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Vol. 47. Issue 2.
Pages 85-93 (January 2011)
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Vol. 47. Issue 2.
Pages 85-93 (January 2011)
Review Article
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Lymphangioleiomyomatosis
Linfangioleiomiomatosis
Visits
5346
Emilio Ansótegui Barreraa, Nuria Mancheño Franchb, Francisco Vera-Sempereb, José Padilla Alarcónc,
Corresponding author
jpadilla@comv.es

Corresponding author.
a Servicio de Neumología, Hospital Universitario La Fe, Valencia, Spain
b Servicio de Anatomía Patológica, Hospital Universitario La Fe, Valencia, Spain
c Servicio de Cirugía Torácica, Instituto Valenciano de Oncología, Valencia, Spain
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Abstract

Lymphangioleiomyomatosis (LAM) is a rare disease that mainly affects women, particularly those of fertile age. Its presentation is sporadic or associated with tuberous sclerosis complex. It is characterized by an abnormal proliferation of immature smooth muscle cells (LAM cells), which grow aberrantly in the airway, parenchyma, lymph nodes and pulmonary blood vessels and can gradually lead to respiratory failure. It affects several systems, affecting the lymphatic ganglia and causing abdominal tumors. Given its very low prevalence, difficulty in establishing early diagnosis, absence of curative treatment and the difficulty in obtaining information, LAM is placed under the heading of the so-called Rare Diseases. There is a growing interest in the study of this disease which has led to the creation of patient registers and an exponential growth in LAM research, both at a clinical and cellular level.

Keywords:
Lymphangioleiomyomatosis
Tuberous sclerosis
Diffuse lung diseases
Resumen

La linfangioleiomiomatosis (LAM) es una enfermedad rara que afecta predominantemente a la mujer, sobre todo en edad fértil. Se presenta de forma esporádica o bien asociada al complejo de esclerosis tuberosa. Se caracteriza por una proliferación anormal de células musculares lisas inmaduras (células LAM), que crecen de manera aberrante en la vía aérea, parénquima, linfáticos y vasos sanguíneos pulmonares, lo que determina una evolución progresiva hacia la insuficiencia respiratoria. Tiene carácter multisistémico, afectando a ganglios linfáticos y produciendo tumores abdominales. Dadas su escasa prevalencia, la dificultad de establecer un diagnóstico precoz, la ausencia de un tratamiento curativo y la dificultad de obtener información encuadran a la LAM dentro del capítulo de las denominadas Enfermedades Raras. Existe un creciente interés en el estudio de esta enfermedad, lo que ha determinado el establecimiento de registros de pacientes y un crecimiento exponencial en la investigación de la LAM, tanto a nivel clínico como celular.

Palabras clave:
Linfangioleiomiomatosis
Esclerosis tuberosa
Neumopatías difusas
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References
[1.]
F.X. McCormack.
Lymphangioleiomyomatosis: a clinical update.
Chest, 133 (2008), pp. 507-516
[2.]
S.R. Johnson, J.F. Cordier, R. Lazor, V. Cottin, U. Costabel, S. Harari, et al.
European respiratory society guidelines for the diagnosis and management of lymphangioleiomyomatosis.
Eur Respir J, 35 (2010), pp. 14-26
[3.]
T. Urban, R. Lazor, J. Lacronique, M. Murris, S. Labrune, D. Valeyre, 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), 78 (1999), pp. 321-337
[4.]
J.H. Ryu, J. Moss, G.J. Beck, J.C. Lee, K.K. Brown, J.T. Chapman, et al.
The NHLBI lymphangioleiomyomatosis registry: characteristics of 230 patients at enrollment.
Am J Respir Crit Care Med, 173 (2006), pp. 105-111
[5.]
E. Antón, A. Casanova, A. Xaubet, A. Román, V. Villena, M.C. Montero, et al.
Lymphangioleiomyomatosis: a study of 72 patients from the Spanish registry.
Sarcoidosis Vasc Diffuse Lung Dis, 26 (2009), pp. 85-91
[6.]
G. Finlay.
The LAM cell: what is it, where does it come from, and why does it grow?.
Am J Physiol Lung Cell Mol Physiol, 286 (2004), pp. L690-L693
[7.]
S.C. Juvet, F.X. McCormack, D.J. Kwiatkowski, G.P. Downey.
Molecular pathogenesis of lymphangioleiomyomatosis: lessons learned from orphans.
Am J Respir Cell Mol Biol, 36 (2007), pp. 398-408
[8.]
V.P. Krymskaya.
Smooth muscle-like cells in pulmonary lymphangioleiomyomatosis.
Proc Am Thorac Soc, 5 (2008), pp. 119-126
[9.]
