Systemic lupus erythematosus presenting as severe alveolar hypoventilation and the shrinking lung syndrome

• Published in: Revue des maladies respiratoires Vol. 34; no. 5; p. 571
• Main Authors: Ammar, Y, Launois, C, Perotin, J-M, Dury, S, Servettaz, A, Perdu, D, Vallerand, H, Nardi, J, Boulagnon-Rombi, C, Pluot, M, Lebargy, F, Deslee, G
• Format: Journal Article
• Language: French
• Published: France 01.05.2017
• Subjects: Aged; Diagnosis, Differential; Humans; Hypoventilation - diagnosis; Hypoventilation - etiology; Lung Diseases - complications; Lung Diseases - diagnosis; Lupus Erythematosus, Systemic - complications; Lupus Erythematosus, Systemic - diagnosis; Male; Radiography, Thoracic; Severity of Illness Index; Syndrome; Insuffisance respiratoire aiguë; Systemic lupus erythematosus; Lupus érythémateux disséminé; Shrinking lung syndrome; Diaphragme; Acute respiratory failure; Alveolar hypoventilation; Diaphragm; Hypoventilation alvéolaire
• ISSN: 1776-2588
• DOI: 10.1016/j.rmr.2016.10.875

The shrinking lung syndrome (SLS) is a rare complication of systemic lupus erythematosus. A 69-year-old man presented with exertional dyspnoea, muscle weakness, and weight loss of 15kg in 6months. Pulmonary function tests revealed a restrictive lung disorder, with a dramatic decrease in maximal inspiratory pressure (17% of theoretical value), and alveolar hypoventilation (pH 7.43; PaCO 55mmHg). A thoracic CT-scan showed bilateral diaphragmatic elevation. The creatinine phophokinase level was increased at 280U/L. Progress was marked by a rapidly increasing respiratory acidosis (pH 7.24, PaCO 109mmHg) requiring invasive ventilation. Auto-immune studies revealed positive anti-nuclear antibodies (1/800) and positive anti-native DNA antibody at 45U/L. Treatment with systemic corticosteroids led to an initial improvement but it was not possible to discontinue mechanical ventilation. The outcome was fatal. Autopsy did not reveal any other cause and a diagnosis of the SLS associated with lupus was confirmed. The interesting features of this case report consist of: 1) the presentation of the SLS as an alveolar hypoventilation with a fatal outcome, 2) the presentation of systemic lupus as SLS.