title: [Systemic lupus erythematosus presenting as severe alveolar hypoventilation and the shrinking lung syndrome].
abstract: BACKGROUND
  The shrinking lung syndrome (SLS) is a rare complication of systemic lupus erythematosus.
  
  
  METHODS
  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; PaCO255mmHg). 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, PaCO2109mmHg) 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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Service des maladies respiratoires, unité Inserm 903, hôpital Maison-Blanche, CHU de Reims, 45, rue Cognacq-Jay, 51092 Reims cedex, France.
authors.forename: Y
authors.initials: Y
authors.lastname: Ammar
authors.affiliation: Service des maladies respiratoires, unité Inserm 903, hôpital Maison-Blanche, CHU de Reims, 45, rue Cognacq-Jay, 51092 Reims cedex, France.
authors.forename: C
authors.initials: C
authors.lastname: Launois
authors.affiliation: Service des maladies respiratoires, unité Inserm 903, hôpital Maison-Blanche, CHU de Reims, 45, rue Cognacq-Jay, 51092 Reims cedex, France.
authors.forename: J-M
authors.initials: JM
authors.lastname: Perotin
authors.affiliation: Service des maladies respiratoires, unité Inserm 903, hôpital Maison-Blanche, CHU de Reims, 45, rue Cognacq-Jay, 51092 Reims cedex, France.
authors.forename: S
authors.initials: S
authors.lastname: Dury
authors.affiliation: Service de médecine interne, hôpital Robert-Debré, CHU de Reims, 51092 Reims, France.
authors.forename: A
authors.initials: A
authors.lastname: Servettaz
authors.affiliation: Service des maladies respiratoires, unité Inserm 903, hôpital Maison-Blanche, CHU de Reims, 45, rue Cognacq-Jay, 51092 Reims cedex, France.
authors.forename: D
authors.initials: D
authors.lastname: Perdu
authors.affiliation: Service des maladies respiratoires, unité Inserm 903, hôpital Maison-Blanche, CHU de Reims, 45, rue Cognacq-Jay, 51092 Reims cedex, France.
authors.forename: H
authors.initials: H
authors.lastname: Vallerand
authors.affiliation: Service des maladies respiratoires, unité Inserm 903, hôpital Maison-Blanche, CHU de Reims, 45, rue Cognacq-Jay, 51092 Reims cedex, France.
authors.forename: J
authors.initials: J
authors.lastname: Nardi
authors.affiliation: Laboratoire d'anatomie pathologie, CHU de Reims, 51092 Reims, France.
authors.forename: C
authors.initials: C
authors.lastname: Boulagnon-Rombi
authors.affiliation: Laboratoire d'anatomie pathologie, CHU de Reims, 51092 Reims, France.
authors.forename: M
authors.initials: M
authors.lastname: Pluot
authors.affiliation: Service des maladies respiratoires, unité Inserm 903, hôpital Maison-Blanche, CHU de Reims, 45, rue Cognacq-Jay, 51092 Reims cedex, France.
authors.forename: F
authors.initials: F
authors.lastname: Lebargy
authors.affiliation: Service des maladies respiratoires, unité Inserm 903, hôpital Maison-Blanche, CHU de Reims, 45, rue Cognacq-Jay, 51092 Reims cedex, France. Electronic address: gdeslee@chu-reims.fr.
authors.forename: G
authors.initials: G
authors.lastname: Deslee
mesh_terms: D000368:Aged; D003937:Diagnosis, Differential; D006801:Humans; D007040:Hypoventilation / Q000175:diagnosis* / Q000209:etiology*; D008171:Lung Diseases / Q000150:complications* / Q000175:diagnosis*; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000175:diagnosis*; D008297:Male; D013902:Radiography, Thoracic; D012720:Severity of Illness Index; D013577:Syndrome
keywords: Acute respiratory failure; Alveolar hypoventilation; Diaphragm; Diaphragme; Hypoventilation alvéolaire; Insuffisance respiratoire aiguë; Lupus érythémateux disséminé; Shrinking lung syndrome; Systemic lupus erythematosus