J. Moss, N.A. Avila, P.M. Barnes, R.A. Litzenberger, J. Bechtle, P.G. Brooks, et al.
Prevalence and clinical characteristics of lymphangioleiomyomatosis (LAM) in patients with tuberous sclerosis complex.
Am J Respir Crit Care Med, 164 (2001), pp. 669-671
[10.]
D.N. Franz, A. Brody, C. Meyer, J. Leonard, G. Chuck, S. Dabora, 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, 164 (2001), pp. 661-668
[11.]
M. Gómez.
Phenotypes of the tuberous sclerosis complex with a revision of diagnostic criteria.
Ann N Y Acad Sci, 615 (1991), pp. 1-7
[12.]
E.S. Roach, F.J. DiMario, R.S. Kand, H. Northrup.
Tuberous sclerosis consensus conference: recommendations for diagnosis evaluation.
J Child Neurol, 14 (1999), pp. 402-407
[13.]
T.A. Smolarek, L.L. Wessner, F.X. McCormack, J.C. Mylet, A.G. Menon, E.P. Henske.
Evidence that lymphangiomyomatosis is caused by TSC2 mutations: chromosome 16p13 loss of heterozygosity in angiomyolipomas and lymph nodes from women with lymphangiomyomatosis.
Am J Hum Genet, 62 (1998), pp. 810-815
[14.]
T. Carsillo, A. Astrinidis, E.P. Henske.
Mutations in the tuberous sclerosis complex gene TSC2 are a cause of sporadic pulmonary lymphangioleiomyomatosis.
Proc Natl Acad Sci U S A, 97 (2000), pp. 6085-6090
[15.]
A. Astrinidis, E.P. Henske.
Tuberous sclerosis complex: linking growth and energy signaling pathways with human disease.
Oncogene, 24 (2005), pp. 7475-7481
[16.]
T.J. Matthews, D. Hornall, M.N. Sheppard.
Comparison of the use of antibodies to alpha smooth muscle actin and desmin in pulmonary lymphangioleiomyomatosis.
J Clin Pathol, 46 (1993), pp. 479-480
[17.]
F. Bonetti, P.L. Chiodera, M. Pea, G. Martignoni, F. Bosi, G. Zamboni, et al.
Transbronchial biopsy in lymphangiomyomatosis of the lung. HMB45 for diagnosis.
Am J Surg Pathol, 17 (1993), pp. 1092-1102
[18.]
V. Hoon, S.N. Thung, M. Kaneko, P.D. Unger.
HMB-45 reactivity in renal angiomyolipoma and lymphangioleiomyomatosis.
Arch Pathol Lab Med, 118 (1994), pp. 732-734
[19.]
K. Matsui, K. Takeda, Z.X. Yu, J. Valencia, W.D. Travis, J. Moss, 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, 161 (2000), pp. 1002-1009
[20.]
X. Zhe, L. Schuger.
Combined smooth muscle and melanocytic differentiation in lymphangioleiomyomatosis.
J Histochem Cytochem, 52 (2004), pp. 1537-1542
[21.]
F. Bonetti, M. Pea, G. Martignoni, G. Zamboni, P. Iuzziolino.
Cellular heterogeneity in lymphangiomyomatosis.
Hum Pathol, 22 (1991), pp. 727-728
[22.]
Y. Matsumoto, K. Horiba, J. Usuki, S.C. Chu, V.J. Ferrans, J. Moss.
Markers of cell proliferation and expression of melanosomal antigen in lymphangioleiomyomatosis.
Am J Respir Cell Mol Biol, 21 (1999), pp. 327-336
[23.]
M.M. Brentani, C.R. Carvalho, P.H. Saldiva, M.M. Pacheco, C.T. Oshima.
Steroid receptors in pulmonary lymphangiomyomatosis.
Chest, 85 (1984), pp. 96-99
[24.]
E.A. Goncharova, D.A. Goncharov, P.N. Lim, D. Noonan, V.P. Krymskaya.
Modulation of cell migration and invasiveness by tumor suppressor TSC2 in lymphangioleiomyomatosis.
Am J Respir Cell Mol Biol, 34 (2006), pp. 473-480
[25.]
D.M. Crooks, G. Pacheco-Rodriguez, R.M. DeCastro, J.P. McCoy Jr, J.A. Wang, F. Kumaki, et al.
Molecular and genetic analysis of disseminated neoplastic cells in lymphangioleiomyomatosis.
Proc Natl Acad Sci U S A, 101 (2004), pp. 17462-17467
[26.]
J.R. Sampson, S.J. Scahill, J.B. Stephenson, L. Mann, J. Connor.
Genetic aspects of tuberous sclerosis in the west of Scotland.
