Lymphangioleiomyomatosis (Lymphangiomyomatosis)

A disease characterized by the progressive invasion of SMOOTH MUSCLE CELLS into the LYMPHATIC VESSELS, and the BLOOD VESSELS. The majority of the cases occur in the LUNGS of women of child-bearing age, eventually blocking the flow of air, blood, and lymph. The common symptom is shortness of breath (DYSPNEA).

Also Known As:

Networked: 1253 relevant articles (32 outcomes, 77 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Tuberous Sclerosis (Bourneville's Disease)
2.	Angiomyolipoma
3.	Pneumothorax
4.	Chylothorax
5.	Lung Diseases (Lung Disease)

Experts

1.	Moss, Joel: 101 articles (01/2022 - 03/2002)
2.	Seyama, Kuniaki: 50 articles (02/2022 - 01/2002)
3.	McCormack, Francis X: 48 articles (04/2022 - 04/2003)
4.	Henske, Elizabeth P: 43 articles (01/2022 - 12/2002)
5.	Johnson, Simon R: 34 articles (04/2022 - 04/2002)
6.	Krymskaya, Vera P: 33 articles (02/2021 - 08/2002)
7.	Taveira-DaSilva, Angelo M: 33 articles (10/2020 - 12/2003)
8.	Pacheco-Rodriguez, Gustavo: 27 articles (01/2022 - 03/2002)
9.	Steagall, Wendy K: 26 articles (01/2022 - 12/2005)
10.	Kwiatkowski, David J: 24 articles (01/2022 - 08/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Lymphangioleiomyomatosis:

1.	Sirolimus (Rapamycin)FDA Link 03/01/2016 - "The Multicenter International Lymphangioleiomyomatosis Efficacy and Safety of Sirolimus (MILES) trial demonstrated that sirolimus stabilized lung function and improved measures of functional performance and quality of life in patients with lymphangioleiomyomatosis. " 06/01/2023 - "MILES was a phase III placebo-controlled trial employing 1:1 randomization that led to US Food and Drug Administration approval of sirolimus for treating patients with lymphangioleiomyomatosis. " 02/01/2020 - "We studied 20 participants from the Multicenter Lymphangioleiomyomatosis Sirolimus Trial for Safety study, who had undergone both thin-section CT scans and pulmonary function tests at baseline, 12, and 24 months. " 11/14/2018 - "Sirolimus showed efficacy in a phase 3 trial of patients with lymphangioleiomyomatosis, but the optimal dose remains unclear. " 08/01/2013 - "In the MILES trial, patients with lymphangioleiomyomatosis who had forced expiratory volume in 1 second (FEV1) of 70% or less of predicted were randomly assigned (1:1) to 12 months masked treatment with sirolimus or placebo. "
2.	lipoarabinomannan (LAM)IBA 08/01/2023 - "The Epidemiology and Clinical Features of Lymphangioleiomyomatosis (LAM): A Descriptive Study of 33 Case Reports." 01/01/2023 - "Lymphangioleiomyomatosis (LAM) is a rare, destructive disease of the lungs with a limited number of determinants of disease activity, which are a critical need for clinical trials. " 04/27/2022 - "To date, no studies have evaluated the quality of information available on YouTube regarding lymphangioleiomyomatosis (LAM). " 01/01/2019 - "The aim of our study was to elucidate the landscapes of genetic alterations of TSC1 and TSC2 as well as other possible non-TSC1/2 in Lymphangioleiomyomatosis (LAM) patients. " 09/01/2017 - "The objective of this study was to report our experience with active surveillance of nonfatty renal masses in a large cohort of patients with lymphangioleiomyomatosis (LAM), correlate their CT features and patterns of growth with histopathology results, and provide guidelines for management. "
3.	trans-sodium crocetinate (crocetin)IBA 09/01/2014 - "Comorbidities of TSC were significantly correlated with pulmonary lymphangiomyomatosis (p < 0.001) and seizure remission (p = 0.018). " 01/01/2021 - "More than half the patients had sclerotic bone lesions (68%), TSC-associated neuropsychiatric disorders (64%) and lymphangioleiomyomatosis (59%). " 01/01/2020 - "The patient presented with solitary renal AML and pulmonary lymphangiomyomatosis and without other findings suggestive of TSC. " 01/01/2018 - "Men were under-represented because the cohort was enriched for patients with TSC with lymphangioleiomyomatosis, which occurs in adult women. " 01/01/2017 - "A retrospective analysis of patient medical records and skin photography was performed; 104 adult patients with TSC were enrolled in an observational cohort study that was enriched for those with pulmonary lymphangioleiomyomatosis, and was therefore composed mostly of women (99 women, 5 men). "
4.	MTOR InhibitorsIBA 04/01/2022 - "A survey of use of mTOR inhibitors in patients with lymphangioleiomyomatosis listed for lung transplant." 01/01/2018 - "The efficacy and adverse events of mTOR inhibitors in lymphangioleiomyomatosis: systematic review and meta-analysis." 12/01/2020 - "COVID-19 and lymphangioleiomyomatosis: Experience at a reference center and the potential impact of the use of mTOR inhibitors." 01/01/2017 - "The effect of mTOR inhibitors on respiratory infections in lymphangioleiomyomatosis." 01/01/2018 - "The main risks of angiomyolipomata, severe bleeding, loss of renal function, and pulmonary lymphangioleiomyomatosis, can be ameliorated by active surveillance and preemptive therapy with mTOR inhibitors. "
5.	TOR Serine-Threonine KinasesIBA 03/01/2021 - "The patient was diagnosed as having the sporadic form of lymphangioleiomyomatosis and was treated with the mammalian target of rapamycin pathway inhibitor sirolumus with clinical improvement." 09/01/2015 - "Lymphangioleiomyomatosis is a rare, progressive cystic lung disorder characterised by dysregulated activation of mammalian target of rapamycin (mTOR) signalling.This" 12/01/2014 - "Should mammalian target of rapamycin inhibitors be stopped in women with lymphangioleiomyomatosis awaiting lung transplantation?" 02/01/2010 - "Mammalian target of rapamycin signaling and autophagy: roles in lymphangioleiomyomatosis therapy." 11/14/2018 - "Lymphangioleiomyomatosis is a rare disease caused by unregulated activation of mammalian target of rapamycin (mTOR) signalling pathway. "
6.	EverolimusFDA Link 03/01/2017 - "Pelvic lymphangioleiomyomatosis treated successfully with everolimus: Two case reports with literature review." 09/01/2015 - " was a phase IIa, multicentre, open-label study of the mTOR inhibitor everolimus (2.5 mg·day(-1) escalated to 10 mg·day(-1)) in 24 women with lymphangioleiomyomatosis. " 09/01/2015 - "Everolimus for the treatment of lymphangioleiomyomatosis: a phase II study." 01/01/2016 - "mTOR treatment in lymphangioleiomyomatosis: the role of everolimus." 01/01/2018 - "During the period of everolimus therapy, among patients with lymphangioleiomyomatosis, the mean forced expiratory volume in 1 s (FEV1) increased by 276 ± 78 ml (P < 0.001), the forced vital capacity (FVC) increased by 433 ± 170 ml (P < 0.001), and the residual volume decreased by 408 ± 243 ml (P = 0.009), as compared with baseline values. "
7.	Biomarkers (Surrogate Marker)IBA 03/01/2016 - "Imaging Biomarkers in Lymphangioleiomyomatosis Clinical Trials. " 01/01/2023 - "Lymphangioleiomyomatosis: Searching for potential biomarkers." 01/01/2018 - "Correction: Lymphangioleiomyomatosis Biomarkers Linked to Lung Metastatic Potential and Cell Stemness." 01/01/2018 - "Emerging biomarkers of lymphangioleiomyomatosis." 01/01/2015 - "Lymphangioleiomyomatosis Biomarkers Linked to Lung Metastatic Potential and Cell Stemness."
8.	Vascular Endothelial Growth Factor DIBA 01/01/2022 - "Something not so new for lymphangioleiomyomatosis: is VEGF-D a glass half empty or half full?" 01/01/2022 - "Diagnostic performance of VEGF-D for lymphangioleiomyomatosis: a meta-analysis." 01/01/2017 - "In clinical isolates of PBMCs from lymphangioleiomyomatosis patients, VEGF-D expression was elevated. " 01/01/2017 - "However, there are gaps in knowledge regarding VEGF-D dysregulation and its cellular origin in lymphangioleiomyomatosis. " 09/01/2016 - "Diagnostic and Treatment Monitoring Potential of Serum Vascular Endothelial Growth Factor-D in Lymphangioleiomyomatosis."
9.	70-kDa Ribosomal Protein S6 Kinases (Ribosomal Protein S6 Kinases, 70 kDa)IBA 06/01/2006 - "This study was undertaken to determine whether the mTOR/p70 S6 kinase signaling pathway is activated in lymphangioleiomyomatosis-associated angiomyolipomas lacking TSC1/TSC2 loss of heterozygosity. " 06/01/2006 - "These data suggest that activation of the Rheb/mTOR/p70 S6 kinase pathway is related to the pathogenesis of lymphangioleiomyomatosis-associated and sporadic angiomyolipomas lacking TSC1/TSC2 loss of heterozygosity. " 06/01/2006 - "Moderate or strong phospho-ribosomal protein S6 immunoreactivity was found in all lymphangioleiomyomatosis-associated and sporadic angiomyolipomas, suggesting a high incidence of mTOR/p70 S6 kinase signaling pathway activation despite a lack of TSC1/TSC2 loss of heterozygosity. " 02/01/2009 - "The pathogenesis of lymphangioleiomyomatosis is determined by mutations affecting tuberous sclerosis complex (TSC) genes, with eventual deregulation of the Rheb/mTOR/p70S6K pathway, and the potential therapeutic activity of mTOR inhibitors is currently under investigation. " 06/01/2006 - "TSC2 mutations and loss of heterozygosity have been identified in sporadic lymphangioleiomyomatosis-associated angiomyolipomas, thus implicating the TSC/Ras homolog-enriched in brain (Rheb)/mammalian target of Rapamycin (mTOR)/p70 S6 kinase signaling pathway in their pathogenesis. "
10.	ResveratrolIBA 01/01/2023 - "Larger controlled trials of this combination might be warranted to assess definitively the usefulness of resveratrol as an additive therapy in lymphangioleiomyomatosis. " 01/01/2023 - "The addition of resveratrol was safe and well tolerated in patients with lymphangioleiomyomatosis taking sirolimus and was associated with modest improvement in HRQOL. " 01/01/2023 - "Safety and Efficacy of Combined Resveratrol and Sirolimus in Lymphangioleiomyomatosis." 01/01/2023 - "We conducted a phase 2, dose-escalating, open-label trial of resveratrol in patients with lymphangioleiomyomatosis receiving a stable regimen of sirolimus. " 01/01/2023 - "Is the addition of resveratrol safe and more efficacious than sirolimus alone in patients with lymphangioleiomyomatosis? "

