[Sporadic lymphangioleiomyomatosis. Clinical and lung functional characteristics of 32 female patients].

BACKGROUND AND 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.

PATIENTS AND 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.

CONCLUSION

Lymphangioleiomyomatosis should be considered in women with spontaneous pneumothorax, unexplainable dyspnoea or angiomyolipoma.