc. The gender frequency as well as age of onset is equally distributed throughout childhood. Usually RA and ANA negative.
Differentiating features of three types of JRA:
Refer to table : Click here

Extraarticular manifestations of systemic onset JRA:

  1. Intermittent fever: A persistent intermittent fever with diurnal variation between 102° f and 106° f with returns to normal is a must for diagnosis.

  2. Skin rash: Evanescent, pink, macular pale centre, on chest and limbs may be seen in associated with fever.

  3. Hepatomegaly / mild liver dysfunction

  4. Pericarditis

  5. Splenomegaly

  6. Leucocytosis

  7. Lymphadenopathy

  8. Severe anaemia

  9. DIC syndome

Diagnostic criteria for JRA

  1. Age of onset below 16 years

  2. Polyarthritis or monoarticular arthritis lasting longer than 3 month

  3. Swelling or presence of atleast 2 of following:

a.       Heat

b.      Pain

c.       Tenderness

d.      Restriction of motion

 

       4.  Type of onset, classified as:

a.       Polyarthritis

b.      Oligoarthritis

c.       Systemic illness: including arthritis, intermittent fever, rheumatoid rash.

 

       5.  Exclusion of other forms of arthritis

 

Differentiating features between systemic JRA and SLE:
Refer to table : Click here

Treatment:

Medical management:

 

A.     Nonsteroidal antiinflammatory drugs

1.      Salicylate: (80 -130 mg/kg 1 day)

2.      Indomethacin: (0.5 -3 mg/kg 1 day)

 

B.   Slowly acting antirheumatic drugs:

  1. 1M gold: 1 mg/kg weekly interval

  2. Auranofin: 0.15 -0.20 mg/kg 1 day

  3. Hydroxychroquine: 5 -7 mg/kg 1 day

  4. Sulfasalazine: 40 mg/kg/day

  5. Methotrexate: 0.15 -0.5 mg/kg/wk.

  6. Etarnacept: 0.4 mg/kg

C.  Steroids

  1. Prednisolone: 1 -2 mg/kg/day

D.  Conservative orthopaedic management:

  1. Rest

  2. Exercise: gradual excercise within pain tolerance limits

  3. Heat therapy

  4. Orthosis or plaster cast

  5. Traction if needed

E  Family support

 

F  Regular eye-check ups and treatment

 

G  Surgery: Surgical options include

  1. Synovectomy

  2. Joints replacement / arthrodesis in severly affected joints

  3. Corrective osteotomies in joint deformities

  4. Soft tissue release in joint deformities