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DOI: 10.1055/s-0032-1325159
Lymphangioleiomyomatosis: New Concepts in Pathogenesis, Diagnosis, and Treatment
Publication History
Publication Date:
21 September 2012 (online)
![](https://www.thieme-connect.de/media/srccm/201205/lookinside/thumbnails/10.1055-s-0032-1325159-1.jpg)
Abstract
Lymphangioleiomyomatosis (LAM) is a slowly progressive lung disease that is associated with mutations in tuberous sclerosis complex genes, infiltration of the pulmonary parenchyma and lymphatics with neoplastic smooth muscle cells, extensive tissue remodeling and architectural distortion of the lung, and tumors of the chest and abdomen, including lymphangiomyomas and angiomyolipomas. LAM occurs in women in the general population and in patients of both genders with tuberous sclerosis. Overt clinical manifestations of LAM occur almost exclusively in females, however, and include progressive dyspnea on exertion, recurrent pneumothorax, and chylous effusions. The molecular basis of LAM has been extensively characterized over the past decade, resulting in the development of a targeted therapy. This article reviews emerging approaches to the diagnosis and treatment of LAM.
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References
- 1 Johnson SR. Lymphangioleiomyomatosis. Eur Respir J 2006; 27 (5) 1056-1065
- 2 McCormack FX. Lymphangioleiomyomatosis: a clinical update. Chest 2008; 133 (2) 507-516
- 3 Matsui K, Tatsuguchi A, Valencia J , et al. Extrapulmonary lymphangioleiomyomatosis (LAM): clinicopathologic features in 22 cases. Hum Pathol 2000; 31 (10) 1242-1248
- 4 Harknett EC, Chang WY, Byrnes S , et al. Use of variability in national and regional data to estimate the prevalence of lymphangioleiomyomatosis. QJM 2011; 104 (11) 971-979
- 5 Harknett E, Chang WY, Hubbard RB, Johnson SR. Prevalence and regional variation of LAM in the UK and USA [abstract]. Am J Respir Cell Mol Biol 2010; 181: A2101
- 6 Muzykewicz DA, Sharma A, Muse V, Numis AL, Rajagopal J, Thiele EA. TSC1 and TSC2 mutations in patients with lymphangioleiomyomatosis and tuberous sclerosis complex. J Med Genet 2009; 46 (7) 465-468
- 7 Adriaensen ME, Schaefer-Prokop CM, Duyndam DA, Zonnenberg BA, Prokop M. Radiological evidence of lymphangioleiomyomatosis in female and male patients with tuberous sclerosis complex. Clin Radiol 2011; 66 (7) 625-628
- 8 Schiavina M, Di Scioscio V, Contini P , et al. Pulmonary lymphangioleiomyomatosis in a karyotypically normal man without tuberous sclerosis complex. Am J Respir Crit Care Med 2007; 176 (1) 96-98
- 9 Nagy B, Nábrády Z, Nemes Z. Pulmonary lymphangiomyomatosis in a preadolescent girl. N Engl J Med 1998; 338 (7) 473-474
- 10 Ho TB, Hull JH, Hughes NC. An 86-year-old female with lymphangioleiomyomatosis. Eur Respir J 2006; 28 (5) 1065
- 11 Almoosa KF, Ryu JH, Mendez J , et al. Management of pneumothorax in lymphangioleiomyomatosis: effects on recurrence and lung transplantation complications. Chest 2006; 129 (5) 1274-1281
- 12 Johnson SR, Tattersfield AE. Clinical experience of lymphangioleiomyomatosis in the UK. Thorax 2000; 55 (12) 1052-1057
- 13 Johnson SR, Cordier JF, Lazor R , et al; Review Panel of the ERS LAM Task Force. European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis. Eur Respir J 2010; 35 (1) 14-26
