title: Shrinking lung syndrome as a manifestation of pleuritis: a new model based on pulmonary physiological studies.
abstract: OBJECTIVE
  The pathophysiology of shrinking lung syndrome (SLS) is poorly understood. We sought to define the structural basis for this condition through the study of pulmonary mechanics in affected patients.
  
  
  METHODS
  Since 2007, most patients evaluated for SLS at our institutions have undergone standardized respiratory testing including esophageal manometry. We analyzed these studies to define the physiological abnormalities driving respiratory restriction. Chest computed tomography data were post-processed to quantify lung volume and parenchymal density.
  
  
  RESULTS
  Six cases met criteria for SLS. All presented with dyspnea as well as pleurisy and/or transient pleural effusions. Chest imaging results were free of parenchymal disease and corrected diffusing capacities were normal. Total lung capacities were 39%-50% of predicted. Maximal inspiratory pressures were impaired at high lung volumes, but not low lung volumes, in 5 patients. Lung compliance was strikingly reduced in all patients, accompanied by increased parenchymal density.
  
  
  CONCLUSIONS
  Patients with SLS exhibited symptomatic and/or radiographic pleuritis associated with 2 characteristic physiological abnormalities: (1) impaired respiratory force at high but not low lung volumes; and (2) markedly decreased pulmonary compliance in the absence of identifiable interstitial lung disease. These findings suggest a model in which pleural inflammation chronically impairs deep inspiration, for example through neural reflexes, leading to parenchymal reorganization that impairs lung compliance, a known complication of persistently low lung volumes. Together these processes could account for the association of SLS with pleuritis as well as the gradual symptomatic and functional progression that is a hallmark of this syndrome.
authors: Division of Immunology, Boston Children's Hospital, Boston, MA 02115, USA.
authors: Lauren A
authors: LA
authors: Henderson
authors: Stephen H
authors: SH
authors: Loring
authors: Ritu R
authors: RR
authors: Gill
authors: Katherine P
authors: KP
authors: Liao
authors: Rumey
authors: R
authors: Ishizawar
authors: Susan
authors: S
authors: Kim
authors: Robin
authors: R
authors: Perlmutter-Goldenson
authors: Deborah
authors: D
authors: Rothman
authors: Mary Beth F
authors: MB
authors: Son
authors: Matthew L
authors: ML
authors: Stoll
authors: Lawrence S
authors: LS
authors: Zemel
authors: Christy
authors: C
authors: Sandborg
authors: Paul F
authors: PF
authors: Dellaripa
authors: Peter A
authors: PA
authors: Nigrovic
mesh_terms: D000293:Adolescent; D000328:Adult; D004417:Dyspnea / Q000000981:diagnostic imaging / Q000209:etiology* / Q000503:physiopathology; D005260:Female; D006801:Humans; D008168:Lung / Q000000981:diagnostic imaging / Q000503:physiopathology*; D008171:Lung Diseases / Q000000981:diagnostic imaging / Q000209:etiology* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D010998:Pleurisy / Q000150:complications* / Q000000981:diagnostic imaging / Q000503:physiopathology; D011859:Radiography; D012129:Respiratory Function Tests; D013577:Syndrome
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