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: 722 relevant articles (14 outcomes, 37 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Tuberous Sclerosis (Bourneville's Disease)
2.	Angiomyolipoma
3.	Pneumothorax
4.	Cysts
5.	Astrocytoma (Pilocytic Astrocytoma)

Experts

1.	Moss, Joel: 60 articles (11/2015 - 03/2002)
2.	McCormack, Francis X: 26 articles (09/2015 - 04/2003)
3.	Seyama, Kuniaki: 25 articles (12/2015 - 01/2002)
4.	Taveira-DaSilva, Angelo M: 23 articles (10/2015 - 12/2003)
5.	Krymskaya, Vera P: 20 articles (12/2015 - 08/2002)
6.	Pacheco-Rodriguez, Gustavo: 18 articles (04/2015 - 03/2002)
7.	Johnson, Simon R: 15 articles (12/2015 - 04/2002)
8.	Steagall, Wendy K: 15 articles (04/2015 - 12/2005)
9.	Moss, J: 14 articles (03/2011 - 02/2000)
10.	Young, Lisa R: 12 articles (08/2013 - 05/2006)

Drugs and Biologics

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

1.	Sirolimus (Rapamycin)FDA Link 08/01/2013 - "Efficacy of sirolimus therapy for chylous effusions in lymphangioleiomyomatosis." 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. " 06/01/2013 - "Using data from the Multicenter International Lymphangioleiomyomatosis Efficacy and Safety of Sirolimus trial, we performed analyses to examine associations between SGRQ scores and values for four external measures (anchors). " 05/01/2006 - "Trials of rapamycin in lymphangioleiomyomatosis are currently underway and offer hope of evidence-based treatment for the disease." 11/01/2015 - "Intracrine steroid production and mammalian target of rapamycin pathways in pulmonary lymphangioleiomyomatosis."
2.	lipoarabinomannan (LAM)IBA 10/25/2001 - "To study the clinical features, treatment, and prognosis of pulmonary lymphangioleiomyomatosis (LAM). " 12/01/2015 - "Lymphangioleiomyomatosis (LAM) is a rare multisystem disease. " 11/01/2015 - "Lymphangioleiomyomatosis (LAM) is a rare, potentially fatal disease primarily affecting young women. " 11/01/2015 - "Lymphangioleiomyomatosis (LAM) is a rare neoplastic metastatic disease affecting women of childbearing age. " 10/01/2015 - "Lymphangioleiomyomatosis (LAM) is a proliferation of perivascular epithelioid cells typically affecting the lung as a low-grade, destructive and progressive disease but may also be found in lymph nodes and other organs. "
3.	Vascular Endothelial Growth Factor DIBA 11/01/2015 - "Correlation of serum vascular endothelial growth factor-D concentration with clinical presentation and course of lymphangioleiomyomatosis." 08/01/2013 - "Serum VEGF-D is a biologically plausible and useful biomarker in lymphangioleiomyomatosis that correlates with disease severity and treatment response. " 09/01/2010 - "Serum vascular endothelial growth factor-D prospectively distinguishes lymphangioleiomyomatosis from other diseases." 05/01/2009 - "Serum vascular endothelial growth factor-D levels in patients with lymphangioleiomyomatosis reflect lymphatic involvement." 01/10/2008 - "Diagnostic potential of serum VEGF-D for lymphangioleiomyomatosis."
4.	70-kDa Ribosomal Protein S6 Kinases (Ribosomal Protein S6 Kinases, 70 kDa)IBA 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 - "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. " 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. "
5.	Medroxyprogesterone Acetate (Depo-Provera)FDA LinkGeneric 01/01/1995 - "[Pulmonary lymphangiomyomatosis: a 3-year follow-up of medroxyprogesterone acetate therapy. " 06/01/1990 - "Successful treatment of pulmonary lymphangioleiomyomatosis with oophorectomy and medroxyprogesterone-acetate: report of a case and brief review of the literature." 04/01/2005 - "We report a rare case ofpost-menopausal onset pulmonary lymphangioleiomyomatosis in a 62-year old Caucasian female that was diagnosed at age 53 and responded well to intramuscular injections of Depo-Provera and bilateral oophorectomy. " 01/01/1996 - "Pulmonary lymphangioleiomyomatosis: a case report in postmenopausal woman treated with pleurodesis and progesterone (medroxyprogesterone acetate)."
6.	Povidone (Crospovidone)IBA 07/01/2008 - "Persistent chylothorax in lymphangioleiomyomatosis treated by intrapleural instillation of povidone." 09/01/2003 - "Refractory chylothorax after lung transplantation for lymphangioleiomyomatosis successfully cured with instillation of povidone."
7.	PicibanilIBA 10/01/2003 - "[High efficacy of pleurodesis using OK-432 for controlling intractable pneumothorax associated with pulmonary lymphangioleiomyomatosis]." 10/01/2003 - "We evaluated the efficacy of pleurodesis using OK-432 (Picibanil) for the treatment of pneumothorax associated with pulmonary lymphangioleiomyomatosis (LAM). "
8.	Ba 598 (flutropium bromide)IBA 11/01/1997 - "It is suggested that flutropium bromide is beneficial for symptomatic improvement in patients with lymphangioleiomyomatosis." 11/01/1997 - "[Flutropium bromide was effective in relieving symptoms of lymphangioleiomyomatosis--a case report]."
9.	everolimusFDA Link 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." 07/22/2013 - "Everolimus treatment of abdominal lymphangioleiomyoma in five women with sporadic lymphangioleiomyomatosis." 03/01/2014 - "Re: everolimus for angiomyolipoma associated with tuberous sclerosis complex or sporadic lymphangioleiomyomatosis (EXIST-2): a multicentre, randomised, double-blind, placebo-controlled trial." 08/01/2013 - "Commentary on: everolimus for angiomyolipoma associated with tuberous sclerosis complex or sporadic lymphangioleiomyomatosis (EXIST-2): a multicentre, randomised, double-blind, placebo-controlled trial."
10.	ProgesteroneFDA LinkGeneric 01/01/2015 - "A case of recurrent retroperitoneal lymphangioleiomyomatosis treated with progesterone therapy." 12/01/2004 - "Decline in lung function in patients with lymphangioleiomyomatosis treated with or without progesterone." 08/01/1999 - "Decline in lung function in lymphangioleiomyomatosis: relation to menopause and progesterone treatment." 11/01/1995 - "Response to treatment with progesterone in a patient with pulmonary lymphangioleiomyomatosis." 08/01/1991 - "Lymphangioleiomyomatosis--treatment with progesterone."