- 14 Ferrans VJ, Yu ZX, Nelson WK , et al. Lymphangioleiomyomatosis (LAM): a review of clinical and morphological features. J Nihon Med Sch 2000; 67 (5) 311-329
- 15 Matsumoto Y, Horiba K, Usuki J, Chu SC, Ferrans VJ, Moss J. Markers of cell proliferation and expression of melanosomal antigen in lymphangioleiomyomatosis. Am J Respir Cell Mol Biol 1999; 21 (3) 327-336
- 16 Logginidou H, Ao X, Russo I, Henske EP. Frequent estrogen and progesterone receptor immunoreactivity in renal angiomyolipomas from women with pulmonary lymphangioleiomyomatosis. Chest 2000; 117 (1) 25-30
- 17 Matsui K, Takeda K, Yu ZX , et al. Downregulation of estrogen and progesterone receptors in the abnormal smooth muscle cells in pulmonary lymphangioleiomyomatosis following therapy: an immunohistochemical study. Am J Respir Crit Care Med 2000; 161 (3 Pt 1) 1002-1009
- 18 Taylor JR, Ryu J, Colby TV, Raffin TA. Lymphangioleiomyomatosis: clinical course in 32 patients. N Engl J Med 1990; 323 (18) 1254-1260
- 19 Taveira-DaSilva AM, Stylianou MP, Hedin CJ, Hathaway O, Moss J. Decline in lung function in patients with lymphangioleiomyomatosis treated with or without progesterone. Chest 2004; 126 (6) 1867-1874
- 20 Urban T, Lazor R, Lacronique J , et al. Pulmonary lymphangioleiomyomatosis: a study of 69 patients. Groupe d'Etudes et de Recherche sur les Maladies “Orphelines” Pulmonaires (GERM”O”P). Medicine (Baltimore) 1999; 78 (5) 321-337
- 21 Winter JA. Oophorectomy in lymphangioleiomyomatosis and benign metastasizing leiomyoma. N Engl J Med 1981; 305 (23) 1416-1417
- 22 Schiavina M, Contini P, Fabiani A , et al. Efficacy of hormonal manipulation in lymphangioleiomyomatosis: a 20-year-experience in 36 patients. Sarcoidosis Vasc Diffuse Lung Dis 2007; 24 (1) 39-50
- 23 Baldi BG, Medeiros Junior P, Pimenta SP, Lopes RI, Kairalla RA, Carvalho CR. Evolution of pulmonary function after treatment with goserelin in patients with lymphangioleiomyomatosis. J Bras Pneumol 2011; 37 (3) 375-379
- 24 Harari S, Cassandro R, Chiodini I, Taveira-DaSilva AM, Moss J. Effect of a gonadotrophin-releasing hormone analogue on lung function in lymphangioleiomyomatosis. Chest 2008; 133 (2) 448-454
- 25 Cordier JF, Johnson SR. Mutiple cystic lung diseases. Eur Respir Mon 2011; 54: 46-83
- 26 Taveira-DaSilva AM, Steagall WK, Moss J. Lymphangioleiomyomatosis. Cancer Control 2006; 13 (4) 276-285
- 27 European Chromosome 16 Tuberous Sclerosis Consortium. Identification and characterization of the tuberous sclerosis gene on chromosome 16. Cell 1993; 75 (7) 1305-1315
- 28 van Slegtenhorst M, de Hoogt R, Hermans C , et al. Identification of the tuberous sclerosis gene TSC1 on chromosome 9q34. Science 1997; 277 (5327) 805-808
- 29 Ito N, Rubin GM. gigas, a Drosophila homolog of tuberous sclerosis gene product-2, regulates the cell cycle. Cell 1999; 96 (4) 529-539
- 30 Tapon N, Ito N, Dickson BJ, Treisman JE, Hariharan IK. The Drosophila tuberous sclerosis complex gene homologs restrict cell growth and cell proliferation. Cell 2001; 105 (3) 345-355
- 31 Potter CJ, Huang H, Xu T. Drosophila Tsc1 functions with Tsc2 to antagonize insulin signaling in regulating cell growth, cell proliferation, and organ size. Cell 2001; 105 (3) 357-368
- 32 Potter CJ, Pedraza LG, Xu T. Akt regulates growth by directly phosphorylating Tsc2. Nat Cell Biol 2002; 4 (9) 658-665
