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Paeds Vivasrheumatology-musculoskeletal-and-sports

Paeds Vivas · rheumatology-musculoskeletal-and-sports

Systemic lupus erythematosus: Viva

Branching clinical structured oral on childhood-onset systemic lupus erythematosus: the EULAR/ACR 2019 weighted classification criteria, the type I interferon and immune-complex pathophysiology, the hydroxychloroquine backbone with retinopathy screening, and the stepwise escalation to mycophenolate, belimumab, and anifrolumab for refractory disease.

branching clinical structured oral3 min readVerification in progress

Target exams

RACP DWERACP DCEMRCPCH Clinical
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Target exams

RACP DWERACP DCEMRCPCH Clinical
Prompt
A 14-year-old girl is referred with three months of fatigue, painful mouth ulcers, joint pains in her wrists and fingers, and a red facial rash that worsens in sunlight. She has a malar rash sparing the nasolabial folds, oral ulcers, wrist synovitis, and a low-grade fever. Her antinuclear antibody is strongly positive, her anti-dsDNA is 260 IU per mL, and her complement C3 and C4 are low. Her urinalysis shows blood and protein. The examiner asks how you would classify this disease, explain the mechanism, and design the treatment plan.

Branch 1: Classification

The candidate should classify this as systemic lupus erythematosus using the 2019 European League Against Rheumatism and American College of Rheumatism criteria. The entry criterion is a positive antinuclear antibody at any time, which is met. The weighted score then exceeds the threshold of 10 points: acute cutaneous lupus (the malar rash) scores 6 in the mucocutaneous domain, joint involvement scores 6 in the musculoskeletal domain, low C3 and C4 together score 4, and anti-dsDNA scores 6 in the SLE-specific antibody domain. A strong candidate states that each criterion counts once, the highest within a domain is taken, and a more likely alternative must be excluded. [1]

If the examiner presses on the older criteria, the candidate should contrast the 2019 system with the 1997 American College of Rheumatology criteria (at least four of eleven criteria) and the Systemic Lupus International Collaborating Clinics 2012 criteria (at least four of seventeen, with one clinical and one immunological), demonstrating familiarity with the lineage and the move toward weighting by specificity. The candidate should also flag that the urinalysis abnormality mandates renal biopsy, and that a biopsy showing class III or IV lupus nephritis would classify lupus regardless of the point total via the biopsy shortcut. [1]

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References4ShowHide
  1. [1]Aringer M, Costenbader K, Daikh D, et al 2019 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus Arthritis Rheumatol, 2019.PMID 31385462
  2. [2]Groot N, de Graeff N, Avcin T, et al European evidence-based recommendations for the diagnosis and treatment of childhood-onset lupus nephritis: the SHARE initiative Ann Rheum Dis, 2017.PMID 28877866
  3. [3]Marmor MF, Kellner U, Lai TY, et al Recommendations on Screening for Chloroquine and Hydroxychloroquine Retinopathy (2016 Revision) Ophthalmology, 2016.PMID 26992838
  4. [4]Morand EF, Furie R, Tanaka Y, et al Trial of Anifrolumab in Active Systemic Lupus Erythematosus N Engl J Med, 2020.PMID 31851795
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