Characteristics of Patients with Lymphangioleiomyomatosis and Pleural Effusion: A Systematic Review

Abstract

The characteristics of patients with lymphangioleiomyomatosis (LAM) are poorly defined, as they may present with or without pleural effusion (PE). We performed a systematic review across four electronic databases searching for studies reporting clinical findings, PE characteristics, and the most effective treatment of LAM. Case descriptions and retrospective studies were included, unrestricted by year of publication. The review consisted of 94 studies (199 patients) spanning a period of nearly 55 years. The median age was 38 years (range: 1 month to 69 years), and 79.7% were between 21 and 50 years old. All cases had dyspnea, 95% had a cough, and 87.5% had chest pain. PE was exudative chylothorax, usually unilateral (76%) and right-sided, predominantly lymphocytic, and with proportionately higher levels of proteins than lactate dehydrogenase. Sirolimus was effective in all cases, completely in 87%, and partially in 13%, although the number of patients receiving sirolimus was small. The present study confirmed that LAM and PE mainly occur in women of childbearing age (third to fifth decade of life). PE was usually unilateral and presented as a lymphocyte-predominant chylous exudate. The most effective treatment for PE seems to be sirolimus, although studies with larger series are needed to confirm this.

Spontaneous pneumothorax in diffuse cystic lung diseases

“…Concurrent chylothorax and pneumothorax can occur (26). The majority (~80%) of the pneumothoraces occur at rest or with minimal activity (15).…”

Lymphangioleiomyomatosis: diagnostic imaging in an uncommon post‐menopausal presentation

“…The same patient had a second presentation with increasing shortness of breath. Chest X‐ray showed mild pleural effusion and pleural fluid cytology showed a lymphocyte‐predominant chylous exudate, globular clusters of spindled to oval cells, with an envelope of epithelioid‐like flat cells, high cholesterol and high triglycerides, consistent with a diagnostic cytopathology sample of pleural effusions in lymphangioleiomyomatosis . An atypical screen was negative and vascular endothelial growth factor D was 1500 pg/mL (ref 600 pg/mL).…”

“…HRCT demonstrates recognisable and characteristic features of diffuse thin‐walled cystic transformation of the lung parenchyma . Chylothorax is a frequent complication, appearing in up to 30% of cases and presents a high risk of malnutrition . Pleural effusion in lymphangioleiomyomatosis is usually unilateral and right‐sided, presenting as a lymphocyte‐predominant chylous exudate with proportionately higher protein levels compared with lactate dehydrogenase .…”

“…Chylothorax is a frequent complication, appearing in up to 30% of cases and presents a high risk of malnutrition . Pleural effusion in lymphangioleiomyomatosis is usually unilateral and right‐sided, presenting as a lymphocyte‐predominant chylous exudate with proportionately higher protein levels compared with lactate dehydrogenase . Triglycerides should be determined to confirm chylothorax in the diagnostic work‐up …”

Clinical Course of Histologically Proven Multifocal Micronodular Pneumocyte Hyperplasia in Tuberous Sclerosis Complex: A Case Series and Comparison with Lymphangiomyomatosis

“…Histological examination typically reveals multicentric, well-demarcated, nodular type II pneumocystic growth [1, 2]. In contrast with lymphangioleiomyomatosis (LAM), which is a more common lung manifestation in patients with TSC [3], MMPH is less progressive [4, 5]. However, because of its rarity, no previous study has described the detailed clinical course of the disease, such as changes in radiographic findings and pulmonary function.…”