Paediatric rheumatology

Question 1

Osgood-Schlatter disease

Answer 1

Inflammation of the patellar ligament at the tibial tuberosity
Male> Female
In athletic adolescent
Tenderness and swelling over attachment of patellar tendon
Radiograph shows soft tissue swelling, enlarged and sometimes fragmented tubercle

Question 2

Chondromalacia Patellae

Answer 2

Inflammation of the underside of the patella and softening of the cartilage
Exertional knee pain. Pain with going up or down stairs. Prefer to sit with straight knee. Positive patellar grind

Question 3

Causes of hip pain in children

Answer 3

1. Transient synovitis, malignancy—local, benign (e.g., osteoid osteoma), malignant (e.g., Ewing’s sarcoma), generalized, leukemia, neuroblastoma
2. Septic arthritis
3. Avascular necrosis (Legg–Calvé–Perthes disease)
4. Slipped capital femoral epiphysis (SCFE)
5. Protrusio acetabuli
6. Osteomyelitis (femur, pelvis)
7. JIA (including enthesitis-related arthritis [ERA]/spondyloarthropathy)
8. Rheumatic fever

Question 4

Classification of JIA

Answer 4

• Systemic JIA (10% to 15% of all JIA)
• Oligoarthritis (30% to 60%)
• Polyarthritis, RF positive (5% to 10%)
• Polyarthritis, RF negative (10% to 30%)
• Psoriatic arthritis (2% to 15%)
• Enthesitis-related arthritis (ERA)/juvenile spondyloarthropathy (JAS) (20%)
• Undifferentiated arthritis

Question 5

Two types of uveitis in JIA

Answer 5

1. Acute uveitis. Seen in SpA. usually hot, red, photophobic eye. Associated with HLA-B27. Usually not much complications. Does not need slit lamp surveillance every year.
2. Chronic uveitis. Seen in oligo JIA. Associated with ANA+, usually asymptomatic. Can lead to synechiae, cataract, glaucoma, band keratopathy. Needs slit lamp exam screening.

Question 6

Enthesitis related arthritis includes:

Answer 6

1. juvenile ankylosing spondylitis
2. Spondylitis, enthesitis and arthritis syndrome (SEA syndrome)
3. Reactive arthritis
4. IBD associated arthritis

Question 7

PLEX is indicated in these conditions

Answer 7

thrombotic thrombocytopenic purpura (TTP), catastrophic antiphospholipid syndrome (CAPS), transverse myelitis (antineuromyelitis optica, anti-NMO), and in some cases pulmonary hemorrhage secondary to vasculitis, very refractory cryoglobulinemic vasculitis

Question 8

Poor Prognostic Factors at Onset of childhood HSP

Answer 8

1. Melena: 7.5-fold increase in renal disease
2. Persistent rash for 2-3 months: Associated with glomerulonephritis
3. Hematuria with proteinuria >1 g/day: 15% progress to renal insufficiency
4. Nephrosis with renal insufficiency: 50% renal insufficiency in 10 years

Question 9

Treatment for childhood HSP?

Answer 9

• Arthritis responds to NSAIDs
• Edema responds to steroids
• Abdominal pain with positive test for blood in stool should be treated with steroids
• Evaluate for and treat infection (e.g., group A β-hemolytic streptococci, Helicobacter pylori)
• Recurrent skin purpura may respond to dapsone
• Aggressive treatment (high-dose or IV pulse corticosteroids) for children with poor prognostic signs
• Severe nephritis: high-dose IV pulse methylprednisolone plus azathioprine or cyclophosphamide; angiotensin-converting enzyme (ACE) inhibitors for proteinuria

Question 10

Treatment of localized scleroderma in children

Answer 10

Localized scleroderma is more common than SSc in children
Treatment: high-dose IV methylprednisolone initially followed by prednisone with tapering over 3 to 6 months. Methotrexate is coadministered with the steroids and continued for at least 2 years.
There is a 30% recurrence rate after methotrexate is stopped.

Question 11

Factors associated with poor prognosis in Kawasaki’s disease

Answer 11

Age < 1 year or > 9 years; 
male sex; 
Asian ancestry; 
fever > 14 days; 
WBC count > 12,000/mm3. 
Patients with these characteristics are more likely to develop coronary aneurysms (50%).

Question 12

Effective treatment of Kawasaki’s disease

Answer 12

1. IVIG at a dose of 2 g/kg given over 12 hours within the first 10 days of illness
2. In conjunction with high-dose aspirin (30 to 50 or 80 to 100 mg/kg/day) .
   IVIG promotes resolution of the acute symptoms and prevention of coronary aneurysm formation.
3. Once the fever has resolved, the dose of aspirin is decreased to 3 to 5 mg/kg/day for its antiplatelet effect.
   The implementation of this approach within 10 days of disease onset reduces the incidence of coronary aneurysms from 20% to 25% to 3% to 5%.