Pleural fibrosis mediates shrinking lungs syndrome in children

Krych, Esther H.; Fischer, Philip R.; Wylam, Mark E.

doi:10.1002/ppul.20946

Cited by 13 publications

(11 citation statements)

References 22 publications

(21 reference statements)

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“…To our knowledge, to date only six cases of SLS in children have been described in the English‐language literature . More recent publications describe children in an age‐range similar to that of our patient: a 14‐year‐old boy and a 12‐year‐old girl …”

Section: Discussionsupporting

confidence: 68%

“…Even if the long-term prognosis of SLS in SLE seems to be favorable 6,15 all pediatric patients who have been reported upon to date 3,4 continued to show more profound lung function impairment than that of our patient. Apart from considerable weight gain due to corticosteroid therapy, our patient has not complained of any limitation in daily life, including physical activity.…”

Section: Discussionmentioning

confidence: 58%

“…In the CT scan of her thorax, inflammatory residues were clearly detected in both lower lung fields. Hence, a potential and comparable pathogenetic sequence could be conceived as follows: after an infection triggers an ongoing immune process in the area of the lower lungs, consecutive scarring at the zone of apposition between the parietal and diaphragmatic pleura develops, leading to subsequent impairment of the diaphragmatic motion.…”

Section: Discussionmentioning

confidence: 99%

“…2 More recent publications describe children in an age-range similar to that of our patient: a 14-yearold boy 3 and a 12-year-old girl. 4 Different hypothetical pathomechanisms of SLS involve a diaphragm dysfunction as a consequence of pleural inflammation or pleural fibrosis 5 or a respiratory myopathy including inspiratory and expiratory muscles. 6 Others postulate that an abnormal chest wall expansion 2 possibly due to pleural thickening 7 or fibrotic changes 8 is the likely etiology for the restrictive pattern.…”

Section: Discussionmentioning

confidence: 99%

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Symptoms of shrinking lung syndrome reveal systemic lupus erythematosus in a 12-year-old girl

et al. 2012

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While pleuropulmonary involvement in systemic lupus erythematosus (SLE) is a common occurrence, shrinking lung syndrome (SLS) is a rare complication of SLE, particularly in children. We report on a teenager girl with a primary SLE diagnosis, which was based upon clinical, imaging, lung-function and histological findings ascertained to be compatible with SLS. Following a pneumonia, the patient developed inflammatory residues in the lower lobes, an event that probably caused diaphragmatic immobility and subsequently led to SLS. Treatment response to steroids, cyclophosphamide and hydroxychloroquine in this case was excellent, and efficacy was more profound than previously has been reported in the literature with respect to pediatric patients. This case report argues that prognosis of SLS in SLE is likely to be favorable when the diagnosis is made early and the disease is treated appropriately.

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Section: Discussionsupporting

confidence: 68%

Section: Discussionmentioning

confidence: 58%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Symptoms of shrinking lung syndrome reveal systemic lupus erythematosus in a 12-year-old girl

et al. 2012

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“…Because pathologic data are unavailable, theories of its etiology are speculative. In the past, it has been attributed successively to a surfactant deficiency, [15,43] isolated diaphragmatic myopathy, [34,39,44,53,65,67] phrenic nerve dysfunction, [45,66] chest wall dysfunction, [19,71] and pleural adhesions, [31,39] but none of these hypotheses has been unequivocally confirmed.…”

Section: Discussionmentioning

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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