Shrinking lung syndrome successfully treated with rituximab and cyclophosphamide.
Respiration
; 84(2): 144-9, 2012.
Article
em En
| MEDLINE
| ID: mdl-22301521
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.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Testes de Função Respiratória
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Síndrome de Sjogren
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Ciclofosfamida
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Anticorpos Monoclonais Murinos
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Pneumopatias
/
Lúpus Eritematoso Sistêmico
Tipo de estudo:
Etiology_studies
Limite:
Adult
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Female
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Humans
Idioma:
En
Revista:
Respiration
Ano de publicação:
2012
Tipo de documento:
Article
País de afiliação:
Suíça