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Pulmonary lymphangioleiomyomatosis

Guidelines

Key sources

The following summarized guidelines for the evaluation and management of pulmonary lymphangioleiomyomatosis are prepared by our editorial team based on guidelines from the International Society for Heart and Lung Transplantation (ISHLT 2021), the Japanese Respiratory Society (JRS/ATS 2017,2016), the Tuberous Sclerosis Complex International (TSCi 2013), and the European Respiratory Society (ERS 2010). ...
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Screening and diagnosis

Indications for screening: as per TSCi 2013 guidelines, obtain clinical screening for lymphangioleiomyomatosis symptoms, including exertional dyspnea and shortness of breath, at each clinic visit of patients with tuberous sclerosis. Review counseling regarding smoking risk and estrogen use at each clinic visit of patients at risk of lymphangioleiomyomatosis.
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Diagnostic investigations

History and physical examination: as per ERS 2010 guidelines, elicit a thorough personal and family history concerning the manifestations of tuberous sclerosis complex in patients presenting with lymphangioleiomyomatosis. Perform a physical examination by a physician familiar with the manifestations of tuberous sclerosis complex to assess the skin, retina, and nervous system.
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  • Chest imaging

  • Pulmonary function tests

  • Blood gas analysis

  • Cardiopulmonary exercise testing

  • Serum VEGF-D

  • Genetic testing

  • Abdominal imaging

  • Brain imaging

  • Screening for pulmonary hypertension

  • Screening for osteoporosis

Diagnostic procedures

Lung biopsy: as per ATS/JRS 2017 guidelines, consider performing transbronchial lung biopsy before surgical lung biopsy when a definitive diagnosis is required in patients with parenchymal cysts on HRCT characteristic of lymphangioleiomyomatosis but not having additional confirmatory features of lymphangioleiomyomatosis (clinical, radiologic or serologic).
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  • Histopathology

Medical management

mTOR inhibitors
As per ATS/JRS 2016 guidelines:
Initiate sirolimus rather than offering observation in patients with lymphangioleiomyomatosis with abnormal/declining lung function.
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Consider initiating sirolimus before invasive management in patients with lymphangioleiomyomatosis with symptomatic chylous fluid accumulations.
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  • Hormone therapy

  • Inhaled bronchodilators

  • Doxycycline

  • Management of osteoporosis

Nonpharmacologic interventions

Pulmonary rehabilitation: as per ERS 2010 guidelines, consider offering pulmonary rehabilitation in patients with lymphangioleiomyomatosis limited by dyspnea.
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Therapeutic procedures

Management of pneumothorax: as per ATS/JRS 2017 guidelines, consider offering ipsilateral pleurodesis after the initial pneumothorax episode rather than waiting for a recurrent pneumothorax before intervening with a pleural symphysis procedure in patients with lymphangioleiomyomatosis.
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  • Management of chylothorax

Surgical interventions

Lung transplantation: as per ISHLT 2021 guidelines, refer patients with lymphangioleiomyomatosis with any of the following despite mTOR inhibitor therapy for the evaluation for lung transplantation:
severely abnormal lung function (such as FEV1 < 30% predicted)
exertional dyspnea (NYHA class III or IV)
resting hypoxemia
pulmonary hypertension
refractory pneumothorax.
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Specific circumstances

Pregnant patients: as per ERS 2010 guidelines, counsel all patients, including patients with few or no symptoms, that there is a greater risk of pneumothorax and chylous effusion during pregnancy.
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  • Patients with renal angiomyolipoma

Patient education

General counseling: as per TSCi 2013 guidelines, counsel adolescent and adult female patients on smoking risks and estrogen use.
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  • Counseling on air travel

Preventative measures

Routine immunizations: as per ERS 2010 guidelines, offer influenza and pneumococcal vaccination in patients with lymphangioleiomyomatosis.
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Follow-up and surveillance

Assessment of disease progression: as per ERS 2010 guidelines, consider repeating lung function tests at 3-6 monthly intervals during the first year following diagnosis, and at 3-12 monthly intervals thereafter depending on the severity and progression of the disease.
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  • Post-transplant management