Pulmonary Lymphangiomyomatosis and Kidney Angiomyolipomas

L. Callol, Servicio de Aparato Respiratorio, Hospital Universitario del Aire, C/ Arturo Soria 82, E-28027 Madrid (Spain) Dear Sir A woman aged 39, with close antecedents of spontaneous pneumothorax receives medical advice for progressive dyspnea and hemoptysis. X-ray shows bilateral diffuse pattern, and thin-walled aerial cysts of several sizes localized diffusely in both lungs, confirmed with high-resolution CT. Abdominal CT and echography reveal angiomyolipomas in both kidneys and uterine leiomyoma. Brain CT is normal. Transbronchial biopsy and bronchoalveolar lavage are un-specific. Routine blood analysis is normal. Tuberculin skin test, mycobacteria and Lö-wenstein culture of sputum are negative. Basal gasometry shows hypoxemia. Functional respiratory exploration: severe obstruction and small airway disease, total lung capacity 138%, diffusion lung of CO 32%. Cycloergometry suggests high arteriolar pulmonary resistance. Gallium lung scan is normal. Open lung biopsy is conclusive of lymphangiomyomatosis (LAM). With immunohistochemical techniques high cyto-plasmatic positivity for desmine, weak for actin and negative for estrogenic receptors are detected. Treatment with medroxypro-gesterone acetate and tamoxifen was attempted. Total hysterectomy and bilateral salping-oophorectomy was made before the existence of gynecological clinics. The patient is currently undergoing treatment with medroxyprogesterone acetate. The rare association of kidney angiomyolipomas and LAM has been reported. In this case the symptoms are typical, but without chylothorax. The existence of airway obstruction in a woman in fertile age with bilateral reticulonodular chest X-ray pattern makes us think of this sickness. High-resolution CT suggests the diagnosis [1], although it is easily confused with lung emphysema or histiocytosis. The diagnosis is histological, usually with open lung biopsy. It is difficult to distinguish LAM from tuberous sclerosis (TS). Despite the fact that kidney angiomyolipomas are characteristic of TS, their association with LAM has been reported [2,3], as it happens in our case. TS has been excluded on the base of the absence of heritage component and intracranial or cutaneous lesions.