Therapies and Procedures

1.	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]." 03/01/1989 - "Anesthetic management for oophorectomy in a patient with lymphangiomyomatosis." 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."
2.	Lung Transplantation 10/19/2013 - "[Lung transplantation in sporadic lymphangioleiomyomatosis: study of 7 cases]." 10/01/2015 - "Mayo clinic experience of lung transplantation in pulmonary lymphangioleiomyomatosis." 07/01/2015 - "Lung Transplantation for Lymphangioleiomyomatosis in a Single Japanese Institute, With a Focus on Late-onset Complications." 04/01/2015 - "Genetic heterogeneity of circulating cells from patients with lymphangioleiomyomatosis with and without lung transplantation." 03/01/2015 - "This article presents the case report of a female patient with lymphangioleiomyomatosis who underwent single lung transplantation on the left side on 4. 10. "
3.	Pleurodesis 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. " 04/01/1993 - "At right and left thoracotomy, lymphangiomyomatosis of the lung was found and treated by pleurodesis. "
4.	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."
5.	Intramuscular Injections 04/01/2005 - "We report a rare case ofpost-menopausal onset pulmonary lymphangioleiomyomatosis in a 62-year old Caucasian female that was diagnosed at age 53 and responded well to intramuscular injections of Depo-Provera and bilateral oophorectomy. "