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.
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.
Authors: Jared Klarquist; Allison Barfuss; Sridhar Kandala; Mary J Reust; Ruedi K Braun; Jennifer Hu; Daniel F Dilling; Mark D McKee; Raymond E Boissy; Robert B Love; Michael I Nishimura; I Caroline Le Poole Journal: Am J Pathol Date: 2009-11-05 Impact factor: 4.307
Authors: Finn M Wilkens; Claudia Ganter; Katharina Kriegsmann; Heinrike Wilkens; Nicolas Kahn; Gillian C Goobie; Christopher J Ryerson; Michael Kreuter Journal: Respir Res Date: 2022-04-27