Shrinking lung syndrome in patients without systemic lupus erythematosus: Comment on the concise communication by Tavoni et al

Ahmed, Shahbaz; Herrick, Ariane L.; O’Driscoll, B R

doi:10.1002/1529-0131(200101)44:1<243::aid-anr36>3.0.co;2-l

Cited by 21 publications

(7 citation statements)

References 3 publications

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“…While SLS has mainly been described in SLE, it has also been reported in association with other connective tissue diseases such as Sjo¨gren's syndrome, scleroderma and rheumatoid arthritis. [14][15][16][17] Its prevalence in lupus patients ranges from 0.5% to 1.1%, 18,19 lower than our estimation of 1.53%. The reasons behind this discrepancy are not clear; however, it is possible that SLS is misdiagnosed in SLE.…”

Section: Discussioncontrasting

confidence: 62%

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Shrinking lung syndrome in systemic lupus erythematosus: a single-centre experience

Deeb

Tselios

Gladman

et al. 2017

Lupus

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Introduction Shrinking lung syndrome (SLS) is a rare manifestation of systemic lupus erythematosus (SLE), characterized by decreased lung volumes and extra-pulmonary restriction. The aim of this study was to describe the characteristics of SLS in our lupus cohort with emphasis on prevalence, presentation, treatment and outcomes. Patients and methods Patients attending the Toronto Lupus Clinic since 1980 ( n = 1439) and who had pulmonary function tests (PFTs) performed during follow-up were enrolled ( n = 278). PFT records were reviewed to characterize the pattern of pulmonary disease. SLS definition was based on a restrictive ventilatory defect with normal or slightly reduced corrected diffusing lung capacity for carbon monoxide (DLCO) in the presence of suggestive clinical (dyspnea, chest pain) and radiological (elevated diaphragm) manifestations. Data on clinical symptoms, functional abnormalities, imaging, treatment and outcomes were extracted in a dedicated data retrieval form. Results Twenty-two patients (20 females) were identified with SLS for a prevalence of 1.53%. Their mean age was 29.5 ± 13.3 years at SLE and 35.7 ± 14.6 years at SLS diagnosis. Main clinical manifestations included dyspnea (21/22, 95.5%) and pleuritic chest pain (20/22, 90.9%). PFTs were available in 20 patients; 16 (80%) had decreased maximal inspiratory (MIP) and/or expiratory pressure (MEP). Elevated hemidiaphragm was demonstrated in 12 patients (60%). Treatment with prednisone and/or immunosuppressives led to clinical improvement in 19/20 cases (95%), while spirometrical improvement was observed in 14/16 patients and was mostly partial. Conclusions SLS prevalence in SLE was 1.53%. Treatment with glucocorticosteroids and immunosuppressives was generally effective. However, a chronic restrictive ventilatory defect usually persisted.

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Section: Discussioncontrasting

confidence: 62%

“…While SLS has mainly been described in SLE, it has also been reported in association with other connective tissue diseases such as Sjögren’s syndrome, scleroderma and rheumatoid arthritis. 14–17…”

Section: Discussionmentioning

confidence: 99%

Shrinking lung syndrome in systemic lupus erythematosus: a single-centre experience

Deeb

Tselios

Gladman

et al. 2017

Lupus

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“…16 Rarely, patients with SLE, as well as other autoimmune diseases, 15,[17][18][19] may develop severe restriction that is not due to interstitial pulmonary fibrosis. 20 …”

Section: Discussionmentioning

confidence: 99%

Pleural fibrosis mediates shrinking lungs syndrome in children

Krych

Fischer

Wylam

2008

Pediatric Pulmonology

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Summary. The etiology of restrictive lung physiology in SLS is not well-defined, and has been hypothesized to be due to defects in lung recoil, phrenic nerve function and diaphragmatic strength. We present a case of SLS in an adolescent in whom imaging and electrophysiology studies demonstrate pleural fibrosis as the fundamental defect accounting for the restrictive lung physiology. Pediatr Pulmonol. 2009; 44:90-92. ß

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“…. The syndrome has been reported in other rheumatologic disorders including scleroderma, Sjögren's syndrome, rheumatoid arthritis, and undifferentiated connective tissue disorder [5] [6] [7] [8]. The pathogenesis of SLS remains unknown.…”

Section: Introductionmentioning

confidence: 99%

Shrinking Lung Detected on Computed Tomography: Pictorial Essay of the Main Findings of the Image

Neto¹,

Lyra²,

Mello³

et al. 2018

OJMI

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Shrinking lung refers to a rare complication of systemic lupus erythematosus and is characterized by unexplained dyspnea, a restrictive pattern in lung function tests, and elevation of the diaphragmatic hemicuples. It is postulated to have a predilection for female involvement and occurs mainly during late stages of the disease. Chest X-rays usually show small, diaphragmatic lungs. Occasional basal atelectasis may be present. Chest tomography usually shows reduced lung volumes with diaphragmatic elevation, occasional basal atelectasis, without severe pulmonary or pleuropulmonary disease. Shrinking lung can cause significant morbidity and occasional mortality. There is no definitive therapy, while corticosteroids may decrease symptoms and improve lung function in some patients. The objective of this study was to describe the main imaging findings in Shrinking Lung, an important pulmonary alteration in lupus patients. We highlight the characteristics observed on radiography and computed tomography, with an emphasis on computed tomography. It is important that every radiologist is prepared to recognize these findings and understand the possible clinical repercussions.

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Shrinking lung syndrome in patients without systemic lupus erythematosus: Comment on the concise communication by Tavoni et al

Cited by 21 publications

References 3 publications

Shrinking lung syndrome in systemic lupus erythematosus: a single-centre experience

Shrinking lung syndrome in systemic lupus erythematosus: a single-centre experience

Pleural fibrosis mediates shrinking lungs syndrome in children

Shrinking Lung Detected on Computed Tomography: Pictorial Essay of the Main Findings of the Image

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