- 33 Saucedo LJ, Gao X, Chiarelli DA, Li L, Pan D, Edgar BA. Rheb promotes cell growth as a component of the insulin/TOR signalling network. Nat Cell Biol 2003; 5 (6) 566-571
- 34 Stocker H, Radimerski T, Schindelholz B , et al. Rheb is an essential regulator of S6K in controlling cell growth in Drosophila. Nat Cell Biol 2003; 5 (6) 559-565
- 35 Zhang Y, Gao X, Saucedo LJ, Ru B, Edgar BA, Pan D. Rheb is a direct target of the tuberous sclerosis tumour suppressor proteins. Nat Cell Biol 2003; 5 (6) 578-581
- 36 Goncharova EA, Goncharov DA, Eszterhas A , et al. Tuberin regulates p70 S6 kinase activation and ribosomal protein S6 phosphorylation: a role for the TSC2 tumor suppressor gene in pulmonary lymphangioleiomyomatosis (LAM). J Biol Chem 2002; 277 (34) 30958-30967
- 37 Kenerson H, Dundon TA, Yeung RS. Effects of rapamycin in the Eker rat model of tuberous sclerosis complex. Pediatr Res 2005; 57 (1) 67-75
- 38 Lee L, Sudentas P, Donohue B , et al. Efficacy of a rapamycin analog (CCI-779) and IFN-gamma in tuberous sclerosis mouse models. Genes Chromosomes Cancer 2005; 42 (3) 213-227
- 39 Lee L, Sudentas P, Dabora SL. Combination of a rapamycin analog (CCI-779) and interferon-gamma is more effective than single agents in treating a mouse model of tuberous sclerosis complex. Genes Chromosomes Cancer 2006; 45 (10) 933-944
- 40 Krueger DA, Care MM, Holland K , et al. Everolimus for subependymal giant-cell astrocytomas in tuberous sclerosis. N Engl J Med 2010; 363 (19) 1801-1811
- 41 Bissler JJ, McCormack FX, Young LR , et al. Sirolimus for angiomyolipoma in tuberous sclerosis complex or lymphangioleiomyomatosis. N Engl J Med 2008; 358 (2) 140-151
- 42 Davies DM, de Vries PJ, Johnson SR , et al. Sirolimus therapy for angiomyolipoma in tuberous sclerosis and sporadic lymphangioleiomyomatosis: a phase 2 trial. Clin Cancer Res 2011; 17 (12) 4071-4081
- 43 Dabora SL, Franz DN, Ashwal S , et al. Multicenter phase 2 trial of sirolimus for tuberous sclerosis: kidney angiomyolipomas and other tumors regress and VEGF- D levels decrease. PLoS ONE 2011; 6 (9) e23379
- 44 Lautenbacher R. Dysembriomes metotipiques des reins, carcinose submiliere aigue puomon avec amphyseme generalise et double pneumothorax. Ann Med Interne (Paris) 1918; 5: 435-450
- 45 von Stossel E. Uber muskulare Cirrhose der Lunge (Muscular cirrhosis of the lung). Beitr Klin Tuberk 1937; 90: 432-442
- 46 Travis WD, Colby TV, Corrin B, Shimosato Y, Brambilla E. Histological Typing of Lung and Pleural Tumors. Berlin: Springer; 1999
- 47 Travis WD, Brambilla E, Muller-Hermlink HK, Harris CC. Pathology and Genetics: Tumours of the Lung, Pleura, Thymus and Heart. Lyon: IARC; 2004
- 48 Henske EP. Metastasis of benign tumor cells in tuberous sclerosis complex. Genes Chromosomes Cancer 2003; 38 (4) 376-381
- 49 Corrin B, Butcher D, McAnulty BJ , et al. Immunohistochemical localization of transforming growth factor-beta 1 in the lungs of patients with systemic sclerosis, cryptogenic fibrosing alveolitis and other lung disorders. Histopathology 1994; 24 (2) 145-150
- 50 Kumasaka T, Seyama K, Mitani K , et al. Lymphangiogenesis in lymphangioleiomyomatosis: its implication in the progression of lymphangioleiomyomatosis. Am J Surg Pathol 2004; 28 (8) 1007-1016
