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PE21.1-5 | Rheumatology — PBL Case

CLINICAL SETTING

The paediatric outpatient department at a district government hospital in Tamil Nadu, India. It is Monday morning and the department is busy. Dr Kavitha, the paediatric registrar, reviews three children referred from primary health centres. Each child has a different rheumatological problem, but all three families are anxious, confused, and have received varied advice at previous visits. Dr Kavitha must assess each child systematically, apply the correct classification criteria, and counsel the families appropriately — all within a morning clinic.

Trigger 1: Child 1: The Boy with Spots and a Sore Tummy

Arjun, a 7-year-old boy, is brought by his mother with a 5-day history of a 'spotty rash' over his legs and buttocks that does not blanch on pressing. He also has severe colicky abdominal pain and vomiting, and his ankles are swollen. His mother reports that the rash appeared after a throat infection two weeks ago. On examination: temperature 37.4°C; palpable purpura over bilateral lower limbs and buttocks; bilateral ankle swelling with tenderness; diffuse periumbilical tenderness on palpation; no guarding. Platelet count: 280,000/µL. Urinalysis: 2+ blood, 2+ protein. Blood pressure: 95/60 mmHg (normal for age).

DISCUSSION POINTS

  • What is the most likely diagnosis and which classification criteria apply? What mandatory finding must be present for this diagnosis?
  • Why is the platelet count normal, and how does this help differentiate this condition from immune thrombocytopenic purpura (ITP)?
  • What does the urinalysis finding indicate, and what further investigation of renal function is required?
  • What are the indications for corticosteroid use in this condition? Is Arjun's abdominal pain an indication?
Click to reveal Trigger 2: Child 2: The Toddler with Fever and Red Eyes (discuss previous trigger first!)

Trigger 2: Child 2: The Toddler with Fever and Red Eyes

Meera, a 3-year-old girl, is brought by her father with 7 days of high fever (up to 39.8°C), red eyes, and a rash over her trunk. On examination: bilateral non-purulent conjunctival injection; cracked, bleeding lips with a 'strawberry tongue'; a polymorphous erythematous rash over the trunk; oedema and erythema of both palms; a single right cervical lymph node measuring 1.8 cm (non-tender, non-fluctuant). The father reports that an 'antibiotic' given at a local clinic for 3 days did not help. Her WBC is 18,000/µL (neutrophilia); CRP 98 mg/L; ESR 88 mm/hour; platelets 480,000/µL. An echocardiogram is urgently arranged.

DISCUSSION POINTS

  • What is the diagnosis? Apply the diagnostic criteria specifically, naming each feature present in the vignette and identifying whether criteria are fully met.
  • What is the single most important complication Meera is at risk for, and what does the echocardiogram look for? Define the Z-score threshold for a normal, small, medium, and giant coronary aneurysm.
  • What is the evidence-based treatment that must be given within 10 days of fever onset? What is the dose, route, and expected outcome?
  • The father asks: 'The antibiotic did nothing — why?' How do you explain the pathophysiology of this condition to a worried parent in simple terms?
Click to reveal Trigger 3: Child 3: The Teenager with Joint Pain — and a Surprise Finding (discuss previous trigger first!)

Trigger 3: Child 3: The Teenager with Joint Pain — and a Surprise Finding

Priya, a 14-year-old girl, is seen with a 6-month history of pain and swelling in both knees, both ankles, both wrists, and the right elbow (6 joints). Morning stiffness lasts about 1 hour. She also reports fatigue, mild hair loss, and two episodes of a butterfly-shaped facial rash after sun exposure. On examination: active synovitis in all 6 joints listed; malar rash; oral ulcer on the left buccal mucosa; mild pallor. Investigations: ANA 1:640 (homogeneous); anti-dsDNA: 1:320; anti-Sm: positive; C3: 0.58 g/L (low); C4: 0.06 g/L (low); Hb 9.2 g/dL (normocytic); WBC 3,200/µL; platelets 95,000/µL; creatinine 0.9 mg/dL; urinalysis: 3+ protein, 2+ blood; 24-hour urine protein: 2.8 g.

DISCUSSION POINTS

  • Does Priya meet SLICC 2012 criteria for SLE? List the criteria she fulfils, distinguishing clinical from immunological domains. Does she meet the minimum threshold?
  • What ISN/RPS histological class of lupus nephritis is most likely given her urinalysis and proteinuria? What would you expect to see on renal biopsy?
  • Plan Priya's initial pharmacological management: which medications form the backbone of SLE treatment regardless of severity? What induction regimen is appropriate for her nephritis?
  • Priya asks: 'Does this mean I can't go to school or exercise?' How do you counsel her about managing SLE as a teenager in India — sun protection, schooling, fertility, and long-term monitoring?
Click to reveal Trigger 4: Afternoon Follow-up: Integrating the Learning (discuss previous trigger first!)

Trigger 4: Afternoon Follow-up: Integrating the Learning

At the end of clinic, Dr Kavitha presents all three cases at the afternoon teaching round. The senior registrar asks the group to reflect on the broader learning. She points out: all three children had delayed diagnosis — Arjun by 5 days (dismissed as 'viral rash'), Meera by 7 days (treated with antibiotics), and Priya by 3 months (initially thought to be 'growing pains'). The trainees are asked to develop a recognition framework for the paediatric primary care setting.

DISCUSSION POINTS

  • Create a structured 'recognition checklist' for the paediatric primary care provider: which clinical features should prompt urgent rheumatology referral in a child with joint pain, rash, or prolonged fever?
  • For each of the three diagnoses, state the single investigation that would be MOST useful as a first-line screening test in a primary care setting with limited resources.
  • Discuss how early recognition and referral impacts long-term outcomes for each condition — specifically, coronary aneurysm prevention in Kawasaki, nephritis prognosis in HSP, visual loss prevention in JIA, and renal/cardiovascular outcomes in SLE.
  • Reflect on a common misconception in each condition that contributed to the diagnostic delay in these three cases. How would you address these misconceptions in a public health education message?

Group Task Assignments

Group 1: Collaborative Task

Group 2: Collaborative Task

Group 3: Collaborative Task

Learning Issues

Research these questions and bring your findings to the discussion.

  1. [PE21.1] What are the common rheumatological conditions in children presenting to an outpatient clinic in India, and what clinical features should prompt urgent referral to a paediatric rheumatologist?
  2. [PE21.2] What is the etiopathogenesis, diagnostic criteria (EULAR/PRINTO/PRES 2010), and management of Henoch-Schönlein Purpura, including indications for corticosteroids and duration of renal monitoring?
  3. [PE21.3] What is the etiopathogenesis, diagnostic criteria (AHA), management (IVIG + aspirin), and approach to IVIG-resistant Kawasaki Disease, and how are coronary artery aneurysms classified and monitored?
  4. [PE21.4] What are the etiopathogenesis, SLICC/EULAR-ACR diagnostic criteria, key autoantibodies, ISN/RPS classification of lupus nephritis, and treatment approach for paediatric SLE?
  5. [PE21.5] What are the ILAR subtypes of JIA, why does oligoarticular ANA-positive JIA carry the highest uveitis risk, and what is the evidence-based treatment ladder from NSAIDs through methotrexate to anti-TNF biologics?