J Med Genet, 26 (1989), pp. 28-31
[27.]
The European Chromosome 16 Tuberous Sclerosis Consortium.
Identification and characterization of the tuberous sclerosis gene on chromosome 16.
Cell, 75 (1993), pp. 1305-1315
[28.]
M. Van Slegtenhorst, R. de Hoogt, C. Hermans, M. Nellist, B. Janssen, S. Verhoef, et al.
Identification of the tuberous sclerosis gene TSC1 on chromosome 9q34.
Science, 277 (1997), pp. 805-808
[29.]
A. Knudson.
Mutation and cancer: statistical study of retinoblastoma.
Proc Natl Acad Sci USA, 68 (1971), pp. 820-823
[30.]
C. Carbonara, L. Longa, E. Grosso, C. Borrone, M.G. Garra, M. Brisigotti, et al.
9q34 loss of heterozygosity in a tuberous sclerosis astrocytoma suggests a growth suppressor-like activity also for the TSC1 gene.
Hum Mol Genet, 3 (1994), pp. 1829-1832
[31.]
E.P. Henske, H.P.H. Neumann, B.W. Scheithauer, E.W. Herbst, M.P. Short, D.J. Kwiatkowski.
Loss of heterozygosity in the tuberous sclerosis (TSC2) region of chromosome band 16p13 occurs in sporadic as well as TSC-associated renal angiomyolipomas.
Genes Chromosomes and Cancer, 13 (1995), pp. 295-298
[32.]
D.J. Kwiatkowski.
Tuberous sclerosis: from tuber to mTOR.
Ann Hum Genet, 67 (2003), pp. 87-96
[33.]
A. Astrinidis, L. Khare, T. Carsillo, T. Smolarek, K.S. Au, H. Northrup, et al.
Mutational analysis of the tuberous sclerosis gene TSC2 in patients with pulmonary lymphangioleiomyomatosis.
J Med Genet, 37 (2000), pp. 55-57
[34.]
O. Sancak, M. Nellist, M. Goedbloed, P. Elfferich, C. Wouters, A. Maat-Kievit, et al.
Mutational analysis of the TSC1 and TSC2 genes in a diagnostic setting: genotype-phenotype correlations and comparison of diagnostic DNA techniques in tuberous sclerosis complex.
Eur J Hum Genet, 13 (2005), pp. 731-741
[35.]
T. Schmelzle, M.N. Hall.
TOR, a central controller of cell growth.
Cell, 103 (2000), pp. 253-262
[36.]
A. Garami, F.J.T. Zwartkruis, T. Nobukuni, M. Joaquin, M. Roccio, H. Stocker, et al.
Insulin activation of Rheb, a mediator of mTOR/S6K/4E-BP signaling, is inhibited by TSC1 and 2.
Mol Cell, 11 (2003), pp. 1457-1466
[37.]
K. Inoki, T. Zhu, K.L. Guan.
TSC2 mediates cellular energy response to control cell growth and survival.
Cell, 115 (2003), pp. 577-590
[38.]
A.R. Tee, B.D. Manning, P.P. Roux, L.C. Cantley, J. Blenis.
Tuberous sclerosis complex gene products, Tuberin and Hamartin, control mTOR signaling by acting as a GTPase-activating protein complex toward Rheb.
Curr Biol, 13 (2003), pp. 1259-1268
[39.]
K. Inoki, Y.W. Li, T. Zhu, J. Wu, K.L. Guan.
TSC2 is phosphorylated and inhibited by Akt and supresses mTOR signalling.
Nat Cell Biol, 4 (2002), pp. 657-684
[40.]
N.C. Barbet, U. Scheiner, S.B. Helliwell, I. Stanfield, M.F. Tuite, M.N. Hall.
TOR controls translation initiation and early G1 progression in yeast.
Mol Biol Cell, 7 (1996), pp. 25-42
[41.]
S.N. Sehgal.
Rapamune (Sirolimus, rapamycine): an overview and mechanism of action.
Ther Drug Monit, 17 (1995), pp. 660-665
[42.]
E. Lesma, V. Grande, S. Carelli, D. Brancaccio, M.P. Canevini, R.M. Alfano, et al.
Isolation and growth of smooth muscle-like cells derived from tuberous sclerosis complex-2 human renal angiomyolipoma: epidermal growth factor is the required growth factor.
Am J Pathol, 167 (2005), pp. 1093-1103
[43.]
H. Watz, K. Engels, S. Loeschke, M. Amthor, D. Kirsten, H. Magnussen.
Lymphangioleiomyomatosis-presence of receptor tyrosine kinases and the angiogenesis factor VEGF-A as potential therapeutic targets.
[44.]
O.J. Shah, Z. Wang, T. Hunter.
