[Spontaneous pneumothorax in young women: possible lymphangioleiomyomatosis].
(Recurrent) pneumothorax developed spontaneously in three women aged 33, 35 and 36 years, two of whom were pregnant. Morbid-anatomical examination of lung biopsy samples in two of them revealed proliferation of smooth muscle cells which through infiltration of pleura, septa, and alveoli had led to loss of pulmonary parenchyma and formation of cystic lesions; the cystic lesions were visible in a CT scan in all three patients. The diagnosis in 2 patients was 'lymphangioleiomyomatosis'; the third patient had anomalies compatible with lymphangioleiomyomatosis, but these were interpreted as tuberous sclerosis because of the presence of renal angiomyolipomas. This disorder occurs exclusively in women in the reproductive age. Treatment consisted in drainage of the pneumothorax, pleurodesis and pleurectomy, with administration of medroxy-progesterone. One year later, the pneumothorax had not recurred.
Louis
H
H
Afd. Interne Geneeskunde, Academisch Ziekenhuis Vrije Universiteit, Amsterdam.
Los
H
H
Lagendijk
J H
JH
de Graaff
C S
CS
Postmus
P E
PE
D000328:Adult; D005260:Female; D006801:Humans; D008175:Lung Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D018192:Lymphangioleiomyomatosis / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D008525:Medroxyprogesterone / Q000627:therapeutic use; D011030:Pneumothorax / Q000209:etiology*; D011247:Pregnancy; D011248:Pregnancy Complications / Q000209:etiology*; D045167:Progesterone Congeners / Q000627:therapeutic use; D012008:Recurrence
D045167:Progesterone Congeners; D008525:Medroxyprogesterone