Lymphangioleiomyomatosis Diagnosis and Management: High-Resolution Chest Computed Tomography, Transbronchial Lung Biopsy, and Pleural Disease Management. An Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guideline

Gupta, Nishant; Finlay, Geraldine A.; Kotloff, Robert M.; Strange, Charlie; Wilson, Kevin C.; Young, Lisa R.; Taveira-DaSilva, Angelo M.; Johnson, Simon R.; Cottin, Vincent; Sahn, Steven A.; Ryu, Jay H.; Seyama, Kuniaki; Inoue, Yoshikazu; Downey, Gregory P.; Han, Mei Lan K.; Colby, Thomas V.; Wikenheiser-Brokamp, Kathryn A.; Meyer, Cristopher A.; Smith, Karen; Moss, Joel; McCormack, Francis X.

doi:10.1164/rccm.201709-1965st

Cited by 189 publications

(221 citation statements)

References 58 publications

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“…A recent study has shown that performing a screening high-resolution computed tomography (HRCT) among patients presenting with an apparent primary spontaneous pneumothorax is cost-effective, and can help in alleviating the delay in diagnosis for at least a proportion of LAM patients (N. Gupta, Langenderfer, McCormack, Schauer, & Eckman, 2017). Gupta, Finlay, et al, 2017). In the absence of one of the above confirmatory features, histopathological diagnosis of LAM may need to be established by a lung biopsy (N. Gupta, Finlay, et al, 2017).…”

Section: Clinical Manifestationsmentioning

confidence: 99%

“…Gupta, Finlay, et al, 2017). In the absence of one of the above confirmatory features, histopathological diagnosis of LAM may need to be established by a lung biopsy (N. Gupta, Finlay, et al, 2017).…”

Section: Clinical Manifestationsmentioning

confidence: 99%

“…Gupta, Finlay, et al, 2017). Most patients with a sentinel spontaneous pneumothorax experience multiple recurrences with an average of 3.2-5 pneumothorax episodes per patient (N. Gupta, Finlay, et al, 2017).…”

Section: Spontaneous Pneumothoraxmentioning

confidence: 99%

“…Given the high recurrence rate, patients with LAM should be offered pleurodesis following the first episode of spontaneous pneumothorax rather than waiting for a recurrent event. Video-Assisted Thoracoscopic Surgery (VATS)guided mechanical abrasion is the preferred modality for pleurodesis in this population, with more aggressive measures, such as talc pleurodesis reserved for refractory or recurrent pneumothoraces (N. Gupta, Finlay, et al, 2017). It is important to note that prior pleurodesis, whether mechanical or talc pleurodesis, is not a contraindication for future lung transplantation (N. Gupta, Finlay, et al, 2017;Weill et al, 2015), and should be offered to all LAM patients with a spontaneous pneumothorax.…”

Section: Spontaneous Pneumothoraxmentioning

confidence: 99%

“…The average age at the time of development of spontaneous pneumothorax in LAM is around 35 years (Almoosa et al, 2006). More than 50% of LAM patients experience at least one spontaneous pneumothorax in their lifetime, with a recurrence rate of approximately 70% (Almoosa et al, 2006; N. Gupta, Finlay, et al, 2017).…”

Section: Introductionmentioning

confidence: 99%

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Pulmonary manifestations in tuberous sclerosis complex

Gupta

Henske

2018

American J of Med Genetics Pt C

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Tuberous sclerosis complex has manifestations in many organ systems, including brain, heart, kidney, skin, and lung. The primary manifestations in the lung are lymphangioleiomyomatosis (LAM) and multifocal micronodular pneumocyte hyperplasia (MMPH). LAM affects almost exclusively women, and causes cystic lung destruction, pneumothorax, and chylous pleural effusions. LAM can lead to dyspnea, oxygen dependence, and respiratory failure, with more rapid disease progression during the premenopausal years. In contrast, MMPH affects men and women equally, causing small nodular pulmonary deposits of type II pneumocytes that rarely progress to symptomatic disease. Here, we review the clinical features and pathogenesis of LAM and MMPH.