- 51 Kumasaka T, Seyama K, Mitani K , et al. Lymphangiogenesis-mediated shedding of LAM cell clusters as a mechanism for dissemination in lymphangioleiomyomatosis. Am J Surg Pathol 2005; 29 (10) 1356-1366
- 52 Hayashi T, Kumasaka T, Mitani K , et al. Prevalence of uterine and adnexal involvement in pulmonary lymphangioleiomyomatosis: a clinicopathologic study of 10 patients. Am J Surg Pathol 2011; 35 (12) 1776-1785
- 53 Yu JJ, Robb VA, Morrison TA , et al. Estrogen promotes the survival and pulmonary metastasis of tuberin-null cells. Proc Natl Acad Sci U S A 2009; 106 (8) 2635-2640
- 54 Parkhitko A, Myachina F, Morrison TA , et al. Tumorigenesis in tuberous sclerosis complex is autophagy and p62/sequestosome 1 (SQSTM1)-dependent. Proc Natl Acad Sci U S A 2011; 108 (30) 12455-12460
- 55 Kumaki F, Takeda K, Yu ZX, Moss J, Ferrans VJ. Expression of human telomerase reverse transcriptase in lymphangioleiomyomatosis. Am J Respir Crit Care Med 2002; 166 (2) 187-191
- 56 Hsu PP, Kang SA, Rameseder J , et al. The mTOR-regulated phosphoproteome reveals a mechanism of mTORC1-mediated inhibition of growth factor signaling. Science 2011; 332 (6035) 1317-1322
- 57 Yu Y, Yoon SO, Poulogiannis G , et al. Phosphoproteomic analysis identifies Grb10 as an mTORC1 substrate that negatively regulates insulin signaling. Science 2011; 332 (6035) 1322-1326
- 58 Soucek T, Rosner M, Miloloza A , et al. Tuberous sclerosis causing mutants of the TSC2 gene product affect proliferation and p27 expression. Oncogene 2001; 20 (35) 4904-4909
- 59 Astrinidis A, Senapedis W, Henske EP. Hamartin, the tuberous sclerosis complex 1 gene product, interacts with polo-like kinase 1 in a phosphorylation-dependent manner. Hum Mol Genet 2006; 15 (2) 287-297
- 60 Mak BC, Kenerson HL, Aicher LD, Barnes EA, Yeung RS. Aberrant beta-catenin signaling in tuberous sclerosis. Am J Pathol 2005; 167 (1) 107-116
- 61 Mak BC, Takemaru K, Kenerson HL, Moon RT, Yeung RS. The tuberin-hamartin complex negatively regulates beta-catenin signaling activity. J Biol Chem 2003; 278 (8) 5947-5951
- 62 Hayashi T, Fleming MV, Stetler-Stevenson WG , et al. Immunohistochemical study of matrix metalloproteinases (MMPs) and their tissue inhibitors (TIMPs) in pulmonary lymphangioleiomyomatosis (LAM). Hum Pathol 1997; 28 (9) 1071-1078
- 63 Fukuda Y, Ishizaki M, Kudoh S, Kitaichi M, Yamanaka N. Localization of matrix metalloproteinases-1, -2, and -9 and tissue inhibitor of metalloproteinase-2 in interstitial lung diseases. Lab Invest 1998; 78 (6) 687-698
- 64 Chilosi M, Pea M, Martignoni G , et al. Cathepsin-k expression in pulmonary lymphangioleiomyomatosis. Mod Pathol 2009; 22 (2) 161-166
- 65 Hayashida M, Seyama K, Inoue Y, Fujimoto K, Kubo K ; Respiratory Failure Research Group of the Japanese Ministry of Health, Labor, and Welfare. The epidemiology of lymphangioleiomyomatosis in Japan: a nationwide cross-sectional study of presenting features and prognostic factors. Respirology 2007; 12 (4) 523-530
- 66 Bense L, Eklund G, Wiman LG. Smoking and the increased risk of contracting spontaneous pneumothorax. Chest 1987; 92 (6) 1009-1012
- 67 Hagaman JT, Schauer DP, McCormack FX, Kinder BW. Screening for lymphangioleiomyomatosis by high-resolution computed tomography in young, nonsmoking women presenting with spontaneous pneumothorax is cost-effective. Am J Respir Crit Care Med 2010; 181 (12) 1376-1382
