Successful treatment of diffuse pulmonary lymphangiomatosis with sirolimus

Effective Initial Treatment of Diffuse Pulmonary Lymphangiomatosis with Sirolimus and Propranolol: A Case Report

“…One limitation of the present report is that we only evaluated the short-term response to the selected treatment, whereas other authors published the results of 12 and 21 months of treatment [8], and 36 months of treatment [19]. Moreover, the only post-initiation of treatment chest CT scan that we performed was carried out during the patient's COVID-19 infection.…”

“…Additionally, dietary treatments such as medium-chain triglycerides and high-protein diets can reportedly have positive effects in patients with DPL [19]. The most recent case reports have shown that sirolimus, an mTOR inhibitor [8,11,20] or bevacizumab, a vascular endothelial growth factor (VEFG) inhibitor [18,21] may be quite effective, improving lung function and achieving good clinical and radiological responses without causing serious adverse effects. Treatment with propranolol has also been shown to be effective in the management of DPL [7].…”

“…Treatment choices are mostly based on case reports because no definitive guidelines for the management of DPL have yet been developed. According to such reports, sirolimus, bevacizumab, and propranolol [3,[6][7][8][9][10][11] are the most commonly chosen and effective treatments for DPL. However, progressive DPL may require lung transplantation [12].…”

Systemic generalised lymphangiomatosis: unknown aetiology and a challenge to treat

“… 21 22 A similar observation has recently been made in the treatment of an adult patient with diffuse pulmonary lymphangiomatosis treated with sirolimus with a decrease in mediastinal masses and an improvement in pulmonary function. 23 …”

A case of diffuse pulmonary lymphangiomatosis with a venous anomaly presenting with acute respiratory failure and hemoptysis

“…However, since standard clinical studies on DPL are scarce, the clinical course and the prognosis can only be approximated based on the previous case reports and case series. Although most of the previously reported cases presented with chronic symptoms such as exertional dyspnea and cough, attention should be paid to the fact that a small number of patients present with acute and intense respiratory failure due to hemoptysis [ [3] , [4] , [5] ]. Libby et al reported a case of GLA involving thoracic lesions who underwent lobectomy due to uncontrollable massive hemoptysis [ 3 ].…”