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Paeds Vivasrespiratory-sleep-and-airway

Paeds Vivas · respiratory-sleep-and-airway

Respiratory manifestations of systemic disease — structured oral (viva)

Branching structured oral on a child with juvenile dermatomyositis and evolving breathlessness, testing recognition of connective tissue interstitial lung disease, the disease-specific reflex, and the danger of the anterior mediastinal mass.

branching clinical structured oral2 min readVerification in progress

Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics
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Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics
Prompt
A 12-year-old girl with juvenile dermatomyositis, diagnosed eight months ago and on maintenance therapy, is reviewed with two months of progressive exertional breathlessness, a dry cough, and reduced exercise tolerance. Her rash and proximal weakness are currently mild. On examination she has fine bibasal crackles, early digital clubbing, and an oxygen saturation of 94 percent.

Branch 1 — The concern and first thought

Examiner: "What concerns you most about this girl, and what is your leading diagnosis?" Candidate: What concerns me most is progressive exertional breathlessness with a dry cough, fine bibasal crackles, and early clubbing in a child with juvenile dermatomyositis, because that combination points to interstitial lung disease rather than deconditioning. In juvenile dermatomyositis the lung can be affected out of proportion to the skin and muscle, so her currently mild rash and weakness do not reassure me. My leading diagnosis is connective tissue disease-associated interstitial lung disease, and I would treat this as a lung complication of her systemic disease. [1]

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References3ShowHide
  1. [1]Lepage M, Pereira G, Akhalwaya S, et al MDA5-associated juvenile dermatomyositis and interstitial lung disease from rapidly progressive to silent: a report of three cases in South African children and a review of the literature. Clin Rheumatol, 2026.PMID 42018270
  2. [2]Ikeda O, Tsujioka Y, Nishimura G, et al Imaging Spectrum of Childhood Interstitial Lung Diseases: Focus on Disorders Not Specific to Infancy. Radiographics, 2026.PMID 42241322
  3. [3]Ramirez V, Mercier-Ross J Acute Chest Syndrome in Children with Sickle Cell Disease: A Narrative Review. Children (Basel), 2026.PMID 42194196
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