Clinical and Imaging Manifestations of Patients with Pulmonary Lymphangioleiomyomatosis_Chinese Journal of Respiratory and Critical Care Medicine

Authors：

CaoXiaoyun ,  ShaoChangzhou , HeLixian , BaiChunxue

Department of Pulmonary Medicine, Zhongshan Hospital, Fudan University, Shanghai, 200032, China;

Corresponding author：

ShaoChangzhou, Email: sczsmh@aliyun.com.cn

Keywords：

Pulmonary lymphangioleiomyomatosis; Clinical manifestations; Imaging

DOI：

10.7507/1671-6205.2014009

Video：

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Objective To investigate the clinical and imaging features of patients with pulmonary lymphangioleiomyomatosis (PLAM),to improve the knowledge of this disease. Methods Eighteen patients with PLAM admitted into Zhongshan Hospital between 2003 and 2013 were retrospectively analyzed. Relevant literatures were reviewed. Results All the 18 cases were female and had dyspnea on exertion. Three of them suffered from chylothorax,6 from pneumothorax and 5 from hemoptysis. The high resolution CT (HRCT) findings in all the 18 cases showed multiple small cysts with thin wall diffusely distributing in the bilateral lung. PLAM was confirmed by biopsy in 14 cases. Pulmonary function tests in 4 cases showed obstructive ventilatory dysfunction. Nine cases (50%) were misdiagnosed as other diseases. All the patients were given symptomatic and supportive treatment. Literatures review showed that as a rare diffuse lung disease,PLAM is more common in women of childbearing age,mainly presenting with dyspnea,hemoptysis,pneumothorax and chylothorax. Diagnosis of the disease relies on medical history,HRCT features and lung tissue biopsy pathology. HRCT examination has diagnostic value for PLAM. Although anti-estrogen treatment has certain effects,rapamycin may be a promising drug for PLAM therapy. Conclusion Patients with PLAM have characteristic clinical and radiographic manifestations. Early diagnosis and effective treatment are keys to the management of patients with PLAM.

Citation： CaoXiaoyun, ShaoChangzhou, HeLixian, BaiChunxue. Clinical and Imaging Manifestations of Patients with Pulmonary Lymphangioleiomyomatosis. Chinese Journal of Respiratory and Critical Care Medicine, 2014, 13(1): 34-37. doi: 10.7507/1671-6205.2014009 Copy

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2.	Johnson SR,Cordier JF,Lazor R,et al.European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis.Eur Respir J,2010,35:14-26.
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7.	卢韶华,谭云山,许建芳,等.肺淋巴管肌瘤病的临床及病理特点.中华结核和呼吸杂志,2009,32:664-669.
8.	Xu F,Yang Y,Xia JY,et al.Clinical profiles of pulmonary lymphangioleiomyomatosis in the mainland of China.Chin Med J (Engl),2009,122:1473-1476.
9.	王涛.肺淋巴管平滑肌瘤病的X线、CT表现.当代医学,2011,17:11-13.
10.	叶伶,金美玲.肺淋巴管平滑肌瘤病发病机制的研究进展.国际呼吸杂志,2006,26:932-934.
11.	Mitani K,Kumasaka T,Takemura H,et al.Cytologic,immunocytochemical and ultrastructural characterization of lymphangioleiomyomatosis cell clusters in chylous effusions of patients with lymphangioleiomyomatosis.Acta Cytol,2009,53:402-409.
12.	Flavin RJ,Cook J,Fiorentino M,et al.β-Catenin is a useful adjunct immunohistochemical marker for the diagnosis of pulmonary lymphangioleiomyomatosis.Am J Clin Pathol,2011,135:776-782.
13.	Karbowniczek M,Astrinidis A,Balsara BR,et al.Recurrent lymphangiomyomatosis after transplantation:genetic analyses reveal a metastatic mechanism.Am J Respir Crit Care Med,2003,167:976-982.
14.	Bittmann I,Rolf B,Amann G,et al.Recurrence of lymphangioleiomyomatosis after single lung transplantation:new insights into pathogenesis.Hum Pathol,2003,34:95-98.
15.	Karbowniczek M,Astrinidis A,Balsara BR,et al.Recurrent lymphangiomyomatosis after transplantation:genetic analyses reveal a metastatic mechanism.Am J Respir Crit Care Med,2003,167:976-982.
16.	Johnson SR,Whale CI,Hubbard RB,et al.Survival and disease progression in UK patients with lymphangioleiomyomatosis.Thorax,2004,59:800-803.
17.	Bissler JJ,McCormack FX,Young LR,et al.Sirolimus for angiomyolipoma in tuberous sclerosis complex or lymphangioleiomyomatosis.N Engl J Med,2008,358:140-151.

