[Sporadic lymphangioleiomyomatosis. Clinical and lung functional characteristics of 32 female patients].
OBJECTIVE
Lymphangioleiomyomatosis (LAM) is a rare and progressive multi-systemic disorder almost exclusively of women. Clinical and lung functional data of a substantial number of German patients have not so far been published.


METHODS
In 32 women with sporadic LAM we performed the following investigations: medical history, clinical examination, lung function (spirometry, bodyplethysmography, diffusion capacity for carbon monoxide), ECG, echocardiography, and abdominal ultrasound.


RESULTS
The mean age at the time of investigation was 48 (25 - 66) years, while the mean age at time of diagnosis had been 45 (21 - 61) years. The median time between onset of first symptoms and diagnosis was 8 years (1 months - 30 years). Most frequently stated first symptom was dyspnea on exertion (12/32 patients) and the most frequently stated first clinical sign was pneumothorax (12/32). Changes of hormonal status (contraception, treatment for fertility, pregnancy) occurred in 31 of the 32 patients before onset of first symptoms or clinical signs. Hormonal (16/32) and antiobstructive therapy (16/32) were the most frequently applied forms of treatment. Airway obstruction (26/32 patients), hyperinflation (20/32) and reduced diffusion capacity for carbon monoxide (24/32) were found on lung function test. Abnormal auscultation (6 patients), ECG signs of chronic cor pulmonale (4/32) and an elevated systolic pulmonary artery pressure (2 patients) were rare findings. Abdominal lesions (angiomyolipoma, 11/32 patients; lymphangioleiomyoma, 4 patients) were common.


CONCLUSIONS
Lymphangioleiomyomatosis should be considered in women with spontaneous pneumothorax, unexplainable dyspnoea or angiomyolipoma.
Watz
H
H
Krankenhaus Grosshansdorf, Zentrum für Pneumologie und Thoraxchirurgie. h.watz@pulmoresearch.de
Oltmanns
A
A
Kimmel
S
S
Magnussen
H
H
Wirtz
H
H
Kirsten
D
D
D000008:Abdominal Neoplasms / Q000150:complications; D000328:Adult; D000368:Aged; D018207:Angiomyolipoma / Q000150:complications; D004417:Dyspnea / Q000209:etiology; D004562:Electrocardiography; D005260:Female; D005858:Germany / Q000453:epidemiology; D006801:Humans; D018192:Lymphangioleiomyomatosis / Q000150:complications / Q000453:epidemiology / Q000503:physiopathology*; D008875:Middle Aged; D011030:Pneumothorax / Q000209:etiology; D011660:Pulmonary Heart Disease / Q000209:etiology; D011669:Pulmonary Wedge Pressure; D012129:Respiratory Function Tests