Correlation of serum vascular endothelial growth factor-D concentration with clinical presentation and course of lymphangioleiomyomatosis

Radzikowska, E; Jagus, Paulina; Sobiecka, Małgorzata; Chorostowska‐Wynimko, Joanna; Wiatr, Elżbieta; Kuś, Jan; Roszkowski‐Śliż, Kazimierz

doi:10.1016/j.rmed.2015.09.005

Cited by 25 publications

(22 citation statements)

References 23 publications

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“…The majority (~80%) of the pneumothoraces occur at rest or with minimal activity (15). Patients with larger cyst size on HRCT (>5mm) (13) and those with a history of smoking (27) are more likely to develop a pneumothorax. Pregnancy may be associated with increased risk of pneumothorax (28).…”

Section: Introductionmentioning

confidence: 99%

Spontaneous pneumothorax in diffuse cystic lung diseases

Cooley

Lee

Gupta

2017

Current Opinion in Pulmonary Medicine

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Purpose of review Diffuse cystic lung diseases (DCLDs) are a heterogeneous group of disorders with varying pathophysiologic mechanisms that are characterized by the presence of air-filled lung cysts. These cysts are prone to rupture, leading to the development of recurrent spontaneous pneumothoraces. In this article, we review the epidemiology, clinical features, and management DCLD-associated spontaneous pneumothorax, with a focus on lymphangioleiomyomatosis (LAM), Birt-Hogg-Dubé syndrome (BHD), and pulmonary Langerhans cell histiocytosis (PLCH). Recent findings DCLDs are responsible for approximately 10% of apparent primary spontaneous pneumothoraces. CT screening for DCLDs (BHD, LAM, and PLCH) following the first spontaneous pneumothorax has recently been shown to be cost-effective and can help facilitate early diagnosis of the underlying disorders. Patients with DCLD-associated spontaneous pneumothorax have a very high rate of recurrence, and thus pleurodesis should be considered following the first episode of spontaneous pneumothorax in these patients, rather than waiting for a recurrent episode. Prior pleurodesis is not a contraindication to future lung transplant. Summary Although DCLDs are uncommon, spontaneous pneumothorax is often the sentinel event that provides an opportunity for diagnosis. By understanding the burden and implications of pneumothoraces in DCLDs, clinicians can facilitate early diagnosis and appropriate management of the underlying disorders.

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Section: Introductionmentioning

confidence: 99%

Spontaneous pneumothorax in diffuse cystic lung diseases

Cooley

Lee

Gupta

2017

Current Opinion in Pulmonary Medicine

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“…Bei LAM-Patientinnen mit niedrigen VEGF-D-Spiegeln blieb die Lungenfunktion im Verlauf stabil, während Patientinnen mit VEGF-D-Spiegeln > 800 pg/ml eine kontinuierliche Verschlechterung von FVC und TLC zeigten. Bei unklaren klinischen Befunden und zur Prognoseabschätzung kann daher eine Bestimmung des VEGF-D-Spiegels zur Diagnosestellung herangezogen werden [34,35]. Von zentraler Bedeutung für die Einschätzung des Krankheitsverlaufs sind regelmäßige Untersuchungen der Lungenfunktion und in Abhängigkeit der zystischen Veränderungen der Lunge eine CT-Verlaufskontrolle [1].…”

Section: Lungeunclassified

Sporadische Lymphangioleiomyomatose (sLAM) und Tuberöse-Sklerose-Komplex (TSC) – Pulmonale Manifestationen

et al. 2016

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Lymphangioleiomyomatosis (LAM) is a rare multi-system disorder affecting predominantly women of childbearing age. The disease entity is divided in sporadic LAM (sLAM) and LAM associated with tuberous sclerosis complex (TSC). In up to 50 % of female TSC-patients pulmonary involvement (TSC-LAM) can be found, with first clinical symptoms usually starting between 25 and 30 years of age. Progressive deterioration of lung function of 3 - 11 % of diffusion capacity per year has been described, that's why all female TSC patients should be screened for LAM (pulmonary function testing, 6-minute walk test, high-resolution chest CT scan). MTOR inhibitors such as Everolimus or Sirolimus are implemented in the treatment of TSC/LAM and found to control disease burden. Screening for all organ manifestations in TSC is recommended and allows to improve prognosis and to prevent complications in TSC.

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“…In a retrospective study, serum VEGF-D levels greater than 800 pg·mL −1 were associated with a faster rate of decline in forced vital capacity (FVC) and a faster rate of increase in total lung capacity and residual volume (RV), compared to patients with serum VEGF-D levels less than 800 pg·mL −1 [33].…”

Section: Biomarkersmentioning

confidence: 99%

New insights in lymphangioleiomyomatosis and pulmonary Langerhans cell histiocytosis

2017

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Lymphangioleiomyomatosis (LAM) and pulmonary Langerhans cell histiocytosis (PLCH) are rare diseases that lead to progressive cystic destruction of the lungs. Despite their distinctive characteristics, these diseases share several features. Patients affected by LAM or PLCH have similar radiological cystic patterns, a similar age of onset, and the possibility of extrapulmonary involvement. In this review, the recent advances in the understanding of the molecular pathogenesis, as well as the current and most promising biomarkers and therapeutic approaches, are described.

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Correlation of serum vascular endothelial growth factor-D concentration with clinical presentation and course of lymphangioleiomyomatosis

Abstract: Serum VEGF-D is the useful biomarker of LAM extension, and might also prove predictive towards therapeutic decision.

Cited by 25 publications

References 23 publications

Spontaneous pneumothorax in diffuse cystic lung diseases

Spontaneous pneumothorax in diffuse cystic lung diseases

Sporadische Lymphangioleiomyomatose (sLAM) und Tuberöse-Sklerose-Komplex (TSC) – Pulmonale Manifestationen

New insights in lymphangioleiomyomatosis and pulmonary Langerhans cell histiocytosis

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