Abstract
Systemic lupus erythematosus is an autoimmune systemic disease that commonly affects the respiratory system. Shrinking lung syndrome is a rare respiratory complication associated with systemic lupus erythematosus. Patients present with dyspnea alone or associated with chest pain and orthopnea, lung volume reduction with no parenchymal abnormalities and a restrictive ventilatory defect on pulmonary function tests. The pathogenesis, treatment, and prognosis of shrinking lung syndrome remain controversial. This study describes the clinical features, investigations, and outcome of a series of four patients with systemic lupus erythematosus and shrinking lung syndrome regularly followed on Rheumatology Service of the Clinics Hospital of the Federal University of Minas Gerais, Brazil, with a brief review of literature. It emphasizes that, despite prognosis of shrinking lung syndrome has been reported as good, it may cause severe functional pulmonary abnormalities and must be treated promptly and aggressively in order to, at least, stabilize pulmonary function tests.
References
Karim MY, Miranda LC, Tench CM, Gordon PA, D’Cruz DP, Khamasthta MA, Hughes GRV (2002) Presentation and prognosis of the shrinking lung syndrome in systemic lupus erythematosus. Semin Artrhitis Rheum 31:289–298
Costa CA, Castro DO Jr, Jezler S, Santiago M (2004) Síndrome do pulmão encolhido no lúpus eritematoso sistêmico. J Bras Pneumol 30(3):260–263
Hardy K, Herry I, Attali V, Cadranel J, Similowski T (2001) Bilateral phrenic paralysis in a patient with systemic lupus erythematosus. Chest 119(4):1274–1277
Toya SP, Tzelepis GE (2009) Association of the shrinking lung syndrome in systemic lupus erythematosus with pleurisy: a systematic review. Semin Arthritis Rheum 39(1):30–37
Singh R, Huang W, Menon Y, Espinoza LR (2002) Shrinking lung syndrome in systemic lupus erythematosus and Sjögren’s syndrome. J Clin Rheumatol 8(6):340–345
Tan EM, Cohen AS, Fries JF, Masi AT, McShane DJ, Rothfield NF, Schaller JG, Talal N, Winchester RJ (1982) The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 25(11):1271–1277
Al-Raqum HA, Uppal SS, Al-Mutairy M, Kumari R (2006) Shrinking lung syndrome as a presenting manifestation of systemic lupus erythematosus in a female Kuwaiti. Clin Rheumatol 25:412–414
Laroche CM, Mulvey DA, Hawkins PN, Walport MJ, Strickland B, Moxham J et al (1989) Diaphragm strength in the shrinking lung syndrome in systemic lupus erythmatosus. Q J Med 71:429–439
Martens J, Demeds M, Vanmeenen MT, Dequeker J (1990) Respiratory muscle dysfunction in systemic lupus erythematosus. Aust N Z J Med 20:67–70
Gibson CJ, Edmonds JP, Hughes GR (1977) Diaphragm function and lung involvement in systemic lupus erythematosus. Am J Med 63:926–932
Wilcox PG, Stein HB, Clarke SD, Pare PD, Pardy RL (1998) Phrenic nerve function in patients with diaphragmatic weakness and systemic lupus erythematosus. Chest 93:352–358
Cavalasca JA, Dubinsky D, Nasswetter GG (2006) Shrinking lungs syndrome, a rare manifestation of systemic lupus erythemaosus. Int J Clin Pract 60(12):1683–1686
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Calderaro, D.C., Ferreira, G.A. Presentation and prognosis of shrinking lung syndrome in systemic lupus erythematosus: report of four cases. Rheumatol Int 32, 1391–1396 (2012). https://doi.org/10.1007/s00296-011-1863-5
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DOI: https://doi.org/10.1007/s00296-011-1863-5