title: Shrinking lung syndrome in systemic lupus erythematosus: A case series and review of the literature.
abstract: Shrinking lung syndrome (SLS) is a rare and less known complication mainly associated with systemic lupus erythematosus (SLE). In this study, we analyze the clinical features, investigation findings, approaches to management, and outcome in a case series of 9 adult patients with SLE and SLS diagnosed during a 35-year period in 3 referral tertiary care hospitals in Spain. Additionally, we reviewed 80 additional cases previously reported (PubMed 1965-2015). These 80 cases, together with our 9 patients, form the basis of the present analysis.The overall SLS prevalence in our SLE population was 1.1% (9/829). SLS may complicate SLE at any time over its course, and it usually occurs in patients without previous or concomitant major organ involvement. More than half of the patients had inactive lupus according to SELENA-systemic lupus erythematosus disease activity index (SLEDAI) scores. Typically, it presents with progressive exertional dyspnea of variable severity, accompanied by pleuritic chest pain in 76% of the cases.An important diagnostic delay is common. The diagnostic tools that showed better yield for SLS detection are the imaging techniques (chest x-ray and high-resolution computed tomography) along with pulmonary and diaphragmatic function tests. Evaluation of diaphragm dome motion by M-mode ultrasonography and phrenic nerve conduction studies are less useful.There are no standardized guidelines for the treatment of SLS in SLE. The majority of patients were treated with medium or high doses of glucocorticoids. Several immunosuppressive agents have been used in conjunction with steroids either if the patient fails to improve or since the beginning of the treatment. Theophylline and beta-agonists, alone or in combination with glucocorticoids, have been suggested with the intent to increase diaphragmatic strength.The overall long-term prognosis was good. The great majority of patients had significant clinical improvement and stabilization, or mild to moderate improvement on pulmonary function tests. The mortality rate was very low.
authors.affiliation: Department of Rheumatology, Hospital Universitario de Bellvitge-IDIBELL, Barcelona Department of Rheumatology, Hospital Universitario Dr. Peset, Valencia Rheumatology Unit, Hospital de Sant Pau Department of Internal Medicine Department of Pneumology, Hospital Universitario de Bellvitge-IDIBELL, Barcelona, Spain.
authors.forename: Helena
authors.initials: H
authors.lastname: Borrell
authors.forename: Javier
authors.initials: J
authors.lastname: Narváez
authors.forename: Juan José
authors.initials: JJ
authors.lastname: Alegre
authors.forename: Ivan
authors.initials: I
authors.lastname: Castellví
authors.forename: Francesca
authors.initials: F
authors.lastname: Mitjavila
authors.forename: María
authors.initials: M
authors.lastname: Aparicio
authors.forename: Eulàlia
authors.initials: E
authors.lastname: Armengol
authors.forename: María
authors.initials: M
authors.lastname: Molina-Molina
authors.forename: Joan M
authors.initials: JM
authors.lastname: Nolla
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D008171:Lung Diseases / Q000209:etiology* / Q000473:pathology; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D013577:Syndrome