EJMCR. 2020; 4(4): 119-122

Shrinking lung syndrome - a diagnostic dilemma: a case report

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

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)


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Shrinking lung syndrome - a diagnostic dilemma: a case report


Authors
Xerri Thelma Dionne
Trainee in General Medicine, Mater Dei Hospital, Msida, Malta
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Grech Paula
Trainee in General Medicine, Mater Dei Hospital, Msida, Malta
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Vassallo Christian
Higher Specialist Trainee in Rheumatology, Mater Dei Hospital, Msida, Malta
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Borg Andrew
Consultant Rheumatologist, Mater Dei Hospital, Msida, Malta
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Correspondence to:
. Thelma Dionne Xerri, Trainee in general medicine, Mater Dei Hospital, Malta.; thelma-dionne.xerri@gov.mt

Publication history
Received 31 Jul 2019
Revised 14 Apr 2020
Accepted 06 May 2020
Published online 20 May 2020
Published in print 24 May 2020

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Pubmed Style

Dionne XT, Paula G, Christian V, Andrew B. Shrinking lung syndrome - a diagnostic dilemma: a case report. EJMCR. 2020; 4(4): 119-122. doi:10.24911/ejmcr/173-1564597182


Web Style

Dionne XT, Paula G, Christian V, Andrew B. Shrinking lung syndrome - a diagnostic dilemma: a case report. https://www.ejmcr.com/?mno=59456 [Access: July 11, 2020]. doi:10.24911/ejmcr/173-1564597182


AMA (American Medical Association) Style

Dionne XT, Paula G, Christian V, Andrew B. Shrinking lung syndrome - a diagnostic dilemma: a case report. EJMCR. 2020; 4(4): 119-122. doi:10.24911/ejmcr/173-1564597182


Vancouver/ICMJE Style

Dionne XT, Paula G, Christian V, Andrew B. Shrinking lung syndrome - a diagnostic dilemma: a case report. EJMCR. (2020), [cited July 11, 2020]; 4(4): 119-122. doi:10.24911/ejmcr/173-1564597182


Harvard Style

Dionne, X. T., Paula, . G., Christian, . V. & Andrew, . B. (2020) Shrinking lung syndrome - a diagnostic dilemma: a case report. EJMCR, 4 (4), 119-122. doi:10.24911/ejmcr/173-1564597182


Turabian Style

Dionne, Xerri Thelma, Grech Paula, Vassallo Christian, and Borg Andrew. 2020. Shrinking lung syndrome - a diagnostic dilemma: a case report. European Journal of Medical Case Reports, 4 (4), 119-122. doi:10.24911/ejmcr/173-1564597182


Chicago Style

Dionne, Xerri Thelma, Grech Paula, Vassallo Christian, and Borg Andrew. "Shrinking lung syndrome - a diagnostic dilemma: a case report." European Journal of Medical Case Reports 4 (2020), 119-122. doi:10.24911/ejmcr/173-1564597182


MLA (The Modern Language Association) Style

Dionne, Xerri Thelma, Grech Paula, Vassallo Christian, and Borg Andrew. "Shrinking lung syndrome - a diagnostic dilemma: a case report." European Journal of Medical Case Reports 4.4 (2020), 119-122. Print. doi:10.24911/ejmcr/173-1564597182


APA (American Psychological Association) Style

Dionne, X. T., Paula, . G., Christian, . V. & Andrew, . B. (2020) Shrinking lung syndrome - a diagnostic dilemma: a case report. European Journal of Medical Case Reports, 4 (4), 119-122. doi:10.24911/ejmcr/173-1564597182


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