Inappropriate activation of the TSC/Rheb/mTOR/S6K cassette induces IRS1/2 depletion, insulin resistance, and cell survival deficiencies.
Curr Biol, 14 (2004), pp. 1650-1656
[45.]
L.S. Harrington, G.M. Findlay, A. Gray, Tolkacheva, S. Wigfield, H. Rebholz, et al.
The TSC1-2 tumor suppressor controls insulin-PI3K signaling via regulation of IRS proteins.
J Cell Biol, 166 (2004), pp. 213-223
[46.]
L. Ma, Z. Chen, H. Erdjument-Bromage, P. Tempst, P.P. Pandolfi.
Phosphorylation and functional inactivation of TSC2 by Erk: Implications for tuberous sclerosis and cancer pathogenesis.
[47.]
K. Inoki, T. Zhu, K.L. Guan.
TSC2 mediates cellular energy response to control cell growth and survival.
Cell, 115 (2003), pp. 577-590
[48.]
N. El-Hashemite, D.J. Kwiatkowski.
Interferon-gamma-Jak-Stat signaling in pulmonary lymphangioleiomyomatosis and renal angiomyolipoma: a potential therapeutic target.
Am J Respir Cell Mol Biol, 33 (2005), pp. 227-230
[49.]
L. Lee, P. Sudentas, S.L. Dabora.
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, 45 (2006), pp. 933-944
[50.]
D.J. Noonan, D. Lou, N. Griffith, T.C. Vanaman.
A calmodulin binding site in the tuberous sclerosis 2 gene product is essential for regulation of transcription events and is altered by mutations linked to tuberous sclerosis and lymphangioleiomyomatosis.
Arch Biochem Biophys, 398 (2002), pp. 132-140
[51.]
J. Yu, A. Astrinidis, S. Howard, E.P. Henske.
Estradiol and tamoxifen stimulate LAM-associated angiomyolipoma cell growth and activate both genomic and nongenomic signaling pathways.
Am J Physiol Lung Cell Mol Physiol, 286 (2004), pp. L694-L700
[52.]
B. York, D. Lou, R.A. Panettieri Jr, V.P. Krymskaya, T.C. Vanaman, D.J. Noonan.
Crosstalk between tuberin, calmodulin, and estrogen signaling pathways.
FASEB J, 19 (2005), pp. 1202-1204
[53.]
T. Simoncini, A. Hafezi-Moghadam, D.P. Brazil, K. Ley, W.W. Chin, J.K. Llao.
Interaction of oestrogen receptor with the regulatory subunit of phosphatidylinositol-3-OH kinase.
Nature, 407 (2000), pp. 538-541
[54.]
G. Flores-Delgado, K.D. Anderson, D. Warburton.
Nongenomic estrogen action regulates tyrosine phosphatase activity and tuberin stability.
Mol Cell Endocrinol, 199 (2003), pp. 143-151
[55.]
A. Astrinidis, T.P. Cash, D.S. Hunter, C.L. Walker, J. Chernoff, E.P. Henske.
Tuberin, the tuberous sclerosis complex 2 tumor suppressor gene product, regulates Rho activation, cell adhesion and migration.
Oncogene, 21 (2002), pp. 8470-8476
[56.]
R.C. Ji.
Lymphatic endothelial cells, lymphangiogenesis, and extracellular matrix.
Lymphat Res Biol, 4 (2006), pp. 83-100
[57.]
H.C. Crawford, L.M. Matrisian.
Tumor and stromal expression of matrix metalloproteinases and their role in tumor progression.
Invasion Metastasis, 14 (1994), pp. 234-245
[58.]
T. Hayashi, M.V. Fleming, W.G. Stetler-Stevenson, L.A. Liotta, J. Moss, V.J. Ferrans, et al.
Immunohistochemical study of matrix metalloproteinases (MMPs) and their tissue inhibitors (TIMPs) in pulmonary lymphangioleiomyomatosis (LAM).
Hum Pathol, 28 (1997), pp. 1071-1078
[59.]
K. Matsui, K. Takeda, Z.X. Yu, W.D. Travis, J. Moss, V.J. Ferrans.
Role for activation of matrix metalloproteinases in the pathogenesis of pulmonary lymphangioleiomyomatosis.
Arch Pathol Lab Med, 124 (2000), pp. 267-275
[60.]
J. Yu, A. Astrinidis, E.P. Henske.
Chromosome 16 loss of heterozygosity in tuberous sclerosis and sporadic lymphangiomyomatosis.
Am J Respir Crit Care Med, 164 (2001), pp. 1537-1540
[61.]
V.P. Krymskaya, J.M. Shipley.
