Shrinking lung syndrome successfully treated with rituximab and cyclophosphamide.

Details

Serval ID
serval:BIB_2818C5D8AE61
Type
Article: article from journal or magazin.
Publication sub-type
Case report (case report): feedback on an observation with a short commentary.
Collection
Publications
Institution
Title
Shrinking lung syndrome successfully treated with rituximab and cyclophosphamide.
Journal
Respiration
Author(s)
Langenskiöld E., Bonetti A., Fitting J.W., Heinzer R., Dudler J., Spertini F., Lazor R.
ISSN
1423-0356 (Electronic)
ISSN-L
0025-7931
Publication state
Published
Issued date
2012
Volume
84
Number
2
Pages
144-149
Language
english
Notes
Publication types: Journal ArticlePublication Status: ppublish
Abstract
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.
Pubmed
Web of science
Open Access
Yes
Create date
20/09/2012 18:17
Last modification date
20/08/2019 13:07
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