title: Shrinking lung syndrome associated with systemic lupus erythematosus: A multicenter collaborative study of 15 new cases and a review of the 155 cases in the literature focusing on treatment response and long-term outcomes.
abstract: BACKGROUND
  Shrinking lung syndrome (SLS) is a rare respiratory manifestation of systemic lupus erythematosus (SLE), characterized by dyspnea, chest pain, elevated hemidiaphragm and a restrictive pattern on pulmonary function tests. Here, we report 15 new observations of SLS during SLE and provide a systematic literature review. We studied the clinical, biological, functional and morphologic characteristics, the treatments used and their efficacy.
  
  
  METHODS
  The inclusion criteria were all patients with SLE defined by the American College of Rheumatology criteria Hochberg (1997) , associated with a restrictive pattern on pulmonary function tests. The exclusion criteria were all differential diagnoses of restrictive patterns, including obesity and pulmonary fibrosis. The patients were recruited from local databases through chest physicians, rheumatologists and internists. The data for the literature review were extracted from the Medline database using "shrinking lung syndrome" and "lupus" as key words.
  
  
  RESULTS
  All 15 new cases were women with a median age at SLS onset of 27years old (range 17-67years). All of them complained of dyspnea and all but one of chest pain. The antibodies were similar to those found in SLE, although the anti-SS-A was positive in 10 of 13 cases. Thoracic imaging showed elevated hemidiaphragm (12/15) and/or basal atelectasia (8/15). All of the patients had an isolated restrictive pattern on PFT, with a median decrease >50% of lung volume. All of the patients were treated, using corticosteroids (11/15), immunosuppressive drugs (8/15), beta-mimetics (2/15), physiotherapy (3/15) and/or colchicine (1/15). Improvement was described in 9 of 12 patients and stability in 3 of 12. We extracted 155 cases of SLE-associated SLS from the Medline database. The clinical, biological and functional parameters were similar to our cases. Clinical improvement was described in 48 of 52 cases (94%) and PFT improvement in 36 of 47 cases. Worsening occurred in 4 cases.
  
  
  CONCLUSIONS
  SLS is a rare SLE manifestation. Pain and parietal inflammation seem to play important pathogenic roles. Steroids and antalgics are the most commonly used therapies with good responses. There is no proof of efficacy with immunosuppressive drugs for this entity. Rituximab can be discussed after failure of corticosteroids, as well as antalgics, theophylline and beta-mimetics.
authors.affiliation: AP-HP, Service de Médecine Interne 2, Centre National de Référence du Lupus Systémique, Syndrome des Anticorps Anti-phospholipides et Maladies Auto-immunes Systémiques Rares, Groupe Hospitalier Pitié Salpêtrière, Paris 75013, France.
authors.forename: Loïc
authors.initials: L
authors.lastname: Duron
authors.affiliation: AP-HP, Service de Médecine Interne 2, Centre National de Référence du Lupus Systémique, Syndrome des Anticorps Anti-phospholipides et Maladies Auto-immunes Systémiques Rares, Groupe Hospitalier Pitié Salpêtrière, Paris 75013, France; Université Paris VI Pierre et Marie Curie, Sorbonnes Universités, Paris 75013, France. Electronic address: fleur.cohen@psl.aphp.fr.
authors.forename: Fleur
authors.initials: F
authors.lastname: Cohen-Aubart
authors.affiliation: Service de Médecine Interne B, Pôle Médecine Interne et Gériatrique, Pneumologie, CHRU de Tours-Hôpital Bretonneau, 37044 Tours Cedex 9, France.
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Diot
authors.affiliation: Service de Pneumologie A, Groupe Hospitalier Bichat-Claude Bernard, APHP, Paris, France.
authors.forename: Raphaël
authors.initials: R
authors.lastname: Borie
authors.affiliation: Service de Médecine Interne, Hôpital Avicenne, APHP, 93000 Bobigny, France.
authors.forename: Sébastien
authors.initials: S
authors.lastname: Abad
authors.affiliation: Département de Rhumatologie, Hôpital Pellegrin, CHU de Bordeaux, Place Amélie-Raba-Léon, 33076 Bordeaux, France.
authors.forename: Christophe
authors.initials: C
authors.lastname: Richez
authors.affiliation: Service de Rhumatologie, CHU-Hôpitaux de Rouen, Inserm U905, CRB CIC 1404, IRIB, Université de Rouen, 76031 Rouen Cedex, France.
authors.forename: Christopher
authors.initials: C
authors.lastname: Banse
authors.affiliation: Service de Rhumatologie, CHU-Hôpitaux de Rouen, Inserm U905, CRB CIC 1404, IRIB, Université de Rouen, 76031 Rouen Cedex, France.
authors.forename: Olivier
authors.initials: O
authors.lastname: Vittecoq
authors.affiliation: AP-HP, Service de Médecine Interne 1, Groupe Hospitalier Pitié-Salpêtrière, Paris, France.
authors.forename: David
authors.initials: D
authors.lastname: Saadoun
authors.affiliation: AP-HP, Service de Médecine Interne 2, Centre National de Référence du Lupus Systémique, Syndrome des Anticorps Anti-phospholipides et Maladies Auto-immunes Systémiques Rares, Groupe Hospitalier Pitié Salpêtrière, Paris 75013, France; Université Paris VI Pierre et Marie Curie, Sorbonnes Universités, Paris 75013, France.
authors.forename: Julien
authors.initials: J
authors.lastname: Haroche
authors.affiliation: AP-HP, Service de Médecine Interne 2, Centre National de Référence du Lupus Systémique, Syndrome des Anticorps Anti-phospholipides et Maladies Auto-immunes Systémiques Rares, Groupe Hospitalier Pitié Salpêtrière, Paris 75013, France; Université Paris VI Pierre et Marie Curie, Sorbonnes Universités, Paris 75013, France.
authors.forename: Zahir
authors.initials: Z
authors.lastname: Amoura
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008171:Lung Diseases / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000188:drug therapy; D008875:Middle Aged; D015337:Multicenter Studies as Topic; D012189:Retrospective Studies; D013577:Syndrome; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D007166:Immunosuppressive Agents
keywords: Corticosteroids; Immunosuppressive drugs; Outcomes; Shrinking lung syndrome; Systemic lupus erythematosus