Please use this identifier to cite or link to this item: http://hdl.handle.net/2445/126507
Title: Shrinking lung syndrome in systemic lupus erythematosus: A case series and review of the literature
Author: Borrell, Helena
Narváez García, Francisco Javier
Alegre-Sancho, Juan José
Castellví, Ivan
Mitjavila Villeró, Francesca
Aparicio, María
Armengol Camps, Eulàlia
Molina Molina, María
Nolla Solé, Joan Miquel
Keywords: Lupus eritematós
Malalties del pulmó
Diagnòstic per la imatge
Fisiologia patològica
Lupus erythematosus
Pulmonary diseases
Diagnostic imaging
Pathological physiology
Issue Date: Aug-2016
Publisher: Lippincott, Williams & Wilkins. Wolters Kluwer Health
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.
Note: Reproducció del document publicat a: https://doi.org/10.1097/MD.0000000000004626
It is part of: Medicine, 2016, vol. 95, num. 33, p. e4626
URI: http://hdl.handle.net/2445/126507
Related resource: https://doi.org/10.1097/MD.0000000000004626
ISSN: 0025-7974
Appears in Collections:Articles publicats en revistes (Ciències Clíniques)
Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL))

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