Pleural fibrosis mediates shrinking lungs syndrome in children

Esther H. Krych, Philip R. Fischer, Mark E. Wylam

Research output: Contribution to journalArticlepeer-review

10 Scopus citations


The etiology of restrictive lung physiology in SLS is not well-defined, and has been hypothesized to be due to defects in lung recoil, phrenic nerve function and diaphragmatic strength. We present a case of SLS in an adolescent in whom imaging and electrophysiology studies demonstrate pleural fibrosis as the fundamental defect accounting for the restrictive lung physiology.

Original languageEnglish (US)
Pages (from-to)90-92
Number of pages3
JournalPediatric pulmonology
Issue number1
StatePublished - Jan 2009


  • Pleural fibrosis
  • SLE/lupus
  • Shrinking lungs syndrome
  • Vanishing lungs syndrome
  • Zone of apposition

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Pulmonary and Respiratory Medicine


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