1. Cornog JL Jr,Enterline HT.Lymphangiomyoma,a benign lesion of chyliferous lymphatics synonymous with lymphangiopericytoma.Cancer,1966,19:1909-1930.
2. Johnson SR,Cordier JF,Lazor R,et al.European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis.Eur Respir J,2010,35:14-26.
3. Miyake M,Tateishi U,Maeda T,et al.Pulmonary lymphangioleiomyomatosis in a male patient with tuberous sclerosis complex.Radiat Med,2005,23:525-527.
4. Whale CI,Johnson SR,Phillips KG,et al.Lymphangioleioomyomatosis:a case-control study of perinatal and early life events.Thorax,2003,58:979-982.
5. Hayashida M,Seyama K,Inoue Y,et al.The epidemiology of lymphangioleiomyomatosis in Japan:a nationwide cross-sectional study of presenting features and prognostic factors.Respirology,2007,12:523-530.
6. Zhang X,Travis WD.Pulmonary lymphangioleiomyomatosis.Arch Pathol Lab Med,2010,134:1823-1828.
7. 卢韶华,谭云山,许建芳,等.肺淋巴管肌瘤病的临床及病理特点.中华结核和呼吸杂志,2009,32:664-669.
8. Xu F,Yang Y,Xia JY,et al.Clinical profiles of pulmonary lymphangioleiomyomatosis in the mainland of China.Chin Med J (Engl),2009,122:1473-1476.
9. 王涛.肺淋巴管平滑肌瘤病的X线、CT表现.当代医学,2011,17:11-13.
10. 叶伶,金美玲.肺淋巴管平滑肌瘤病发病机制的研究进展.国际呼吸杂志,2006,26:932-934.
11. Mitani K,Kumasaka T,Takemura H,et al.Cytologic,immunocytochemical and ultrastructural characterization of lymphangioleiomyomatosis cell clusters in chylous effusions of patients with lymphangioleiomyomatosis.Acta Cytol,2009,53:402-409.
12. Flavin RJ,Cook J,Fiorentino M,et al.β-Catenin is a useful adjunct immunohistochemical marker for the diagnosis of pulmonary lymphangioleiomyomatosis.Am J Clin Pathol,2011,135:776-782.
13. Karbowniczek M,Astrinidis A,Balsara BR,et al.Recurrent lymphangiomyomatosis after transplantation:genetic analyses reveal a metastatic mechanism.Am J Respir Crit Care Med,2003,167:976-982.
14. Bittmann I,Rolf B,Amann G,et al.Recurrence of lymphangioleiomyomatosis after single lung transplantation:new insights into pathogenesis.Hum Pathol,2003,34:95-98.
15. Karbowniczek M,Astrinidis A,Balsara BR,et al.Recurrent lymphangiomyomatosis after transplantation:genetic analyses reveal a metastatic mechanism.Am J Respir Crit Care Med,2003,167:976-982.
16. Johnson SR,Whale CI,Hubbard RB,et al.Survival and disease progression in UK patients with lymphangioleiomyomatosis.Thorax,2004,59:800-803.
17. Bissler JJ,McCormack FX,Young LR,et al.Sirolimus for angiomyolipoma in tuberous sclerosis complex or lymphangioleiomyomatosis.N Engl J Med,2008,358:140-151.

Chinese Journal of Respiratory and Critical Care Medicine

Clinical and Imaging Manifestations of Patients with Pulmonary Lymphangioleiomyomatosis

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