Diffuse Pulmonary Lymphangiomatosis

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This 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

TREATMENT

  • Recommendation 1

    Surgical excision of affected tissue is sometimes effective. Sclerotherapy with doxycycline can be injected into the lesion

PRIMARY CARE

  • Recommendation 1

    Interferon-alpha 2B and glucocorticoids

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

Sources

No data available

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