Shrinking lung syndrome in systemic lupus erythematosus patients; clinical characteristics, disease activity and damage

Gheita, Tamer A.; Azkalany, Ghada S.; El-Fishawy, Hussein S.; El-Din, Abeer M. Nour

doi:10.1111/j.1756-185x.2011.01651.x

Cited by 25 publications

(8 citation statements)

References 31 publications

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“…The frequency of NPSLE has been reported in many previous Egyptian studies to range from 23.3% to 35%. [4][5][6][7][8][9]. Higher frequencies were reported in a study from Zagazig (42.9%) [10] and another from Assiut (46.7%) [11].…”

Section: Introductionmentioning

confidence: 58%

Magnetic resonance imaging (MRI) brain abnormalities of neuropsychiatric systemic lupus erythematosus patients in Mansoura city: Relation to disease activity

Zaky

Shaat

El-Bassiony

et al. 2015

The Egyptian Rheumatologist

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Aim of the work: To assess brain abnormalities in neuropsychiatric systemic lupus erythematosus (NPSLE) patients using magnetic resonance imaging (MRI) and to compare the findings to those in SLE patients without neuropsychiatric manifestations as well as to compare disease activity between SLE patients with and without MRI brain abnormalities.Patients and methods: The study was conducted on 55 SLE patients (48 female/7 male) with a mean age of 27.3 ± 4.6 years and disease duration of 4 ± 1.6 years. Patients with NPSLE (n = 30) and without neuropsychiatric (no-NPSLE) (n = 25) underwent MRI brain. The SLE disease activity index (SLEDAI), headache disability index and mini mental state score were considered.Results: Brain abnormalities were detected in 21 SLE patients (38.2%); 16 NPSLE and 5 no-NPSLE. The brain abnormalities included a significantly higher frequency of white matter changes in NPSLE (14 patients; 46%) compared to no-NPSLE patients (5 patients; 20%) (p = 0.038). In NPSLE patients, ischemia was present in 5 patients (16.7%), hemorrhage in 3 (10%) and encephalopathy in another 3 (10%). The SLEDAI was significantly higher in NPSLE patients with cognitive impairment(33.6 ± 9.1) and NPSLE patients with headache (24.2 ± 5.6) compared to the score in no-NPSLE patients (7.2 ± 4.7) (p = 0.001). Patients with MRI brain abnormalities had significantly longer disease duration (p = 0.03), higher SLEDAI (p = 0.001), mini mental state score (p = 0.005) and headache score (p = 0.019) than those without. The study revealed an MRI sensitivity of 0.64.

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Section: Introductionmentioning

confidence: 58%

Magnetic resonance imaging (MRI) brain abnormalities of neuropsychiatric systemic lupus erythematosus patients in Mansoura city: Relation to disease activity

Zaky

Shaat

El-Bassiony

et al. 2015

The Egyptian Rheumatologist

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“…[1–4] Finally, we also excluded the cases reported by Allen et al [38] (11 cases), Gibson et al [39] (7 cases), Gheita et al [40] (8 cases), Elkayam et al [41] (2 cases), and 1 case published by Rubin and Urowitz [42] because their clinical characteristics were not sufficiently detailed to be individually analyzed.…”

Section: Patient and Methodsmentioning

confidence: 99%

Shrinking lung syndrome in systemic lupus erythematosus

et al. 2016

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Shrinking lung syndrome (SLS) is a rare and less known complication mainly associated with systemic lupus erythematosus (SLE). In this study, we analyze the clinical features, investigation findings, approaches to management, and outcome in a case series of 9 adult patients with SLE and SLS diagnosed during a 35-year period in 3 referral tertiary care hospitals in Spain. Additionally, we reviewed 80 additional cases previously reported (PubMed 1965–2015). These 80 cases, together with our 9 patients, form the basis of the present analysis.The overall SLS prevalence in our SLE population was 1.1% (9/829). SLS may complicate SLE at any time over its course, and it usually occurs in patients without previous or concomitant major organ involvement. More than half of the patients had inactive lupus according to SELENA-systemic lupus erythematosus disease activity index (SLEDAI) scores. Typically, it presents with progressive exertional dyspnea of variable severity, accompanied by pleuritic chest pain in 76% of the cases.An important diagnostic delay is common. The diagnostic tools that showed better yield for SLS detection are the imaging techniques (chest x-ray and high-resolution computed tomography) along with pulmonary and diaphragmatic function tests. Evaluation of diaphragm dome motion by M-mode ultrasonography and phrenic nerve conduction studies are less useful.There are no standardized guidelines for the treatment of SLS in SLE. The majority of patients were treated with medium or high doses of glucocorticoids. Several immunosuppressive agents have been used in conjunction with steroids either if the patient fails to improve or since the beginning of the treatment. Theophylline and beta-agonists, alone or in combination with glucocorticoids, have been suggested with the intent to increase diaphragmatic strength.The overall long-term prognosis was good. The great majority of patients had significant clinical improvement and stabilization, or mild to moderate improvement on pulmonary function tests. The mortality rate was very low.

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“…Second, SLS usually presents as a late manifestation of SLE and is more common in females. 1,6 Third, the patient’s history of polysubstance abuse and significant psychosocial impairment in the setting of a clear lung exam and normal lung parenchyma on imaging further complicated the diagnosis.…”

Section: Discussionmentioning

confidence: 99%

“…Shrinking lung syndrome (SLS) is a rare complication of systemic lupus erythematosus (SLE) with an estimated prevalence of <1%. 1,2 SLS was originally described by Hoffbrand and Beck in 1965. 3 The characteristic features of SLS are unexplained dyspnea, pleuritic chest pain, and restrictive defects on pulmonary function testing in the setting of a normal lung parenchyma.…”

Section: Introductionmentioning

confidence: 99%

Shrinking lung syndrome presenting as an initial pulmonary manifestation of SLE

Pillai¹,

Mehta

Levin

et al. 2014

Lupus

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Shrinking lung syndrome in systemic lupus erythematosus patients; clinical characteristics, disease activity and damage

Abstract: Shrinking lung syndrome is not rare and presents a considerable subset of SLE patients. In SLE patients with dyspnea or chest pain, SLS should be looked for and PFTs are highly suggestive.

Cited by 25 publications

References 31 publications

Magnetic resonance imaging (MRI) brain abnormalities of neuropsychiatric systemic lupus erythematosus patients in Mansoura city: Relation to disease activity

Magnetic resonance imaging (MRI) brain abnormalities of neuropsychiatric systemic lupus erythematosus patients in Mansoura city: Relation to disease activity

Shrinking lung syndrome in systemic lupus erythematosus

Shrinking lung syndrome presenting as an initial pulmonary manifestation of SLE

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