Lymphangioleiomyomatosis: a complex tale of serum response factor-mediated tissue inhibitor of metalloproteinase-3 regulation.
Am J Respir Cell Mol Biol, 28 (2003), pp. 546-550
[62.]
X. Zhe, Y. Yang, S. Jakkaraju, L. Schuger.
Tissue inhibitor of metalloproteinase-3 downregulation in lymphangioleiomyomatosis: potential consequence of abnormal serum response factor expression.
Am J Respir Cell Mol Biol, 28 (2003), pp. 504-511
[63.]
T. Sato, K. Seyama, H. Fujii, H. Maruyama, Y. Setoguchi, S. Iwakami, et al.
Mutation analysis of the TSC1 and TSC2 genes in Japanese patients with pulmonary lymphangioleiomyomatosis.
J Hum Genet, 47 (2002), pp. 20-28
[64.]
C. Franco, B. Ho, D. Mulholland, G. Hou, M. Islam, K. Donaldson, et al.
Doxycycline alters vascular smooth muscle cell adhesion, migration, and reorganization of fibrillar collagen matrices.
Am J Pathol, 168 (2006), pp. 1697-1709
[65.]
M.A. Moses, J. Harper, J. Folkman.
Doxycycline treatment for lymphangioleiomyomatosis with urinary monitoring for MMPs.
N Engl J Med, 354 (2006), pp. 2621-2622
[66.]
T. Kumasaka, K. Seyama, K. Mitani, S. Souma, S. Kashiwagi, A. Hebisawa, et al.
Lymphangiogenesis-mediated shedding of LAM cell clusters as a mechanism for dissemination in lymphangioleiomyomatosis.
Am J Surg Pathol, 29 (2005), pp. 1356-1366
[67.]
K. Seyama, T. Kumasaka, S. Souma, T. Sato, M. Kurihara, K. Mitani, et al.
Vascular endothelial growth factor-D is increased in serum of patients with lymphangioleiomyomatosis.
Lymphat Res Biol, 4 (2006), pp. 143-152
[68.]
W. Travis, J. Usuki, K. Horiba, V.J. Ferrans.
Histopathologic studies on lymphangioleiomyomatosis.
LAM and other diseases characterized by smooth muscle proliferation, pp. 171-217
[69.]
V.J. Ferrans, Z.X. Yu, W.K. Nelson, J.C. Valencia, A. Tatsuguchi, N.A. Avila, et al.
Lymphangioleiomyomatosis (LAM): a review of clinical and morphological features.
J Nippon Med Sch, 67 (2000), pp. 311-329
[70.]
B. Corrin, A.A. Liebow, P.J. Friedman.
Pulmonary lymphangioleiomyomatrosis.
Am J Pathol, 79 (1975), pp. 348-382
[71.]
E.J. Sullivan.
Lymphangioleiomyomatosis: a review.
Chest, 114 (1998), pp. 1689-1703
[72.]
K. Matsui, M.B. Beasley, W.K. Nelson, P.M. Barnes, J. Bechtle, R. Falk, et al.
Prognostic significance of pulmonary lymphangioleiomyomatosis histologic score.
Am J Surg Pathol, 25 (2001), pp. 479-484
[73.]
D. Guinee, R. Singh, N. Azumi, G. Singh, R.M. Przygodzki, W. Travis, et al.
Multifocal micronodular pneumocyte hyperplasia: a distinctive pulmonary manifestation of tuberous sclerosis.
Mod Pathol, 8 (1995), pp. 902-906
[74.]
D.B. Flieder, W.D. Travis.
Clear cell “sugar” tumor of the lung: association with lymphangioleiomyomatosis and multifocal micronodular pneumocyte hyperplasia in a patient with tuberous sclerosis.
Am J Surg Pathol, 21 (1997), pp. 1242-1247
[75.]
D.W. Hohman, D. Noghrehkar, S. Ratnayake.
Lymphangioleiomyomatosis: A review.
Eur J Intern Med, 19 (2008), pp. 319-324
[76.]
L.C. Costello, T.E. Hartman, J.H. Ryu.
High frequency of pulmonary lymphangioleiomyomatosis in women with tuberous sclerosis complex.
Mayo Clin Proc, 75 (2000), pp. 591-594
[77.]
A. Román, D. Aristizábal, E. Pallisa, J. Majó, M. Iscar, V. Monforte, et al.
Linfangioleiomiomatosis: estudio de 15 pacientes.
Med Clin (Barc), 115 (2000), pp. 98-102
[78.]
S.R. Johnson, A.E. Tattersfield.
Clinical experience of lymphangioleiomyomatosis in the UK.
Thorax, 55 (2000), pp. 1052-1057
[79.]
