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.
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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]
References3ShowHide
- [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]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]Ramirez V, Mercier-Ross J Acute Chest Syndrome in Children with Sickle Cell Disease: A Narrative Review. Children (Basel), 2026.PMID 42194196