Pediatrics
Rheumatologic Problems
Paediatric rheumatology for NEET-PG: juvenile idiopathic arthritis, acute rheumatic fever, Kawasaki disease, SLE and IgA vasculitis, mapped to NMC PE22 codes.
MedNext Academy | 3 min read
Rheumatologic Problems
Paediatric rheumatology for NEET-PG: juvenile idiopathic arthritis, acute rheumatic fever, Kawasaki disease, SLE and IgA vasculitis, mapped to NMC PE22 codes.
This chapter covers the major paediatric rheumatologic conditions: juvenile idiopathic arthritis, acute rheumatic fever, Kawasaki disease and the connective tissue and vasculitic disorders including SLE, juvenile dermatomyositis and IgA vasculitis. It stresses early recognition to prevent blindness, valve damage and coronary injury.
High-yield: Rheumatologic Problems
- Juvenile idiopathic arthritis requires arthritis lasting at least 6 weeks before 16 years of age, but a child with definite inflammatory arthritis and red flags should be referred without waiting the full 6 weeks.
- ANA-positive girls with oligoarticular JIA onset before age 6 have the highest uveitis risk, so the first slit-lamp examination should happen at diagnosis, not when the eye turns red.
- Before a general anaesthetic in JIA, exclude atlantoaxial instability with a flexion lateral cervical spine X-ray, and remember temporomandibular joint disease can cause micrognathia.
- Unremitting fever with falling platelets and a paradoxically falling erythrocyte sedimentation rate in systemic JIA suggests macrophage activation syndrome and is an emergency.
- The revised Jones criteria in Indian high-risk settings count monoarthritis and polyarthralgia as major criteria, and echocardiographic subclinical carditis counts as carditis.
- In acute rheumatic fever the arthritis and Sydenham chorea resolve completely, but carditis with valvulitis can scar and, on recurrence, becomes rheumatic heart disease.
- The Kawasaki disease answer is fever for at least 5 days plus mucocutaneous signs, a single intravenous immunoglobulin dose of 2 g/kg, aspirin, and serial echocardiography for the coronary arteries.
- Infants under 6 months with Kawasaki disease have the highest coronary aneurysm risk and most often present incompletely, so prolonged fever must not be dismissed as viral.
- A red, swollen BCG scar site in an Indian infant with prolonged fever is a highly specific clue to Kawasaki disease in the first two years of life.
- A giant coronary aneurysm is defined by a Z-score of at least 10 or an absolute diameter of at least 8 mm and carries the highest risk of thrombosis and myocardial infarction.
- In childhood systemic lupus erythematosus, low complement with rising anti-double-stranded DNA during a flare is active lupus nephritis until proven otherwise, and biopsy guides the class.
- Juvenile dermatomyositis presents with proximal muscle weakness, a heliotrope rash, Gottron papules and raised creatine phosphokinase, and calcinosis develops in undertreated disease.
- Henoch-Schonlein purpura (IgA vasculitis) shows palpable purpura over the buttocks and lower limbs with a normal platelet count; thrombocytopenic purpura should prompt a different diagnosis.
- In HSP the key long-term risk is renal, so urine should be followed for at least 6 months after the rash resolves, and significant proteinuria warrants renal biopsy.
- In an anti-Ro positive pregnancy, fetal heart rate surveillance between 18 and 26 weeks detects early atrioventricular block, and third-degree block is usually irreversible.
Key rheumatology anchors
- **JIA definition:** Arthritis for at least 6 weeks with onset before 16 years of age.
- **Kawasaki treatment:** IVIG 2 g/kg once plus aspirin, with serial echocardiography for coronary arteries.
- **Giant coronary aneurysm:** Z-score of at least 10 or absolute diameter of at least 8 mm.
- **Macrophage activation syndrome:** Falling platelets and paradoxically falling ESR with rising ferritin in systemic JIA.
NMC competencies in this chapter
- **PE22.1:** Juvenile Idiopathic Arthritis: Recognition, Subtypes, Complications and Management
- **PE22.2:** Acute Rheumatic Fever: Jones Criteria, Management and Prevention
- **PE22.3:** Kawasaki Disease: Diagnosis, Management and Coronary Complications
- **PE22.4:** SLE, Juvenile Dermatomyositis and IgA Vasculitis in Children
Frequently Asked Questions
How is Kawasaki disease treated and why is timing critical?
Treatment is a single intravenous immunoglobulin dose of 2 g/kg with aspirin, plus serial echocardiography of the coronary arteries. Infants under 6 months have the highest aneurysm risk and often present incompletely, so prolonged fever must not be dismissed as viral.
Which JIA children are at highest risk of uveitis?
ANA-positive girls with oligoarticular onset before age 6 have the highest uveitis risk. The first slit-lamp examination should happen at diagnosis, because by the time the eye turns red, damage is already present.
What is the key long-term concern in Henoch-Schonlein purpura?
Renal involvement is the key long-term risk. Urine should be followed for at least 6 months after the rash resolves, and significant proteinuria warrants renal biopsy, since HSP nephritis is histologically identical to IgA nephropathy.
How do you know acute rheumatic fever carditis has become important?
Arthritis and chorea resolve completely, but carditis with valvulitis can scar. Repeated attacks cause cumulative valve damage, which becomes rheumatic heart disease, so secondary prophylaxis is central to prevention.
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