PT  - JOURNAL ARTICLE
AU  - LAUREN A. HENDERSON
AU  - STEPHEN H. LORING
AU  - RITU R. GILL
AU  - KATHERINE P. LIAO
AU  - RUMEY ISHIZAWAR
AU  - SUSAN KIM
AU  - ROBIN PERLMUTTER-GOLDENSON
AU  - DEBORAH ROTHMAN
AU  - MARY BETH F. SON
AU  - MATTHEW L. STOLL
AU  - LAWRENCE S. ZEMEL
AU  - CHRISTY SANDBORG
AU  - PAUL F. DELLARIPA
AU  - PETER A. NIGROVIC
TI  - Shrinking Lung Syndrome as a Manifestation of Pleuritis: A New Model Based on Pulmonary Physiological Studies
AID  - 10.3899/jrheum.121048
DP  - 2013 Mar 01
TA  - The Journal of Rheumatology
PG  - 273--281
VI  - 40
IP  - 3
4099  - http://www.jrheum.org/content/40/3/273.short
4100  - http://www.jrheum.org/content/40/3/273.full
SO  - J Rheumatol2013 Mar 01; 40
AB  - 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. Conclusion. 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.