Shrinking lung syndrome successfully treated with rituximab and cyclophosphamide.

Détails

ID Serval
serval:BIB_2818C5D8AE61
Type
Article: article d'un périodique ou d'un magazine.
Sous-type
Etude de cas (case report): rapporte une observation et la commente brièvement.
Collection
Publications
Institution
Titre
Shrinking lung syndrome successfully treated with rituximab and cyclophosphamide.
Périodique
Respiration
Auteur⸱e⸱s
Langenskiöld E., Bonetti A., Fitting J.W., Heinzer R., Dudler J., Spertini F., Lazor R.
ISSN
1423-0356 (Electronic)
ISSN-L
0025-7931
Statut éditorial
Publié
Date de publication
2012
Volume
84
Numéro
2
Pages
144-149
Langue
anglais
Notes
Publication types: Journal ArticlePublication Status: ppublish
Résumé
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
Oui
Création de la notice
20/09/2012 18:17
Dernière modification de la notice
20/08/2019 13:07
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