Autopsy and Case Reports
http://autopsyandcasereports.org/journal/autopsy/article/doi/10.4322/acr.2014.041
Autopsy and Case Reports
Article / Clinical Case Report

Severe cognitive dysfunction and shrinking lung syndrome in systemic lupus erythematous

Breno José Alencar Pires Barbosa; Francisco Akira Malta Cardozo; João Francisco Figueiredo Marcondes Ferraz; Jairo Rays; Márcia Yoshie Kanegae; Vilma Takayasu

Downloads: 10
Views: 267

Abstract

Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect any organ or system. Neuropsychiatric and pulmonary involvement can occur in 40 and 50% of patients respectively, and may occur in several different clinical forms. While the main neuropsychiatric manifestations are represented by cognitive impairment, organic cerebral syndromes, delirium, psychosis, seizures, and peripheral neuropathies, the main forms of pulmonary involvement are pleurisy with or without pleural effusion, pneumonitis, interstitial disease, pulmonary hypertension, and alveolar hemorrhage. The authors report the case of a 49-year-old woman whose first manifestation of SLE was represented by two rare manifestations: rapidly progressive cognitive impairment, which was associated with respiratory failure caused by the shrinking lung syndrome. The authors call attention to the under-diagnosis of lupus pulmonary complications and its association with severe cognitive impairment that often necessitates aggressive treatment.
 

Keywords

Lupus Erythematosus, Systemic, Lung Diseases, Cognition Disorders

References

Fanouriakis A, Boumpas DT, Bertsias GK. Pathogenesis and treatment of CNS lupus. Curr Opin Rheumatol. 2013;25(5):577-83. 10.1097/BOR.0b013e328363eaf1.23836074

Mittoo S, Fell CD. Pulmonary manifestations of systemic lupus erythematosus. Semin Respir Crit Care Med. 2014;35(2):249-54. 10.1055/s-0034-1371537.24668539

Bertsias GK, Boumpas DT. Pathogenesis, diagnosis and management of neuropsychiatric SLE manifestations. Nat Rev Rheumatol. 2010;6(6):358-67. 10.1038/nrrheum.2010.62.20458332

McLaurin EY, Holliday SL, Williams P, Brey RL. Predictors of cognitive dysfunction in patients with systemic lupus erythematosus. Neurology. 2005;64(2):297-303.

Fisk JD, Eastwood B, Sherwood G, Hanly JG. Patterns of cognitive impairment in patients with systemic lupus erythematosus. Br J Rheumatol. 1993;32(6):458-62. 10.1093/rheumatology/32.6.458.8508281

Kozora E, Hanly JG, Lapteva L, Filley CM. Cognitive dysfunction in systemic lupus erythematosus: past, present, and future. Arthritis Rheum. 2008;58(11):3286-98. 10.1002/art.23991.18975345

Henderson LA, Loring SH, Gill RR, et al. Shrinking lung syndrome as a manifestation of pleuritis: a new model based on pulmonary physiological studies. J Rheumatol. 2013;40(3):273-81. 10.3899/jrheum.121048.23378468

Allen D, Fischer A, Bshouty Z, et al. Evaluating systemic lupus erythematosus patients for lung involvement. Lupus. 2012;21(12):1316-25. 10.1177/0961203312454343.22813546

Tomietto P, Annese V, D’agostini S, et al. General and specific factors associated with severity of cognitive impairment in systemic lupus erythematosus. Arthritis Rheum. 2007;57(8):1461-72. 10.1002/art.23098.18050188

Bhangle SD, Kramer N, Rosenstein ED. Corticosteroid-induced neuropsychiatric disorders: review and contrast with neuropsychiatric lupus. Rheumatol Int. 2013;33(8):1923-32. 10.1007/s00296-013-2750-z.23588411

Denburg SD, Carbotte RM, Denburg JA. Corticosteroids and neuropsychological functioning in patients with systemic lupus erythematosus. Arthritis Rheum. 1994;37(9):1311-20. 10.1002/art.1780370907.7945494

Hoffbrand BI, Beck ER. “Unexplained” dyspnoea and shrinking lungs in systemic lupus erythematosus. BMJ. 1965;1(5445):1273-7. 10.1136/bmj.1.5445.1273.14278816

Rubin LA, Urowitz MB. Shrinking lung syndrome in SLE—a clinical pathologic study. J Rheumatol. 1983;10(6):973-6.6663599.

Hardy K, Herry I, Attali V, Cadranel J, Similowski T. Bilateral phrenic paralysis in a patient with systemic lupus erythematosus. Chest. 2001;119(4):1274-7. 10.1378/chest.119.4.1274.11296200

Silberstein SL, Barland P, Grayzel AI, Koerner SK. Pulmonary dysfunction in systemic lupus erythematosus:prevalence classification and correlation with other organ involvement. J Rheumatol. 1980;7(2):187-95.7373620.

Bertoli AM, Vila LM, Apte M, et al. Systemic lupus erythematosus in a multiethnic US Cohort LUMINA XLVIII: factors predictive of pulmonary damage. Lupus. 2007;16(6):410-7. 10.1177/0961203307079042.17664231

Pillai S, Mehta J, Levin T, Muzumdar H, Nandalike K. Shrinking lung syndrome presenting as an initial pulmonary manifestation of SLE. Lupus. 2014;23(11):1201-3. 10.1177/0961203314536248.24836585

Warrington KJ, Moder KG, Brutinel WM. The shrinking lungs syndrome in systemic lupus erythematosus. Mayo Clin Proc. 2000;75(5):467-72. 10.1016/S0025-6196(11)64215-8.10807075

Costa CA, Castro DO Jr, Jezler S, Santiago M. Shrinking lung syndrome in systemic lupus erythematosus. J Bras Pneumol. 2004;30(3):261-3. 10.1590/S1806-37132004000300012.

Santis M, Martins V, Moita J. Síndroma do pulmão encolhido: Relato de um caso clínico e revisão da literatura. Rev Port Pneumol. 2010;16(4):687-91. [Portuguese.].20700566

Piccolo-Daher S, Magalhães E. Anesthesia in patient with shrinking lung syndrome: case report. Rev Bras Anestesiol. 2012;62(2):274-80. 10.1016/S0034-7094(12)70126-5.22440383

Calderaro DC, Ferreira GA. Presentation and prognosis of shrinking lung syndrome in systemic lupus erythematosus: report of four cases. Rheumatol Int. 2012;32(5):1391-6. 10.1007/s00296-011-1863-5.21431288

Toya SP, Tzelepis GE. Association of the shrinking lung syndrome in systemic lupus erythematosus with pleurisy: a systematic review. Semin Arthritis Rheum. 2009;39(1):30-7. 10.1016/j.semarthrit.2008.04.003.18585760

Langenskiöld E, Bonetti A, Fitting JW, et al. Shrinking lung syndrome successfully treated with rituximab and cyclophosphamide. Respiration. 2012;84(2):144-9. 10.1159/000334947.22301521

 

569696b964cc8b5cda711c0e autopsy Articles

Autops Case Rep

Share this page
Page Sections