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

Y Ammar; C Launois; J-M Perotin; S Dury; A Servettaz; D Perdu; H Vallerand; J Nardi; C Boulagnon-Rombi; M Pluot; F Lebargy; G Deslee

doi:10.1016/j.rmr.2016.10.875

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

Rev Mal Respir. 2017 May;34(5):571-575. doi: 10.1016/j.rmr.2016.10.875. Epub 2017 Feb 8.

[Article in French]

Authors

Y Ammar¹, C Launois¹, J-M Perotin¹, S Dury¹, A Servettaz², D Perdu¹, H Vallerand¹, J Nardi¹, C Boulagnon-Rombi³, M Pluot³, F Lebargy¹, G Deslee⁴

Affiliations

¹ Service des maladies respiratoires, unité Inserm 903, hôpital Maison-Blanche, CHU de Reims, 45, rue Cognacq-Jay, 51092 Reims cedex, France.
² Service de médecine interne, hôpital Robert-Debré, CHU de Reims, 51092 Reims, France.
³ Laboratoire d'anatomie pathologie, CHU de Reims, 51092 Reims, France.
⁴ Service des maladies respiratoires, unité Inserm 903, hôpital Maison-Blanche, CHU de Reims, 45, rue Cognacq-Jay, 51092 Reims cedex, France. Electronic address: [email protected].

PMID: 28189434
DOI: 10.1016/j.rmr.2016.10.875

Abstract

Introduction: The shrinking lung syndrome (SLS) is a rare complication of systemic lupus erythematosus.

Case report: 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; PaCO₂ 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, PaCO₂ 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.

Conclusion: 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.

Keywords: Acute respiratory failure; Alveolar hypoventilation; Diaphragm; Diaphragme; Hypoventilation alvéolaire; Insuffisance respiratoire aiguë; Lupus érythémateux disséminé; Shrinking lung syndrome; Systemic lupus erythematosus.

Publication types

Case Reports

MeSH terms

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