EJMCR |

Case Report

Volume: 4 | Issue: 4 | Published: May 20, 2020 | Pages: 119 - 122 | DOI: 10.24911/ejmcr/173-1564597182

Shrinking lung syndrome - a diagnostic dilemma: a case report

Authors: Xerri Thelma Dionne , Grech Paula , Vassallo Christian , Borg Andrew

Article Info

Authors

Xerri Thelma Dionne

Trainee in General Medicine, Mater Dei Hospital, Msida, Malta

Grech Paula

Trainee in General Medicine, Mater Dei Hospital, Msida, Malta

Vassallo Christian

Higher Specialist Trainee in Rheumatology, Mater Dei Hospital, Msida, Malta

Borg Andrew

Consultant Rheumatologist, Mater Dei Hospital, Msida, Malta

Publication History

Received: July 31, 2019

Revised: April 14, 2020

Accepted: May 06, 2020

Published: May 20, 2020

Abstract

Background: Systemic lupus erythematosus (SLE) is a chronic inflammatory connective tissue disorder with multi-organ involvement. A rare complication of SLE is shrinking lung syndrome (SLS), characterized by progressive exertional dyspnoea, reduced lung volumes, and diaphragmatic elevation. Case Presentation: We report a case of SLS secondary to SLE, who required an extensive work up to exclude other more common causes of lung involvement in such a condition. This case report highlights the disease burden of SLS in a once active, independent young lady requiring nocturnal non-invasive ventilation (NIV) to relieve shortness of breath in a recumbent position. This patient had the triad of hypoxia at rest, type 2 respiratory failure, and diaphragmatic palsy. Several different immunomodulators were used initially with little success and it was not until she received six cycles of intravenous cyclophosphamide, that she no longer required NIV support, displayed improvements in pulmonary function tests and diaphragmatic motility and attained a normal lifestyle. Conclusion: The use of immunomodulators appeared to be particularly effective in restoring normal functional capacity in shrinking lung syndrome. However, the pathophysiology of this condition requires further studies.

Keywords: Systemic lupus erythematosus (SLE), dyspnoea, diaphragmatic elevation, shrinking lung syndrome (SLS), immunomodulators, cyclophosphosphomide, case report, hydroxychloroquine (HCQ), mycofenolate mofetil (MMF), green

Pubmed Style

Xerri Thelma Dionne, Grech Paula, Vassallo Christian, Borg Andrew. Shrinking lung syndrome - a diagnostic dilemma: a case report. EJMCR. 2020; 20 (May 2020): 119-122. doi:10.24911/ejmcr/173-1564597182

Web Style

Xerri Thelma Dionne, Grech Paula, Vassallo Christian, Borg Andrew. Shrinking lung syndrome - a diagnostic dilemma: a case report. https://ejmcr.com/articles/1436 [Access: December 25, 2024]. doi:10.24911/ejmcr/173-1564597182

AMA (American Medical Association) Style

Vancouver/ICMJE Style

Xerri Thelma Dionne, Grech Paula, Vassallo Christian, Borg Andrew. Shrinking lung syndrome - a diagnostic dilemma: a case report. EJMCR. (2020), [cited December 25, 2024]; 20 (May 2020): 119-122. doi:10.24911/ejmcr/173-1564597182

Harvard Style

Xerri Thelma Dionne, Grech Paula, Vassallo Christian, Borg Andrew (2020) Shrinking lung syndrome - a diagnostic dilemma: a case report. EJMCR, 20 (May 2020): 119-122. doi:10.24911/ejmcr/173-1564597182

Chicago Style

Xerri Thelma Dionne, Grech Paula, Vassallo Christian, Borg Andrew. "Shrinking lung syndrome - a diagnostic dilemma: a case report." 20 (2020), 119-122. doi:10.24911/ejmcr/173-1564597182

MLA (The Modern Language Association) Style

Xerri Thelma Dionne, Grech Paula, Vassallo Christian, Borg Andrew. "Shrinking lung syndrome - a diagnostic dilemma: a case report." 20.May 2020 (2020), 119-122. Print. doi:10.24911/ejmcr/173-1564597182

APA (American Psychological Association) Style

Xerri Thelma Dionne, Grech Paula, Vassallo Christian, Borg Andrew (2020) Shrinking lung syndrome - a diagnostic dilemma: a case report. , 20 (May 2020), 119-122. doi:10.24911/ejmcr/173-1564597182