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Section: Clinical Manifestationsmentioning

confidence: 99%

Section: Clinical Manifestationsmentioning

confidence: 99%

Section: Spontaneous Pneumothoraxmentioning

confidence: 99%

Section: Spontaneous Pneumothoraxmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Pulmonary manifestations in tuberous sclerosis complex

Gupta

Henske

2018

American J of Med Genetics Pt C

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Serum Vascular Endothelial Growth Factor D in Cystic Lung Disease

Ataya

Riley

Brantly

2019

JAMA

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A 28-year-old nonsmoking woman was referred to the pulmonary clinic for further evaluation of shortness of breath with exertion. She had slowly progressing dyspnea over the past year and general fatigue. She did not report a history of xerostomia, keratoconjunctivitis sicca, pleural effusions, pneumothoraces, seizure, cognitive impairment, recurrent sinopulmonary infections, or previous autoimmune disease diagnosis and had no relevant family history of lung disease. Her physical examination findings were unremarkable and there was no evidence of cutaneous lesions. A high-resolution computed tomographic (CT) scan of her chest revealed diffuse thin-walled pulmonary cysts without nodules, parenchymal changes, or lymphadenopathy (Figure). Imaging of her abdomen did not show kidney lesions or abdominal masses. The patient's serum vascular endothelial growth factor D (VEGF-D) level was elevated at 1300 pg/mL (reference range, <600 pg/mL).

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Evolution of lung pathology in lymphangioleiomyomatosis: associations with disease course and treatment response

Miller

Stewart²,

Clements

et al. 2020

The Journal of Pathology CR

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Lymphangioleiomyomatosis (LAM) is a rare multisystem disease with a variable clinical course. The lungs are infiltrated by nodules of LAM cells, stromal cells and inflammatory cells, causing lung cysts and respiratory failure. We used immunohistochemical markers in lung biopsy and transplant samples from a national cohort of women with LAM with linked clinical data to understand how LAM nodule cell populations changed with disease progression. Marker distribution was examined qualitatively by dual immunohistochemistry, and markers for LAM cells, fibroblasts, lymphatics, mast cells, proliferation, cathepsin K and mTOR pathway activity were quantitated in LAM nodules and compared with clinical features and prospective lung function loss. The LAM cell marker PNL2 was more extensively expressed in those with higher forced expiratory volume in one second (FEV 1 ), higher diffusion in the lung for carbon monoxide (DL CO ) and less extensive disease involvement whilst the converse was true for the protease cathepsin K. Each percentage increase in cathepsin K reactivity was associated with a 0.65% decrease in FEV 1 (95% CI −1.11 to −0.18) and a 0.50% decrease in DL CO (95% CI −0.96 to −0.05). Higher reactivity to the mTOR complex 1 activation marker, phospho‐ribosomal protein S6, was associated with a better lung function response to rapamycin ( p = 0.0001). We conclude that LAM nodules evolve with disease progression, with LAM cells becoming outnumbered by fibroblasts. Increasing cathepsin K expression is associated with more severe disease and lung function loss. Markers of mTOR activation predict the response to rapamycin, suggesting that more advanced LAM may be less mTOR responsive and treatments specifically targeted towards LAM associated fibroblasts may have roles as adjuncts to mTOR inhibition.

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Lymphangioleiomyomatosis Diagnosis and Management: High-Resolution Chest Computed Tomography, Transbronchial Lung Biopsy, and Pleural Disease Management. An Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guideline

Abstract: Evidence-based recommendations for the diagnosis and treatment of patients with LAM are provided. Frequent reassessment and updating will be needed.

Cited by 189 publications

References 58 publications

Pulmonary manifestations in tuberous sclerosis complex

Pulmonary manifestations in tuberous sclerosis complex

Serum Vascular Endothelial Growth Factor D in Cystic Lung Disease

Evolution of lung pathology in lymphangioleiomyomatosis: associations with disease course and treatment response

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