M.C. Aubry, J.L. Myers, J.H. Ryu, E.P. Henske, H. Logginidou, S.M. Jalal, et al.
Pulmonary lymphangioleiomyomatosis in a man.
Am J Respir Crit Care Med, 162 (2000), pp. 749-752
[80.]
N.R. Kim, M.P. Chung, C.K. Park, K.S. Lee, J. Han.
Pulmonary lymphangioleiomyomatosis and multiple hepatic angiomyolipomas in a man.
Pathol Int, 53 (2003), pp. 231-235
[81.]
M. Miyake, U. Tateishi, T. Maeda, M. Kusumoto, M. Satake, Y. Arai, et al.
Pulmonary lymphangioleiomyomatosis in a male patient with tuberous sclerosis complex.
Radiat Med, 23 (2005), pp. 525-527
[82.]
M. Schiavina, V. Di Scioscio, P. Contini, A. Cavazza, A. Fabiani, M. Barberis, et al.
Pulmonary lymphangioleiomyomatosis in a karyotypically normal man without tuberous sclerosis complex.
Am J Respir Crit Care Med, 176 (2007), pp. 96-98
[83.]
F.X. McCormack, J. Moss.
S-LAM in a man?.
Am J Respir Crit Care Med, 176 (2007), pp. 3-5
[84.]
A. Sandrini, A. Krishnan, D.H. Yates.
S-LAM in a man: the first case report.
Am J Respir Crit Care Med, 177 (2008), pp. 356
[85.]
A.M. Taveira-DaSilva, W.K. Steagall, J. Moss.
Lymphangioleiomyomatosis.
Cancer Control, 13 (2006), pp. 276-285
[86.]
S.R. Johnson.
Lymphangioleiomyomatosis.
Eur Respir J, 27 (2006), pp. 1056-1065
[87.]
A.M. Taveira-DaSilva, M.P. Stylianou, C.J. Hedin, O. Hathaway, J. Moss.
Decline in lung function in patients with lymphangioleiomyomatosis treated with or without progesterone.
Chest, 126 (2004), pp. 1867-1874
[88.]
K.F. Almoosa, F.X. McCormack, S.A. Sahn.
Pleural disease in lymphangioleiomyomatosis.
Clin Chest Med, 27 (2006), pp. 355-368
[89.]
J.R. Taylor, J. Ryu, T.V. Colby, T.A. Raffin.
Lymphangioleiomyomatosis. Clinical course in 32 patients.
N Engl J Med, 323 (1990), pp. 1254-1260
[90.]
M. Kitaichi, K. Nishimura, H. Itoh, T. Izumi.
Pulmonary lymphangioleiomyomatosis: a report of 46 patients including a clinicopathologic study of prognostic factors.
Am J Respir Crit Care Med, 151 (1995), pp. 527-533
[91.]
Y.M. Oh, E.K. Mo, S.H. Jang, C.G. Yoo, Y.W. Kim, J.W. Seo, et al.
Pulmonary lymphangioleiomyomatosis in Korea.
Thorax, 54 (1999), pp. 618-621
[92.]
S.C. Chu, K. Horiba, J. Usuki, N.A. Avila, C.C. Chen, W.D. Travis, et al.
Comprehensive evaluation of 35 patients with lymphangioleiomyomatosis.
Chest, 115 (1999), pp. 1041-1052
[93.]
I. Wahedna, S. Cooper, J. Williams, I.C. Paterson, J.R. Britton, A.E. Tattersfield.
Relation of pulmonary lymphangio-leiomyomatosis to use of the oral contraceptive pill and fertility in the UK: a national case control study.
Thorax, 49 (1994), pp. 910-914
[94.]
L.R. Young, K.F. Almoosa, S. Pollock-Barziv, M. Coutinho, F.X. McCormack, S.A. Sahn.
Patient perspectives on management of pneumothorax in lymphangioleiomyomatosis.
Chest, 129 (2006), pp. 1267-1273
[95.]
K.F. Almoosa, J.H. Ryu, J. Mendez, J.T. Huggins, L.R. Young, E.J. Sullivan, et al.
Management of pneumothorax in lymphangioleiomyomatosis: effects on recurrence and lung transplantation complications.
Chest, 129 (2006), pp. 1274-1281
[96.]
V.G. Valentine, T.A. Raffin.
The management of chylothorax.
Chest, 102 (1992), pp. 586-591
[97.]
W. Ramos, J. Frainchut.
Nutritional management of thoracic duct fistulas: a comparative study of parenteral versus enteral nutrition.
J Parenter Enteral Nutr, 10 (1986), pp. 519-521
[98.]
I. Kalomenidis.
Octreotide chylothorax.
Curr Opin Pulm Med, 12 (2006), pp. 264-267
[99.]
