Pulmonary Artery Pressure in Lymphangioleiomyomatosis

Taveira-DaSilva, Angelo M.; Hathaway, Olanda; Sachdev, Vandana; Shizukuda, Yukitaka; Birdsall, Charles W.; Moss, Joel

doi:10.1378/chest.07-1205

Cited by 78 publications

(56 citation statements)

References 39 publications

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“…PH is relatively uncommon in patients with lymphangioleiomyomatosis (112,113). Chronic hypoxemia and pulmonary capillary destruction caused by cystic lung lesions probably represent the predominant causes of PH.…”

Section: 2mentioning

confidence: 99%

Updated Clinical Classification of Pulmonary Hypertension

Simonneau

Robbins

Beghetti

et al. 2009

Journal of the American College of Cardiology

2,259

1,805

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The aim of a clinical classification of pulmonary hypertension (PH) is to group together different manifestations of disease sharing similarities in pathophysiologic mechanisms, clinical presentation, and therapeutic approaches. In 2003, during the 3rd World Symposium on Pulmonary Hypertension, the clinical classification of PH initially adopted in 1998 during the 2nd World Symposium was slightly modified. During the 4th World Symposium held in 2008, it was decided to maintain the general architecture and philosophy of the previous clinical classifications. The modifications adopted during this meeting principally concern Group 1, pulmonary arterial hypertension (PAH). This subgroup includes patients with PAH with a family history or patients with idiopathic PAH with germline mutations (e.g., bone morphogenetic protein receptor-2, activin receptor-like kinase type 1, and endoglin). In the new classification, schistosomiasis and chronic hemolytic anemia appear as separate entities in the subgroup of PAH associated with identified diseases. Finally, it was decided to place pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis in a separate group, distinct from but very close to Group 1 (now called Group 1'). Thus, Group 1 of PAH is now more homogeneous.

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Section: 2mentioning

confidence: 99%

Updated Clinical Classification of Pulmonary Hypertension

Simonneau

Robbins

Beghetti

et al. 2009

Journal of the American College of Cardiology

2,259

1,805

View full text Add to dashboard Cite

show abstract

“…2,9 Cardiopulmonary exercise testing and 6-minute walk test may reveal arterial desaturation. 10,11 Because LAM is almost exclusively a disease of women, therapy for the disease has been based on suppression of estrogen activity (e.g., progesterone, oophorectomy), but the effectiveness of these treatments is unproven.…”

Section: Introductionmentioning

confidence: 99%

Involvement of Lymphatics in Lymphangioleiomyomatosis

Glasgow

Taveira-DaSilva

Pacheco–Rodriguez

et al. 2009

Lymphatic Research and Biology

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Lymphangioleiomyomatosis (LAM), a rare multisystem disease, occurs primarily in women, with cystic destruction of the lungs, abdominal tumors, and involvement of the axial lymphatics in the thorax and abdomen. To understand the pathogenesis of LAM, we initiated a longitudinal study of patients with LAM; over 500 patients have been enrolled. LAM results from the proliferation of a neoplastic cell (LAM cell), which has mutations in the tuberous sclerosis complex (TSC) genes, TSC1 or TSC2. Consistent with their metastatic behavior, LAM cells were isolated from blood, urine, and chylous effusions. Surface proteins on LAM cells include those found on metastatic cells and those involved in cell migration. In the lung, LAM cells are found clustered in nodules, which appear in the walls of the cysts, and in the interstitium. LAM lung nodules are traversed by slitlike vascular structures, with lining cells showing reactivity with antibodies against components of lymphatic endothelial cells. The axial lymphatics appear to be infiltrated by LAM cells, which may result in obstruction and formation of chyle-filled lymphangioleiomyomas. LAM cell clusters have been isolated from chylous pleural effusions, and it is hypothesized that these clusters may be responsible for metastatic spread of LAM cells via lymphatic vessels. Consistent with a lymphangiogenic process, levels of VEGF-D, a lymphangiogenic factor, were higher in sera of patients with LAM and lymphatic involvement (i.e., lymphangioleiomyoma, adenopathy) than in healthy volunteers or LAM patients with cystic disease limited to the lung. These findings are consistent with an important function for lymphangiogenesis in LAM.

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“…Abnormal smooth muscle cells proliferate along the pulmonary lymphatics and have recently been demonstrated in the pulmonary arterial walls (Cottin et al 2012). PH prevalence in this population is unknown, but appears to range from 7 % (as assessed by echo > 35 mm) (Taveira-DaSilva et al 2007) to 45 % in patients awaiting lung transplantation (assessed by right heart catheterization; (Reynaud-Gaubert et al 2008). Reported severity is generally mild, with two studies demonstrating average mean PAP in the 32-33 mmHg range (Cottin et al 2012;Reynaud-Gaubert et al 2008).…”

Section: Interstitial Lung Diseasesmentioning

confidence: 96%

Pulmonary Hypertension Associated with Respiratory Diseases

Kolb

Hassoun

2014

PanVascular Medicine

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Pulmonary hypertension is a common complication of many chronic respiratory diseases. The mechanisms contributing to the development of pulmonary hypertension in this context are multiple and go beyond chronic hypoxic vasoconstriction. Outcomes are universally worsened when pulmonary hypertension complicates chronic respiratory disease. Treatment strategies target the underlying disease, as pulmonary vasodilator therapy has proven largely ineffective in trials to date. This chapter summarizes current understanding of the epidemiology, pathophysiology, and treatment of pulmonary hypertension as it relates to a spectrum of chronic respiratory diseases.

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Pulmonary Artery Pressure in Lymphangioleiomyomatosis

Cited by 78 publications

References 39 publications

Updated Clinical Classification of Pulmonary Hypertension

Updated Clinical Classification of Pulmonary Hypertension

Involvement of Lymphatics in Lymphangioleiomyomatosis

Pulmonary Hypertension Associated with Respiratory Diseases

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