Symptoms of shrinking lung syndrome reveal systemic lupus erythematosus in a 12-year-old girl

Pediatr Pulmonol. 2013 Dec;48(12):1246-9. doi: 10.1002/ppul.22704. Epub 2012 Nov 8.


While pleuropulmonary involvement in systemic lupus erythematosus (SLE) is a common occurrence, shrinking lung syndrome (SLS) is a rare complication of SLE, particularly in children. We report on a teenager girl with a primary SLE diagnosis, which was based upon clinical, imaging, lung-function and histological findings ascertained to be compatible with SLS. Following a pneumonia, the patient developed inflammatory residues in the lower lobes, an event that probably caused diaphragmatic immobility and subsequently led to SLS. Treatment response to steroids, cyclophosphamide and hydroxychloroquine in this case was excellent, and efficacy was more profound than previously has been reported in the literature with respect to pediatric patients. This case report argues that prognosis of SLS in SLE is likely to be favorable when the diagnosis is made early and the disease is treated appropriately.

Keywords: corticosteroids; immunosuppressive therapy; restrictive ventilatory disorder; shrinking lung syndrome; systemic lupus erythematosus.

Publication types

  • Case Reports

MeSH terms

  • Chest Pain / etiology
  • Child
  • Cyclophosphamide / therapeutic use
  • Diaphragm / diagnostic imaging*
  • Diaphragm / physiopathology
  • Dyspnea / etiology
  • Female
  • Humans
  • Hydroxychloroquine / therapeutic use
  • Immunosuppressive Agents / therapeutic use
  • Lupus Erythematosus, Systemic / complications
  • Lupus Erythematosus, Systemic / diagnosis*
  • Lupus Erythematosus, Systemic / drug therapy
  • Methylprednisolone / therapeutic use
  • Mycophenolic Acid / analogs & derivatives
  • Mycophenolic Acid / therapeutic use
  • Pleurisy / etiology
  • Pulmonary Atelectasis / diagnostic imaging*
  • Pulmonary Atelectasis / etiology
  • Pulmonary Atelectasis / physiopathology
  • Radiography
  • Syndrome
  • Treatment Outcome


  • Immunosuppressive Agents
  • Hydroxychloroquine
  • Cyclophosphamide
  • Mycophenolic Acid
  • Methylprednisolone