Shrinking lung syndrome (SLS) is an uncommon feature of systemic lupus erythematosus (SLE) characterized by dyspnea, pleuritic chest pain, diaphragmatic elevation, restrictive ventilatory defect and reduced respiratory muscle strength as measured by volitional tests. We report the case of a 28-year-old woman with overlapping features of SLE and Sjögren syndrome who developed severe SLS while receiving corticosteroids and azathioprine for severe polyarthritis. She was treated with a combination of rituximab and cyclophosphamide, which led to a dramatic improvement in her clinical condition and respiratory function tests. The increase in vital capacity was one of the highest among 35 published cases of SLS. Thus, restoring a near-normal lung function is an achievable goal in SLS, and the use of rituximab, with or without concomitant cyclophosphamide, certainly deserves further study in this setting.

Keywords:
Diaphragm, Respiratory paralysis, Systemic lupus erythematosus, Shrinking lung syndrome, Sjögren syndrome, Rituximab, Cyclophosphamide
1.
Toya SP, Tzelepis GE: Association of the shrinking lung syndrome in systemic lupus erythematosus with pleurisy: a systematic review. Semin Arthritis Rheum 2009;39:30–37.
2.
Karim MY, Miranda LC, Tench CM, Gordon PA, D’Cruz DP, Khamashta MA, Hughes GR: Presentation and prognosis of the shrinking lung syndrome in systemic lupus erythematosus. Semin Arthritis Rheum 2002;31:289–298.
3.
Pego-Reigosa JM, Medeiros DA, Isenberg DA: Respiratory manifestations of systemic lupus erythematosus: old and new concepts. Best Pract Res Clin Rheumatol 2009;23:469–480.
4.
Ahmed S, Herrick A, O’Driscoll BR: Shrinking lung syndrome in patients without systemic lupus erythematosus: comment on the concise communication by Tavoni et al. Arthritis Rheum 2001;44:243–245.
5.
Scire CA, Caporali R, Zanierato M, Mojoli F, Braschi A, Montecucco C: Shrinking lung syndrome in systemic sclerosis. Arthritis Rheum 2003;48:2999–3000.
6.
Tavoni A, Vitali C, Cirigliano G, Frigelli S, Stampacchia G, Bombardieri S: Shrinking lung in primary Sjögren’s syndrome. Arthritis Rheum 1999;42:2249–2250.
7.
Warrington KJ, Moder KG, Brutinel WM: The shrinking lungs syndrome in systemic lupus erythematosus. Mayo Clin Proc 2000;75:467–472.
8.
Gibson CJ, Edmonds JP, Hughes GR: Diaphragm function and lung involvement in systemic lupus erythematosus. Am J Med 1977;63:926–932.
9.
Hawkins P, Davison AG, Dasgupta B, Moxham J: Diaphragm strength in acute systemic lupus erythematosus in a patient with paradoxical abdominal motion and reduced lung volumes. Thorax 2001;56:329–330.
10.
Hoffbrand BI, Beck ER: ‘Unexplained’ dyspnoea and shrinking lungs in systemic lupus erythematosus. Br Med J 1965;i:1273–1277.
11.
Laroche CM, Mulvey DA, Hawkins PN, Walport MJ, Strickland B, Moxham J, Green M: Diaphragm strength in the shrinking lung syndrome of systemic lupus erythematosus. Q J Med 1989;71:429–439.
12.
Hardy K, Herry I, Attali V, Cadranel J, Similowski T: Bilateral phrenic paralysis in a patient with systemic lupus erythematosus. Chest 2001;119:1274–1277.
13.
Al-Raqum HA, Uppal SS, Al-Mutairy M, Kumari R: Shrinking lung syndrome as a presenting manifestation of systemic lupus erythematosus in a female Kuwaiti. Clin Rheumatol 2006;25:412–414.
14.
Wilcox PG, Stein HB, Clarke SD, Pare PD, Pardy RL: Phrenic nerve function in patients with diaphragmatic weakness and systemic lupus erythematosus. Chest 1988;93:352–358.
15.
Rubin LA, Urowitz MB: Shrinking lung syndrome in SLE – a clinical pathologic study. J Rheumatol 1983;10:973–976.
16.
