[A rare interstitial lung disease].

Pulmonary lymphangioleiomyomatosis (p.l.) is a rare disease of unknown etiology, and restricted to fertile women. It is characterized by a nodular proliferation of smooth muscle cells in the peribronchial, perivascular and perilymphatic lung tissue, accompanied by cystic dilations of the alveoles, rupture of the alveolar wall, lymphangiectasis, and septal collagen fiber deposition. Radiological-alterations range from enhanced interstitial shadowing to honey comb lung. Common clinical symptoms are progressive dyspnea, pneumothorax, chylous pleural effusion and hemoptysis. Here we present the case of a 43 years old woman, undergoing nephrectomy because of hamartoma of the left kidney, with recurrent pneumothorax and progressive dyspnea, verifying the diagnosis of p.l. by open lung biopsy. Pathogenesis of the disease, differential diagnosis and possible therapeutic approaches are discussed.