Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guidelines: Lymphangioleiomyomatosis Diagnosis and Management

McCormack, Francis X.; Gupta, Nishant; Finlay, Geraldine R.; Young, Lisa R.; Silva, Angelo M. Taveira-Da; Glasgow, Connie G.; Steagall, Wendy K.; Johnson, Simon R.; Sahn, Steven A.; Ryu, Jay H.; Strange, Charlie; Seyama, Kuniaki; Sullivan, Eugene J.; Kotloff, Robert M.; Downey, Gregory P.; Chapman, Jeffrey T.; Han, MeiLan K.; DʼArmiento, Jeanine; Inoue, Yoshikazu; Henske, Elizabeth P.; Bissler, John J.; Colby, Thomas V.; Kinder, Brent W.; Wikenheiser-Brokamp, Kathryn A.; Brown, Kevin K.; Cordier, J.-F.; Meyer, Cristopher A.; Cottin, Vincent; Brožek, Jan; Smith, Karen; Wilson, Kevin C.; Moss, Joel

doi:10.1164/rccm.201607-1384st

Cited by 253 publications

(305 citation statements)

References 104 publications

(103 reference statements)

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“…TSC, angiomyolipomas, chylous pleural effusions or ascites, and cystic lymphangioleiomyomas) are lacking [29,30]. The suggested diagnostic threshold is 800 pg·mL −1 (table 1).…”

Section: Biomarkersmentioning

confidence: 99%

See 1 more Smart Citation

New insights in lymphangioleiomyomatosis and pulmonary Langerhans cell histiocytosis

2017

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Lymphangioleiomyomatosis (LAM) and pulmonary Langerhans cell histiocytosis (PLCH) are rare diseases that lead to progressive cystic destruction of the lungs. Despite their distinctive characteristics, these diseases share several features. Patients affected by LAM or PLCH have similar radiological cystic patterns, a similar age of onset, and the possibility of extrapulmonary involvement. In this review, the recent advances in the understanding of the molecular pathogenesis, as well as the current and most promising biomarkers and therapeutic approaches, are described.

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“…TSC, angiomyolipomas, chylous pleural effusions or ascites, and cystic lymphangioleiomyomas) are lacking [29,30]. The suggested diagnostic threshold is 800 pg·mL −1 (table 1).…”

Section: Biomarkersmentioning

confidence: 99%

“…The panel members for those guidelines also recommend sirolimus for patients with symptomatic chylous fluid effusions before invasive procedures are considered [29].…”

Section: Lung Function Impairmentmentioning

confidence: 99%

New insights in lymphangioleiomyomatosis and pulmonary Langerhans cell histiocytosis

2017

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“…When screened by HRCT, the prevalence of pulmonary LAM in TSC appears to increase with age, from 27% in women under 21 years to 81% in women over 40 years [16-19]. At diagnosis of LAM, patients with TSC-LAM have better lung function than patients with S-LAM, although this probably results from earlier diagnosis [10, 20].…”

Section: Lam and Tuberous Sclerosismentioning

confidence: 99%

Cystic Lung Disease in Genetic Syndromes with Deficient Tumor Suppressor Gene Function

Daccord¹,

Nicod²,

Lazor³

2017

Respiration

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Cystic lung diseases constitute a distinct group of rare lung disorders, among which two result from monogenic defects affecting tumor suppressor genes: lymphangioleiomyomatosis, either sporadic or associated with tuberous sclerosis complex, and Birt-Hogg-Dubé syndrome. These disorders have similarities in their clinical expression, including occurrence in young adults, multiple pulmonary cysts, recurrent pneumothorax, skin hamartomas, and renal tumors. However, they markedly differ in their gender distribution, pathogenesis, disease course, and prognosis. Our knowledge on these two rare conditions is rapidly expanding. 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. Birt-Hogg-Dubé syndrome has been described more recently and still awaits deeper understanding of its pathophysiology.

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“…По мнению большинства исследователей, основ-ным методом лучевой диагностики ЛАМ является КТ, которая, особенно при бессимптомном течении забо-левания, является неоспоримо ведущим методом имидж диагностики [3,7,12,54,75].…”

Section: рентгенологические проявления ламunclassified

“…Для подведения итогов полученной информации из приведенных выше литературных источников и собст-венного клинического опыта считаем целесообразным познакомить с некоторыми положениями основных ре-комендаций по лимфангиолеиомиоматозу, подготов-ленными Европейским респираторным обществом (ERS) [47] и Американским торакальным обществом совместно с Японским респираторным обществом (ATS/JRS) [54].…”

Section: собственное наблюдениеunclassified

Benign Uterus Cancer With Lung Metastases: Leiomyomatosis, Lymphangioleimyomatosis, Thoracic Endometriosis (Literature Review, Personal Clincial Observations)

Леншин¹,

Леншин

Ильин³

et al. 2017

Bulletin Physiology and Pathology of Respiration

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Own clinical observations of rare pulmonary diseases related to the uterus pathology are presented. These observations reflect clinical-roentgenologic features of benign uterus leiomyoma with lung metastases, lymphangioleiomyomatosis and thoracic endometriosis. The description of three different diseases in one report is conditioned by the fact that they all have one and the same lesion of metastases, i.e. hormonally active uterus in women of reproductive age. Though these diseases are different, but they demand differential diagnostics both between each other and other disseminated pulmonary diseases. This work demonstrates the possibilities of application of multispiral computed tomography with postprocessing imaging. A lot of attention is paid to roentgen semiotics of pulmonary changes typical for each of these diseases.

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Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guidelines: Lymphangioleiomyomatosis Diagnosis and Management

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

Cited by 253 publications

References 104 publications

New insights in lymphangioleiomyomatosis and pulmonary Langerhans cell histiocytosis

New insights in lymphangioleiomyomatosis and pulmonary Langerhans cell histiocytosis

Cystic Lung Disease in Genetic Syndromes with Deficient Tumor Suppressor Gene Function

Benign Uterus Cancer With Lung Metastases: Leiomyomatosis, Lymphangioleimyomatosis, Thoracic Endometriosis (Literature Review, Personal Clincial Observations)

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