Ernest D, Leung A: Ventilatory failure in shrinking lung syndrome is associated with reduced chest compliance. Intern Med J 2010;40:66–68.
17.
Cavallasca JA, Dubinsky D, Nasswetter GG: Shrinking lungs syndrome, a rare manifestation of systemic lupus erythematosus. Int J Clin Pract 2006;60:1683–1686.
18.
Bertsias GK, Salmon JE, Boumpas DT: Therapeutic opportunities in systemic lupus erythematosus: state of the art and prospects for the new decade. Ann Rheum Dis 2010;69:1603–1611.
19.
Meijer JM, Meiners PM, Vissink A, Spijkervet FK, Abdulahad W, Kamminga N, Brouwer E, Kallenberg CG, Bootsma H: Effectiveness of rituximab treatment in primary Sjögren’s syndrome: a randomized, double-blind, placebo-controlled trial. Arthritis Rheum 2010;62:960–968.
20.
Merrill JT, Neuwelt CM, Wallace DJ, Shanahan JC, Latinis KM, Oates JC, Utset TO, Gordon C, Isenberg DA, Hsieh HJ, Zhang D, Brunetta PG: Efficacy and safety of rituximab in moderately-to-severely active systemic lupus erythematosus: the randomized, double-blind, phase II/III systemic lupus erythematosus evaluation of rituximab trial. Arthritis Rheum 2010;62:222–233.
21.
Furie R, Looney RJ, Rovin B, Latinis KM, Appel G, Sanchez-Guerrero J, Fervenza FC: Efficacy and safety of rituximab in subjects with active proliferative lupus nephritis: results from the randomized, double-blind phase III lunar study. Arthritis Rheum 2009;60:S429.
22.
Lateef A, Petri M: Biologics in the treatment of systemic lupus erythematosus. Curr Opin Rheumatol 2010;22:504–509.
23.
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.
24.
Elkayam O, Segal R, Caspi D, Yaron M, Greif Y: Restrictive lung disease due to diaphragmatic dysfunction in systemic lupus erythematosus. Two case reports. Clin Exp Rheumatol 1992;10:267–269.
25.
Oud KT, Bresser P, ten Berge RJ, Jonkers RE: The shrinking lung syndrome in systemic lupus erythematosus: improvement with corticosteroid therapy. Lupus 2005;14:959–963.
26.
Leandro MJ, Edwards JC, Cambridge G, Ehrenstein MR, Isenberg DA: An open study of B lymphocyte depletion in systemic lupus erythematosus. Arthritis Rheum 2002;46:2673–2677.
27.
Van Veen S, Peeters AJ, Sterk PJ, Breedveld FC: The ‘shrinking lung syndrome’ in SLE, treatment with theophylline. Clin Rheumatol 1993;12:462–465.
28.
Thompson PJ, Dhillon DP, Ledingham J, Turner-Warwick M: Shrinking lungs, diaphragmatic dysfunction, and systemic lupus erythematosus. Am Rev Respir Dis 1985;132:926–928.
29.
Stevens WM, Burdon JG, Clemens LE, Webb J: The ‘shrinking lungs syndrome’ – an infrequently recognised feature of systemic lupus erythematosus. Aust NZ J Med 1990;20:67–70.
30.
Soubrier M, Dubost JJ, Piette JC, Urosevic Z, Rami S, Oualid T, Ristori JM, Bussiere JL: Shrinking lung syndrome in systemic lupus erythematosus. A report of three cases. Rev Rhum Engl Ed 1995;62:395–398.
31.
Munoz-Rodriguez FJ, Font J, Badia JR, Miret C, Barbera JA, Cervera R, Ingelmo M: Shrinking lungs syndrome in systemic lupus erythematosus: improvement with inhaled β-agonist therapy. Lupus 1997;6:412–414.
32.
Walz-Leblanc BA, Urowitz MB, Gladman DD, Hanly PJ: The ‘shrinking lungs syndrome’ in systemic lupus erythematosus – improvement with corticosteroid therapy. J Rheumatol 1992;19:1970–1972.
33.
Branger S, Schleinitz N, Gayet S, Veit V, Kaplanski G, Badier M, Magnan A, Harle JR: Le syndrome des poumons rétractés et les maladies auto-immunes. Rev Méd Interne 2004;25:83–90.
© 2012 S. Karger AG, Basel
2012
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