- 68 McCormack FX, Inoue Y, Moss J , et al; National Institutes of Health Rare Lung Diseases Consortium; MILES Trial Group. Efficacy and safety of sirolimus in lymphangioleiomyomatosis. N Engl J Med 2011; 364 (17) 1595-1606
- 69 Ryu JH, Hartman TE, Torres VE, Decker PA. Frequency of undiagnosed cystic lung disease in patients with sporadic renal angiomyolipomas. Chest 2012; 141 (1) 163-168
- 70 Franz DN, Brody A, Meyer C , et al. Mutational and radiographic analysis of pulmonary disease consistent with lymphangioleiomyomatosis and micronodular pneumocyte hyperplasia in women with tuberous sclerosis. Am J Respir Crit Care Med 2001; 164 (4) 661-668
- 71 Costello LC, Hartman TE, Ryu JH. High frequency of pulmonary lymphangioleiomyomatosis in women with tuberous sclerosis complex. Mayo Clin Proc 2000; 75 (6) 591-594
- 72 Moss J, Avila NA, Barnes PM , et al. Prevalence and clinical characteristics of lymphangioleiomyomatosis (LAM) in patients with tuberous sclerosis complex. Am J Respir Crit Care Med 2001; 164 (4) 669-671
- 73 Roach ES, Gomez MR, Northrup H. Tuberous sclerosis complex consensus conference: revised clinical diagnostic criteria. J Child Neurol 1998; 13 (12) 624-628
- 74 Menko FH, van Steensel MA, Giraud S , et al; European BHD Consortium. Birt-Hogg-Dubé syndrome: diagnosis and management. Lancet Oncol 2009; 10 (12) 1199-1206
- 75 Seaman DM, Meyer CA, Gilman MD, McCormack FX. Diffuse cystic lung disease at high-resolution CT. AJR Am J Roentgenol 2011; 196 (6) 1305-1311
- 76 Young LR, Inoue Y, McCormack FX. Diagnostic potential of serum VEGF-D for lymphangioleiomyomatosis. N Engl J Med 2008; 358 (2) 199-200
- 77 Young LR, Vandyke R, Gulleman PM , et al. Serum vascular endothelial growth factor-D prospectively distinguishes lymphangioleiomyomatosis from other diseases. Chest 2010; 138 (3) 674-681
- 78 Vassallo R, Ryu JH, Colby TV, Hartman T, Limper AH. Pulmonary Langerhans'-cell histiocytosis. N Engl J Med 2000; 342 (26) 1969-1978
- 79 Tobino K, Hirai T, Johkoh T , et al. Differentiation between Birt-Hogg-Dubé syndrome and lymphangioleiomyomatosis: quantitative analysis of pulmonary cysts on computed tomography of the chest in 66 females. Eur J Radiol 2012; 81 (6) 1340-1346
- 80 Tobino K, Gunji Y, Kurihara M , et al. Characteristics of pulmonary cysts in Birt-Hogg-Dubé syndrome: thin-section CT findings of the chest in 12 patients. Eur J Radiol 2011; 77 (3) 403-409
- 81 Kunogi M, Kurihara M, Ikegami TS , et al. Clinical and genetic spectrum of Birt-Hogg-Dube syndrome patients in whom pneumothorax and/or multiple lung cysts are the presenting feature. J Med Genet 2010; 47 (4) 281-287
- 82 Gunji Y, Akiyoshi T, Sato T , et al. Mutations of the Birt Hogg Dube gene in patients with multiple lung cysts and recurrent pneumothorax. J Med Genet 2007; 44 (9) 588-593
- 83 Vortmeyer AO, Lubensky IA, Skarulis M , et al. Multiple endocrine neoplasia type 1: atypical presentation, clinical course, and genetic analysis of multiple tumors. Mod Pathol 1999; 12 (9) 919-924
- 84 Seyama K, Kumasaka T, Souma S , et al. Vascular endothelial growth factor-D is increased in serum of patients with lymphangioleiomyomatosis. Lymphat Res Biol 2006; 4 (3) 143-152
- 85 Glasgow CG, Avila NA, Lin JP, Stylianou MP, Moss J. Serum vascular endothelial growth factor-D levels in patients with lymphangioleiomyomatosis reflect lymphatic involvement. Chest 2009; 135 (5) 1293-1300
