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| Disease found: | Diffuse Pulmonary Lymphangiomatosis |
| Current as of: | October 8, 2024 |
| Disease Overview: | Proliferation of lymphatic vessels in the lungs, pleura, and mediastinum, leading to dyspnea, chest pain, chronic cough, and chylous pleural effusions |
| Signs and Symptoms: | Fatigue, nausea, wheezing, dyspnea, chronic cough and hemoptysis, chest tightness, recurrent respirayory infections, and recurrent pneumonia. Can lead to chylous pleural effusions or pneumothorax |
| Diagnosis: | PFTs showing restrictive pattern or mixed restructive and obstructive pattern, lymphoscintigraphy |
| Treatment: | Surgical excision of affected tissue is sometimes effective. Sclerotherapy with doxycycline can be injected into the lesion |
| Clinical Management: | Interferon-alpha 2B and glucocorticoids |
| Referral: | Pulmonologist. Referral to Medical Genetics Department, if available. Initial virtual care is also available through organizations like TeleRare Health. |
| Clinical Trials: | Characterization of the pathogenesis of primary and secondary lymphatic disorders |