121 Lymphangioleiomyomatosis

Clinical Presentation

34-year-old woman with dyspnea

Radiologic Findings

PA chest radiograph (Fig. 121.1) demonstrates increased lung volumes without other radiographic abnormality or evidence of interstitial lung disease. HRCT (Figs. 121.2, 121.3) reveals profuse, uniform, bilateral, small thin-walled cysts affecting both lungs.

Diagnosis

Lymphangioleiomyomatosis

Differential Diagnosis

• Pulmonary Langerhans’ Cell Histiocytosis (PLCH)

• Pneumocystis jiroveci Pneumonia

• Emphysema

Discussion

Background

Lymphangioleiomyomatosis (LAM) is a rare idiopathic disease that affects women of childbearing age and is characterized by abnormal proliferation of smooth muscle cells (LAM cells) along lymphatics in the chest and abdomen but also along vessels and bronchi in the lung. Lymphatic involvement can lead to chylous pleural effusions and/or ascites.