- 86 Nagai A, Aoshiba K, Ishihara Y , et al. Administration of alpha 1-proteinase inhibitor ameliorates bleomycin-induced pulmonary fibrosis in hamsters. Am Rev Respir Dis 1992; 145 (3) 651-656
- 87 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 (4) 402-409
- 88 Yamauchi M, Nakahara H, Uyama K, Tsujimoto A, Tamai M, Aozasa K. Cytologic finding of chyloascites in lymphangioleiomyomatosis. A case report. Acta Cytol 2000; 44 (6) 1081-1084
- 89 Valensi QJ. Pulmonary lymphangiomyoma, a probable forme frust of tuberous sclerosis. A case report and survey of the literature. Am Rev Respir Dis 1973; 108 (6) 1411-1415
- 90 Itami M, Teshima S, Asakuma Y, Chino H, Aoyama K, Fukushima N. Pulmonary lymphangiomyomatosis diagnosed by effusion cytology. A case report. Acta Cytol 1997; 41 (2) 522-528
- 91 Berner A, Franzen S, Heilo A. Fine needle aspiration cytology as a diagnostic approach to lymphangioleiomyomatosis. A case report. Acta Cytol 1997; 41 (3) 877-879
- 92 Buhl L, Larsen KE, Bjørn-Hansen L. Lymphangioleiomyomatosis. Is fine needle aspiration cytodiagnosis possible?. Acta Cytol 1988; 32 (4) 559-562
- 93 Ye L, Jin M, Bai C. Clinical analysis of patients with pulmonary lymphangioleiomyomatosis (PLAM) in mainland China. Respir Med 2010; 104 (10) 1521-1526
- 94 Naalsund A, Johansen B, Foerster A, Kolbenstvedt A. When to suspect and how to diagnose pulmonary lymphangioleiomyomatosis. Respirology 1996; 1 (3) 207-212
- 95 Guinee Jr DG, Feuerstein I, Koss MN, Travis WD. Pulmonary lymphangioleiomyomatosis. Diagnosis based on results of transbronchial biopsy and immunohistochemical studies and correlation with high-resolution computed tomography findings. Arch Pathol Lab Med 1994; 118 (8) 846-849
- 96 Delgrange E, Delgrange B, Wallon J, Rosoux P. Diagnostic approach to pulmonary lymphangioleiomyomatosis. J Intern Med 1994; 236 (4) 461-464
- 97 Torre O, Harari S. The diagnosis of cystic lung diseases: a role for bronchoalveolar lavage and transbronchial biopsy?. Respir Med 2010; 104 (Suppl. 01) S81-S85
- 98 Chen F, Bando T, Fukuse T , et al. Recurrent lymphangioleiomyomatosis after living-donor lobar lung transplantation. Transplant Proc 2006; 38 (9) 3151-3153
- 99 Zavala DC. Diagnostic fiberoptic bronchoscopy: techniques and results of biopsy in 600 patients. Chest 1975; 68 (1) 12-19
- 100 Descombes E, Gardiol D, Leuenberger P. Transbronchial lung biopsy: an analysis of 530 cases with reference to the number of samples. Monaldi Arch Chest Dis 1997; 52 (4) 324-329
- 101 Herf SM, Suratt PM, Arora NS. Deaths and complications associated with transbronchial lung biopsy. Am Rev Respir Dis 1977; 115 (4) 708-711
- 102 Simpson FG, Arnold AG, Purvis A, Belfield PW, Muers MF, Cooke NJ. Postal survey of bronchoscopic practice by physicians in the United Kingdom. Thorax 1986; 41 (4) 311-317
- 103 Kreider ME, Hansen-Flaschen J, Ahmad NN , et al. Complications of video-assisted thoracoscopic lung biopsy in patients with interstitial lung disease. Ann Thorac Surg 2007; 83 (3) 1140-1144
- 104 Ryu JH, Moss J, Beck GJ , et al; NHLBI LAM Registry Group. The NHLBI lymphangioleiomyomatosis registry: characteristics of 230 patients at enrollment. Am J Respir Crit Care Med 2006; 173 (1) 105-111
- 105 Ryu JH, Doerr CH, Fisher SD, Olson EJ, Sahn SA. Chylothorax in lymphangioleiomyomatosis. Chest 2003; 123 (2) 623-627