H.C. Lu, J. Wang, Y.M. Tsang, M.C. Lin, Y.W. Li.
Lymphangioleiomyomatosis initially presenting with abdominal pain: a case report.
Clin Imaging, 27 (2003), pp. 166-170
[100.]
Y.Y. Wong, T.K. Yeung, W.C. Chu.
Atypical presentation of lymphangioleiomyomatosis as acute abdomen: CT diagnosis.
Am J Roentgenol, 181 (2003), pp. 284-285
[101.]
N.A. Avila, J.A. Kelly, S.C. Chu, A.J. Dwyer, J. Moss.
Lymphangioleiomyomatosis: abdominopelvic CT and US findings.
[102.]
N.A. Avila, J. Bechtle, A.J. Dwyer, V.J. Ferrans, J. Moss.
Lymphangioleiomyomatosis: CT of diurnal variation of lymphangioleiomyomas.
Radiology, 221 (2001), pp. 415-421
[103.]
S.M. Bernstein, J.D. Newell Jr, D. Adamczyk, R.L. Mortenson, T.E. King Jr, D.A. Lynch.
How common are renal angiomyolipomas in patients with pulmonary lymphangiomyomatosis?.
Am J Respir Crit Care Med, 152 (1995), pp. 2138-2143
[104.]
D.E. Maziak, S. Kesten, D.C. Rappaport, J. Maurer.
Extrathoracic angiomyolipomas in lymphangioleiomyomatosis.
Eur Respir J, 9 (1996), pp. 402-405
[105.]
N.A. Avila, A.J. Dwyer, A. Rabel, J. Moss.
Sporadic lymphangioleiomyomatosis and tuberous sclerosis complex with lymphangioleiomyomatosis: comparison of CT features.
Radiology, 242 (2007), pp. 277-285
[106.]
K. Yamakado, N. Tanaka, T. Nakagawa, S. Kobayashi, M. Yanagawa, K. Takeda.
Renal angiomyolipoma: Relationships between tumor size, aneurysm formation, and rupture.
Radiology, 225 (2002), pp. 78-82
[107.]
J. Moss, R. DeCastro, N.J. Patronas, A. Taveira-DaSilva.
Meningiomas in lymphangioleiomyomatosis.
JAMA, 286 (2001), pp. 1879-1881
[108.]
R. Mascarenhas, P. McLaughlin.
Haemorrhage from angiomyolipoma of kidney during pregnancy - A diagnostic dilemma.
Irish Med J, 94 (2001), pp. 84-85
[109.]
J.L. Berger.
X-ray of the month. Pulmonary lymphangioleiomyomatosis.
J Tenn Med Assoc, 73 (1980), pp. 657-658
[110.]
D.C. Rappaport, G.L. Weisbrod, S.J. Herman, D.W. Chamberlain.
Pulmonary lymphangioleiomyomatosis: high-resolution CT findings in four cases.
Am J Roentgenol, 152 (1989), pp. 961-964
[111.]
P.A. Templeton, T.C. McLoud, N.L. Muller, J.A. Shepard, E.H. Moore.
Pulmonary lymphangioleiomyomatosis: CT and pathologic findings.
J Comput Assist Tomogr, 13 (1989), pp. 54-57
[112.]
W.K. Steagall, C.G. Glasgow, O.M. Hathaway, N.A. Avila, A.M. Taveira-Dasilva, A. Rabel, et al.
Genetic and morphologic determinants of pneumothorax in lymphangioleiomyomatosis.
Am J Physiol Lung Cell Mol Physiol, 293 (2007), pp. L800-L808
[113.]
A.M. Taveira-DaSilva, C. Hedin, M.P. Stylianou, W.D. Travis, K. Matsui, V.J. Ferrans, et al.
Reversible airflow obstruction, proliferation of abnormal smooth muscle cells, and impairment of gas exchange as predictors of outcome in lymphangioleiomyomatosis.
Am J Respir Crit Care Med, 164 (2001), pp. 1072-1076
[114.]
A.M. Taveira-DaSilva, M.P. Stylianou, C.J. Hedin, A.S. Kristof, N.A. Avila, A. Rabel, et al.
Maximal oxygen uptake and severity of disease in lymphangioleiomyomatosis.
Am J Respir Crit Care Med, 168 (2003), pp. 1427-1431
[115.]
R.S. Crausman, C.A. Jennings, R.L. Mortenson, L.M. Ackerson, C.G. Irvin, T.E. King Jr.
Lymphangioleiomyomatosis: the pathophysiology of diminished exercise capacity.
Am J Respir Crit Care Med, 153 (1996), pp. 1368-1376
[116.]
