Shrinking lungs syndrome, a rare manifestation of systemic lupus erythematosus

Alberto, Chloé; Diana, D.; Guillermo, N.

doi:10.1111/j.1742-1241.2006.00683.x

Cited by 15 publications

(1 citation statement)

References 12 publications

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“…Several reports describe the efficacy of variable doses of corticosteroids, either alone or combined with azathioprine, cyclophosphamide or methotrexate [1,2,7,10,13,17]. Anecdotic reports suggest a modest benefit of theophylline and β-agonists [7].…”

Section: Discussionmentioning

confidence: 99%

Shrinking Lung Syndrome Successfully Treated with Rituximab and Cyclophosphamide

Langenskiöld¹,

Bonetti²,

Jw³

et al. 2012

Respiration

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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.

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Section: Discussionmentioning

confidence: 99%

Shrinking Lung Syndrome Successfully Treated with Rituximab and Cyclophosphamide

Langenskiöld¹,

Bonetti²,

Jw³

et al. 2012

Respiration

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Presentation and prognosis of shrinking lung syndrome in systemic lupus erythematosus: report of four cases

Calderaro

Ferreira

2011

Rheumatol Int

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Systemic lupus erythematosus is an autoimmune systemic disease that commonly affects the respiratory system. Shrinking lung syndrome is a rare respiratory complication associated with systemic lupus erythematosus. Patients present with dyspnea alone or associated with chest pain and orthopnea, lung volume reduction with no parenchymal abnormalities and a restrictive ventilatory defect on pulmonary function tests. The pathogenesis, treatment, and prognosis of shrinking lung syndrome remain controversial. This study describes the clinical features, investigations, and outcome of a series of four patients with systemic lupus erythematosus and shrinking lung syndrome regularly followed on Rheumatology Service of the Clinics Hospital of the Federal University of Minas Gerais, Brazil, with a brief review of literature. It emphasizes that, despite prognosis of shrinking lung syndrome has been reported as good, it may cause severe functional pulmonary abnormalities and must be treated promptly and aggressively in order to, at least, stabilize pulmonary function tests.

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Reinforcing a medical hypothesis with a new question: is there a subgroup of shrinking lungs syndrome that is induced by pleurisy in systemic lupus erythematosus and is this subgroup marked by anti-Ro/SSA?

et al. 2010

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Shrinking lungs syndrome (SLS) is a rare entity associated with autoimmune diseases and its underlying pathogenesis is still unclear. We describe a series of seven consecutive cases of SLS in systemic lupus erythematosus, all of them with serositis and six (85.7%) with anti-Ro/SSA antibodies. Our findings reinforce the hypothesis that SLS may be, in some cases, a consequence of diaphragmatic restriction due to pleuritic pain, and we suggest anti-Ro/SSA as a marker of this subgroup of SLS.

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Shrinking lungs syndrome, a rare manifestation of systemic lupus erythematosus

Cited by 15 publications

References 12 publications

Shrinking Lung Syndrome Successfully Treated with Rituximab and Cyclophosphamide

Shrinking Lung Syndrome Successfully Treated with Rituximab and Cyclophosphamide

Presentation and prognosis of shrinking lung syndrome in systemic lupus erythematosus: report of four cases

Reinforcing a medical hypothesis with a new question: is there a subgroup of shrinking lungs syndrome that is induced by pleurisy in systemic lupus erythematosus and is this subgroup marked by anti-Ro/SSA?

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