Clinical outcomes and survival following lung transplantation in patients with lymphangioleiomyomatosis

Khawar, Muhammad Umair; Yazdani, Dina; Zhu, Zheng; Jandarov, Roman; Dilling, Daniel F.; Gupta, Nishant

doi:10.1016/j.healun.2019.06.015

Cited by 36 publications

(18 citation statements)

References 28 publications

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“…There is a substance in the urine of LAM patients called matrix metalloproteinase (MMP), and doxycycline has the effect of inhibiting MMP. In the New England Journal, it once reported [22,23] that a case of LAM patients received doxycycline treatment results: the patient's lung function was significantly improved, FEV1 increased from 21% to 35% after treatment (proportion to the expected value), and oxygen saturation and activity endurance have been significantly improved.…”

Section: Discussionmentioning

confidence: 99%

Video Assisted Thoracoscopic Surgery (VATS) of Lymphangiomyomatosis (LAM) with Multiple Pulmonary Bullae in a Woman: A Case Report and Literature Review

Wu¹,

Liu²,

Luo³

et al. 2021

J Clin Case Stu

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Background: Pulmonary lymphangiomyomatosis is relatively rare disease and it is a systemic disease with an unknown etiology. The abnormal proliferation of smooth muscle leads to the obstruction of bronchi, lymphatic vessels and small blood vessels. At present, there are not many clinical reports on the treatment of LAM thoracoscopic surgery. So this article reports a case of LAM in a middle-aged woman and reviews of the relevant literature.

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Section: Discussionmentioning

confidence: 99%

Video Assisted Thoracoscopic Surgery (VATS) of Lymphangiomyomatosis (LAM) with Multiple Pulmonary Bullae in a Woman: A Case Report and Literature Review

Wu¹,

Liu²,

Luo³

et al. 2021

J Clin Case Stu

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“…Lung transplantation is a reliable therapeutic option for end-stage pulmonary LAM. A recent study on the outcome of 138 LAM patients who underwent transplant in the USA showed a 10-year survival of 56%, which was significantly higher than in other lung diseases (32%): this advantage persisted after age-and sex-matched analysis [70]. Positive outcomes were also reported by two retrospective studies in a German and a Japanese centre even if the latter found a high rate of LAM-related complications in long-term follow-up (e.g.…”

Section: Lammentioning

confidence: 91%

Ultra-rare cystic disease

et al. 2020

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Diffuse cystic lung diseases include a group of heterogeneous disorders characterised by the presence of cysts within the lung parenchyma, sometimes showing a characteristic computed tomography scan pattern that allows diagnosis. The pathogenetic mechanisms underlying cyst formation in the lung are still not clear and a number of hypotheses have been postulated according to the different aetiologies: ball-valve effect, ischaemic dilatation of small airways and alveoli related to infiltration and obstruction of small vessels and capillaries that supply the terminal bronchioles and connective tissue degradation by matrix metalloproteases. A wide number of lung cyst diseases have been classified into six diagnostic groups according to the aetiology: neoplastic, congenital/genetic, lymphoproliferative, infective, associated with interstitial lung diseases, and other causes. This article focuses on lymphangioleiomyomatosis, pulmonary Langerhans cell histiocytosis and Erdheim–Chester disease, Birt–Hogg–Dubé, follicular bronchiolitis and lymphocytic interstitial pneumonia, light-chain deposition disease and amyloidosis, congenital lung disease associated with aberrant lung development and growth, and cystic lung disease associated with neoplastic lesion. These cystic diseases are epidemiologically considered as ultra-rare conditions as they affect fewer than one individual per 50 000 or fewer than 20 individuals per million. Despite the rarity of this group of disorders, the increasing use of high-resolution computed tomography has improved the diagnostic yield, even in asymptomatic patients allowing prompt and correct therapy and management without the need for a biopsy.

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“…Lung transplant for LAM is relatively rare; however, it is associated with better post-transplant survival compared to other advanced lung diseases. (1,(303)(304)(305)(306)(307) Fewer patients with LAM have required lung transplant since the standardized use of mTOR inhibitors as treatment, but it remains an indication for those with severely abnormal lung function (FEV 1 < 30%), exertional dyspnea (NYHA class III or IV), or hypoxemia at rest. (303)(304)(305)(306)(308)(309)(310)(311)(312)(313)(314)(315) While disease recurrence does occur post-transplant, it does not appear to limit survival.…”

Section: Lymphangioleiomyomatosis (Lam)mentioning

confidence: 99%

Consensus document for the selection of lung transplant candidates: An update from the International Society for Heart and Lung Transplantation

Leard

Holm

Valapour

et al. 2021

The Journal of Heart and Lung Transplantation

393

314

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This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.

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Clinical outcomes and survival following lung transplantation in patients with lymphangioleiomyomatosis

Cited by 36 publications

References 28 publications

Video Assisted Thoracoscopic Surgery (VATS) of Lymphangiomyomatosis (LAM) with Multiple Pulmonary Bullae in a Woman: A Case Report and Literature Review

Video Assisted Thoracoscopic Surgery (VATS) of Lymphangiomyomatosis (LAM) with Multiple Pulmonary Bullae in a Woman: A Case Report and Literature Review

Ultra-rare cystic disease

Consensus document for the selection of lung transplant candidates: An update from the International Society for Heart and Lung Transplantation

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