Primary care
Recommendation 1
Interferon-alpha 2B and glucocorticoids
Diffuse Pulmonary Lymphangiomatosis
Not yet reviewedThis information is AI generated and has not yet been reviewed by a specialist physician. AI can make mistakes.
Disease overview
Proliferation of lymphatic vessels in the lungs, pleura, and mediastinum, leading to dyspnea, chest pain, chronic cough, and chylous pleural effusions
Common symptoms
Fatiguenauseawheezingdyspneachronic cough and hemoptysischest tightnessrecurrent respirayory infectionsand recurrent pneumonia. Can lead to chylous pleural effusions or pneumothorax
When to suspect
Recommendation 1
PFTs showing restrictive pattern or mixed restructive and obstructive pattern, lymphoscintigraphy
How to test
Recommendation 1
PFTs showing restrictive pattern or mixed restructive and obstructive pattern, lymphoscintigraphy
ZebraMD is partnering with Ambry Genetics for clinical-grade whole genome testing for rare diseases. Ambry accepts insurance. If you don’t have a physician to order this test, join our referral list and our team will reach out.
Treatment
Recommendation 1
Surgical excision of affected tissue is sometimes effective. Sclerotherapy with doxycycline can be injected into the lesion
Further support
Recommendation 1
Pulmonologist. Referral to Medical Genetics Department, if available. Initial virtual care is also available through organizations like TeleRare Health.
Clinical trials
Clinical Trials
Sources
No data available
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