American Journal of Medical Case Reports
ISSN (Print): 2374-2151 ISSN (Online): 2374-216X Website: http://www.sciepub.com/journal/ajmcr Editor-in-chief: Samy, I. McFarlane
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American Journal of Medical Case Reports. 2015, 3(3), 66-68
DOI: 10.12691/ajmcr-3-3-4
Open AccessCase Report

Shrinking Lung Syndrome in Systemic Lupus Erythematosus Department of Internal Medicine. La Rabta.University Hospital

Monia SMITI KHANFIR1, Nabil BEL FEKI1, , Fatma SAID1, Amira HAMZAOUI1, Thouraya BEN SALEM1, Imed BEN GHORBEL1, Mounir LAMLOUM1 and Mohamed Habib Houman1

1Department of Internal Medicine, University Hospital of la Rabta, Tunis, Tunisia

Pub. Date: January 29, 2015

Cite this paper:
Monia SMITI KHANFIR, Nabil BEL FEKI, Fatma SAID, Amira HAMZAOUI, Thouraya BEN SALEM, Imed BEN GHORBEL, Mounir LAMLOUM and Mohamed Habib Houman. Shrinking Lung Syndrome in Systemic Lupus Erythematosus Department of Internal Medicine. La Rabta.University Hospital. American Journal of Medical Case Reports. 2015; 3(3):66-68. doi: 10.12691/ajmcr-3-3-4

Abstract

In systemic lupus erythematosus (SLE), the respiratory system is frequently compromised. One of its uncommon manifestations is the shrinking lung syndrome (SLS), characterized by unexplained dyspnea, a restrictive pattern on pulmonary function tests, and an elevated hemidiaphragm. The pathogenesis of the SLS remains unknown. We report three consecutive cases of women with systemic lupus erythematosus, aged 32, 33, and 35 years. Clinical features included dyspnea and chest pain. Onset of the SLS manifestations occurred in the absence of a SLE flare, in patients treated with low dose of corticosteroids. Diagnosis was based on clinical and laboratory patterns, particularly chest RMI showing basilar atelectasis, elevated diaphragms, and small lung volumes in two cases. Corticosteroid treatment induced improvement in the three cases and no relapse was noted. Clinical, laboratory, physiopathological, and therapeutic aspects of this syndrome are reviewed, particularly RMI findings, never discussed in previous studies.

Keywords:
systemic lupus erythematosus shrinking lung syndrome magnetic resonance imagery

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References:

[1]  Hardy K, Herry I, Attali V, Cadranel J, Similowski T. Bilateral phrenic paralysis in patient with systemic lupus erythematous. Chest 2001; 119: 1274-7.
 
[2]  Toya SP, Tzelepis GE. Association of the shrinking lung syndrome in systemic lupus erythematosus with pleurisy: a systematic review. Semin Arthritis Rheum 2009; 39: 30-7.
 
[3]  Peñacoba Toribio P, Córica Albani ME, Mayos Pérez M, Rodriguez de la Serna A. Rituximab in the treatment of shrinking lung syndrome in systemic lupus erythematosus. Reumatol Clin 2014; 10: 325-7.
 
[4]  Gheita TA, Azkalany GS, El-Fishawy HS, Nour Eldin AM. Shrinking lung syndrome in systemic lupus erythematosus patients; clinical characteristics, disease activity and damage. Int J Rheum Dis 2011; 14: 361-8.
 
[5]  Singh R, Huang W, Menon Y, Espinoza LR. Shrinking lung syndrome in systemic lupus erythematosus and Sjogren's syndrome. J Clin Rheumatol 2002; 8: 340-5.
 
[6]  Warrington KJ, Moder KG, Brutinel WM. The Shrinking lungs syndrome in systemic lupus erythematous. Mayo Clin Proc 2000; 75: 467-72.
 
[7]  Karim MY, Miranda LC, Tench CM, Gordon PA, D'cruz DP, Khamashta MA, et al. Presentation and prognosis of the Shrinking lung syndrome in systemic lupus erythematousus. Semin Arthritis Rheum 2002; 31: 289-98.
 
[8]  Soubrier M, Dubost JJ, Piette JC, Urosevic Z, Rami S, Oualid T, et al. Shrinking Lung syndrome in systemic lupus erythematosus. A report of three cases. Rev Rhum 1995; 62: 395-8.
 
[9]  9-Walz-Leblanc BA, Urowitz MB, Gladman DD, Hanly PJ. The Shrinking lungs syndrome in systemic lupus erythematosus- improvement with corticosteroid therapy. J Rheumatol 1992; 19: 1970-2.
 
[10]  H.A Al-Raqum, S.S Uppal, M Al-Mutairy. Shrinking lung syndrome as a presenting manifestation of systemic lupus erythematosus in a female Kuwaiti. Clin Rheumatol 2006; 25: 412-4.
 
[11]  Luo YM, Polkey MI, Johnson LC, Lyall RA, Harris ML, Green M, et al. Diaphragm EMG measured by cervical magnetic and electrical nerve stimulation. J Appl Physiol 1998; 85: 2089-99.
 
[12]  Van Veen S, Peeters AJ, Sterk PJ. The shrinking lung syndrome in SLE, treatment with theophylline. Clin Rheumatol 1993; 12: 462-5.
 
[13]  Muñoz-Rodríguez FJ, Font J, Badia JR, Miret C, Barberà JA, Cervera R, et al. Shrinking lungs syndrome in systemic lupus erythematosus: improvement with inhaled beta-agonisttherapy. Lupus 1997; 6: 412-4.
 
[14]  Benham H, Garske L, Vecchio P, Eckert BW. Successful treatment of shrinking lung syndrome with rituximab in a patient with systemic lupus erythematosus. J Clin Rheumatol 2010; 16: 68-70.