Shrinking Lung Syndrome in a Young Female: A Rare Pulmonary Manifestation of Systemic Lupus Erythematosus

Abstract

Shrinking lung syndrome (SLS) is a rare pulmonary complication of autoimmune conditions. It has been sparsely described in the literature and its pathophysiology remains unclear. SLS is typically reported in patients with a history of systemic lupus erythematosus (SLE) who present with shortness of breath and chest pain associated with breathing. Chest imaging demonstrates no alveolar, interstitial, or pleural abnormalities. Pulmonary function tests (PFTs) are characterized by a restrictive pattern with reduced lung volumes. SLS is a diagnosis of exclusion and there are no validated criteria for the diagnosis. Evaluation requires extensive testing to rule out alternative causes of dyspnea and pleuritic chest pain. In this report, we present a case of SLS in a young African American woman.

Keywords: interstitial lung disease; pulmonary function tests; restrictive lung disease; shrinking lung syndrome; systemic lupus erythematosus.

Figure 1. Flow-volume loop shows a restrictive defect with reduced lung volumes, particularly TLC and VC

TLC: total lung capacity; VC: vital capacity

Figure 2. Volume-time curve showed a proportional and significant decrease in the FEV1 and FVC

FVC: forced vital capacity; FEV1: forced expiratory volume in 1 second