- 106 Boehler A, Speich R, Russi EW, Weder W. Lung transplantation for lymphangioleiomyomatosis. N Engl J Med 1996; 335 (17) 1275-1280
- 107 Corrin B, Liebow AA, Friedman PJ. Pulmonary lymphangiomyomatosis. A review. Am J Pathol 1975; 79 (2) 348-382
- 108 Taveira-DaSilva AM, Hathaway OM, Sachdev V, Shizukuda Y, Birdsall CW, Moss J. Pulmonary artery pressure in lymphangioleiomyomatosis: an echocardiographic study. Chest 2007; 132 (5) 1573-1578
- 109 Cottin V, Harari S, Humbert M , et al. Pulmonary hypertension in lymphangioleiomyomatosis: characteristics in 20 patients. Eur Respir J 2012; Feb 23. [Epub ahead of print]
- 110 Taveira-DaSilva AM, Hathaway O, Stylianou M, Moss J. Changes in lung function and chylous effusions in patients with lymphangioleiomyomatosis treated with sirolimus. Ann Intern Med 2011; 154 (12) 797-805 , W-292–W-293
- 111 Egan JJ, Remund KF, Corris P. Sirolimus for lymphangioleiomyomatosis lesions. N Engl J Med 2008; 358 (18) 1963-1964 , author reply 1964
- 112 Moua T, Olson EJ, St Jean HC, Ryu JH. Resolution of chylous pulmonary congestion and respiratory failure in lam with sirolimus therapy. Am J Respir Crit Care Med 2012; May 3. [Epub ahead of print]
- 113 Neurohr C, Hoffmann AL, Huppmann P , et al. Is sirolimus a therapeutic option for patients with progressive pulmonary lymphangioleiomyomatosis?. Respir Res 2011; 12: 66
- 114 Kuwabara H, Biyazima S, Osaka T , et al. [A case of pulmonary lymphangiomyomatosis diagnosed by TBLB and treated with progesterone and oophorectomy]. Nihon Kyobu Shikkan Gakkai Zasshi 1984; 22 (9) 795-799
- 115 Yamamoto K, Anzai F, Kusajima K, Yamanishi N, Yamada M. Acute respiratory failure with gross hemoptysis in a patient with lymphangioleiomyomatosis as part of tuberous sclerosis complex. Intern Med 2004; 43 (8) 755-758
- 116 Díaz Pedreira JA, González-Carrero J, Cameselle Veloso R, Barros Tizón JC, Bandrés Gimeno R. Pulmonary lymphangioleiomyomatosis: presentation of a new case [in Spanish]. An Med Interna 1991; 8 (12) 614-616
- 117 Chu SC, Horiba K, Usuki J , et al. Comprehensive evaluation of 35 patients with lymphangioleiomyomatosis. Chest 1999; 115 (4) 1041-1052
- 118 Kitaichi M, Nishimura K, Itoh H, Izumi T. Pulmonary lymphangioleiomyomatosis: a report of 46 patients including a clinicopathologic study of prognostic factors. Am J Respir Crit Care Med 1995; 151 (2 Pt 1) 527-533
- 119 Høie J, Jørstad SO, Odegaard A. Pulmonary lymphangioleiomyomatosis [in Norwegian]. Tidsskr Nor Laegeforen 1995; 115 (10) 1230-1232
- 120 Benden C, Rea F, Behr J , et al. Lung transplantation for lymphangioleiomyomatosis: the European experience. J Heart Lung Transplant 2009; 28 (1) 1-7
- 121 Seyama K, Kira S, Takahashi H , et al. Longitudinal follow-up study of 11 patients with pulmonary lymphangioleiomyomatosis: diverse clinical courses of LAM allow some patients to be treated without anti-hormone therapy. Respirology 2001; 6 (4) 331-340
- 122 Lim KE, Tsai YH, Hsu Yl Y, Hsu Wc W. Pulmonary lymphangioleiomyomatosis. High-resolution CT findings in 11 patients and compared with the literature. Clin Imaging 2004; 28 (1) 1-5
- 123 Bonetti F, Chiodera PL, Pea M , et al. Transbronchial biopsy in lymphangiomyomatosis of the lung. HMB45 for diagnosis. Am J Surg Pathol 1993; 17 (11) 1092-1102