Therapies and Procedures

1.	Therapeutics 01/01/2017 - "Management of lymphangioleiomyomatosis has substantially improved in the past decade with the understanding of its pathogenic mechanisms, the discovery of an effective therapy, and development of large cohorts and international guidelines. " 01/01/2023 - "A critical need exists to develop remission-inducing therapies for lymphangioleiomyomatosis. " 09/01/2011 - "New targeted therapies for lymphangioleiomyomatosis based on molecular and biologic properties of the disease are currently undergoing clinical trials. " 01/01/2020 - ""T"eeing Up A Novel Therapy for Lymphangioleiomyomatosis." 01/01/2018 - "Rates of change in FEV1 and DLCO as potential indicators for mTOR inhibitor therapy in premenopausal lymphangioleiomyomatosis patients."
2.	Lung Transplantation 01/01/2019 - "The purpose of the present study was to evaluate the lung volume after single lung transplantation for lymphangioleiomyomatosis by three-dimensional computed tomography volumetry and investigate the correlation between the native lung volume change and postoperative pulmonary function. " 01/01/2019 - "Whereas native lung overinflation has been thought to happen in recipients of single lung transplantation for lymphangioleiomyomatosis because of its increased compliance, there is no study that has reported the details on the change of the native lung volume after single lung transplantation by three-dimensional computed tomography volumetry. " 10/19/2013 - "[Lung transplantation in sporadic lymphangioleiomyomatosis: study of 7 cases]." 07/01/2023 - "A 55-year-old woman showed progressive renal dysfunction after unilateral deceased-donor lung transplantation for lymphangioleiomyomatosis. " 07/01/2023 - "Kidney Podocyte Zebra Bodies after Lung Transplantation for Lymphangioleiomyomatosis."
3.	Ovariectomy (Oophorectomy) 04/01/1995 - "A 41-year-old woman with pulmonary lymphangiomyomatosis had been scheduled for bilateral oophorectomy which led to amelioration of the pulmonary pathology. " 03/01/1995 - "[Subarachnoid block for ovariectomy in a patient with pulmonary lymphangioleiomyomatosis]." 09/01/1987 - "Anaesthetic management for oophorectomy in pulmonary lymphangiomyomatosis." 01/01/1985 - "We report a patient with pulmonary lymphangioleiomyomatosis successfully treated by pleurectomy and oophorectomy." 01/01/1985 - "Oophorectomy for pulmonary lymphangioleiomyomatosis: a case report."
4.	Pleurodesis 10/13/2021 - "Left uniportal VATS for lung decortication after chemical pleurodesis under spontaneous breathing in patient with lymphangiomyomatosis." 04/01/2006 - "CT of pleural abnormalities in lymphangioleiomyomatosis and comparison of pleural findings after different types of pleurodesis." 04/01/2002 - "Lymphangioleiomyomatosis: correlation of qualitative and quantitative thin-section CT with pulmonary function tests and assessment of dependence on pleurodesis." 10/01/1999 - "In the other two patients, one with lymphangioleiomyomatosis and the other with nonchylous pleural fluid, continued effusion was successfully treated by means of pleurodesis. " 07/01/1987 - "Open lung biopsy confirmed the clinical impression of pulmonary lymphangiomyomatosis, and a pleurodesis was performed. "
5.	Aftercare (After-Treatment) 01/10/2008 - "Some patients with lymphangioleiomyomatosis had improvement in spirometric measurements and gas trapping that persisted after treatment. " 05/01/2011 - "Evolution of pulmonary function after treatment with goserelin in patients with lymphangioleiomyomatosis." 12/01/2012 - "Reduction in size of renal angiomyolipoma after treatment with everolimus in lung transplantation due to lymphangioleiomyomatosis."