Clinical Comprehensive Analysis Of 18 Patients With Pulmonary Lymphangioleiomyomatosis

Objective To improve the understanding of the natural history of pulmonary lymphangioleiomyomatosis (LAM) by the analysis of clinical features, diagnosis, management and prognosis of Chinese patients with LAM. Methods The detail of one case of LAM treated at the second affiliated hospital of Zhejiang University medical college was retrospectively analyzed and reviewed together with those of 17 cases previously reported from the literature and confirmed by pathology of biopsy specimens. These data were compared with those of foreign studies. Results Among the 18 patients, 16 cases were female (89%), and 2 cases were male (11%). Mean age at the onset of symptoms was 35 ± 14 years (range, 5 to 69 years). The clinical features at the time of onset were dyspnea (10/18, 56%), hemoptysis (6/18, 50%), cough (6/18, 33%), pneumothorax (5/18, 28%) and chest pain (3/18, 17%). Meanwhile, major clinical features during the course of LAM were dyspnea (15/18, 83%), cough (14/18, 78%), hemoptysis (12/18, 67%), pneumothorax (8/18, 44%), chylothorax (6/18, 33%). The characteristic thin-walled cysts diffusedly distributing throughout both lung fields on chest computed tomographic scanning occurred in all 16 cases who had already received this scanning. Six of the 10 patients having undergone pulmonary functiontests showed an obstructive ventilated dysfunction, whereas 4 showed a combined restrictive and obstructive ventilated dysfunction. All of 7 cases had reduction of carbon monoxide diffusing capacity. Thirteen cases took arterial blood gas measurement with the result that hypoxemia and hypercapnia occurred in 12 cases (92%) and 4 cases (31%), respectively. The time to diagnosis from the onset of symptoms ranged 1 month to 10 years (median 8 months) in the 18 patients. After the diagnosis of LAM confirmed, 10 cases received hormone therapy with intramuscular medroxyprogesterone and one case was treated with oral medroxyprogesterone acetate. However, the symptoms of LAM were only improved in 5 cases with this therapy during short period (< 6 months), and then 6 cases were ineffective or dead. Eight patients died among the 18 patients nowadays. The duration to death, because of LAM and its complications, from the onset of symptoms in those patients ranged 5months to 26 years (median 42 months). Conclusions LAM is a rare lung interstitial disease almost exclusively affecting women of reproductive age, of which aetiology is unknown hitherto. The clinical characteristics of Chinese patients with LAM include slowly progressive dyspnea, hemoptysis, recurrent spontaneous pneumothorax, chylothorax, chylous ascites, and diffuse cystic shadowing throughout both lungs on high resolution computerized tomography (HRCT). Because medroxyprogesterone is of worse effect on the treatment of LAM, new therapies to improve the prognosis of LAM ought to be researched in the future.