A.M. Taveira-DaSilva, O.M. Hathaway, V. Sachdev, Y. Shizukuda, C.W. Birdsall, J. Moss.
Pulmonary artery pressure in lymphangioleiomyomatosis: an echocardiographic study.
Chest, 132 (2007), pp. 1573-1578
[117.]
M. Koyama, T. Johkoh, O. Honda, M. Tsubamoto, T. Kozuka, N. Tomiyama, et al.
Chronic cystic lung disease: diagnostic accuracy of high-resolution CT in 92 patients.
Am J Roentgenol, 180 (2003), pp. 827-835
[118.]
Y.J. Jeong, K.S. Lee, M.P. Chung, J. Han, M.J. Chung, K.I. Kim, et al.
Amyloidosis and Lymphoproliferative disease in Sjögren syndrome: Thin-section computed tomography findings and histopathologic comparisons.
J Com Assist Tomog, 28 (2004), pp. 776-781
[119.]
T. Johkoh, N.L. Müller, H.A. Pickford, T.E. Hartman, K. Ichikado, M. Akira, et al.
Lymphocytic interstitial pneumonia: Thin-section CT findings in 22 patients.
[120.]
C.I. Silva, A. Churg, N.L. Müller.
Hypersensitivity pneumonitis: Spectrum of highresolution CT and pathologic findings.
Am J Roentgenol, 188 (2007), pp. 334-344
[121.]
M. Colombat, M. Stern, O. Groussard, D. Droz, M. Brauner, D. Valeyre, et al.
Pulmonary cystic disorder related to light chain deposition disease.
Am J Respir Crit Care Med, 173 (2006), pp. 777-780
[122.]
M.C. Aubry, J.L. Myers, T.V. Colby, K.O. Leslie, H.D. Tazelaar.
Endometrial stromal sarcoma metastatic to the lung: a detailed analysis of 16 patients.
Am J Surg Pathol, 26 (2002), pp. 440-449
[123.]
D.S. Ayo, G.L. Aughenbaugh, E.S. Yi, J.L. Hand, J.H. Ryu.
Cystic lung disease in Birt-Hogg-Dubé syndrome.
Chest, 132 (2007), pp. 679-684
[124.]
J.L. Faul, G.J. Berry, T.V. Colby, S.J. Ruoss, M.B. Walter, G.D. Rosen, et al.
Thoracic lymphangiomas, lymphangiectasis, lymphangiomatosis, and lymphatic dysplasia syndrome.
Am J Respir Crit Care Med, 161 (2000), pp. 1037-1046
[125.]
S.R. Johnson, C.I. Whale, R.B. Hubbard, S.A. Lewis, A.E. Tattersfield.
Survival and disease progression in UK patients with lymphangioleiomyomatosis.
Thorax, 59 (2004), pp. 800-803
[126.]
T.B. Ho, J.H. Hull, N.C. Hughes.
An 86-year-old female with lymphangioleiomyomatosis.
Eur Respir J, 28 (2006), pp. 1065
[127.]
S.R. Johnson, A.E. Tattersfield.
Decline in lung function in lymphangioleiomyomatosis: relation to menopause and progesterone treatment.
Am J Respir Crit Care Med, 160 (1999), pp. 628-633
[128.]
R. Lazor, D. Lauque, P. Deleval, J. Lacronique, T. Urban, J.F. Cordier.
Predictors of rapid decline of FEV1 in 50 cases of pulmonary lymphangioleiomyomatosis followed for >1 year.
Am J Respir Crit Care Med, 161 (2000), pp. A15
[129.]
M. Hayashida, K. Seyama, Y. Inoue, K. Fujimoto, K. Kubo.
The epidemiology of lymphangioleiomyomatosis in Japan: a nationwide cross-sectional study of presenting features and prognostic factors.
Respirology, 12 (2007), pp. 523-530
[130.]
R.S. Crausman, D.A. Lynch, R.L. Mortenson, T.E. King Jr, C.G. Irvin, V.A. Hale, et al.
Quantitative CT predicts the severity of physiologic dysfunction in patients with lymphangioleiomyomatosis.
Chest, 109 (1996), pp. 131-137
[131.]
J. De la Fuente, C. Paramo, F. Roman, R. Perez, C. Masa, J.M. De Letona.
Lymphangioleiomyomatosis: unsuccessful treatment with luteinizing-hormonereleasing hormone analogues.
Eur J Med, 2 (1993), pp. 377-378
[132.]
A.H. Eliasson, Y.Y. Phillips, M.F. Tenholder.
Treatment of lymphangioleiomyomatosis.
A meta-analysis Chest, 96 (1989), pp. 1352-1355
[133.]
M. Klein, O. Krieger, R. Ruckser, A. Rosen, R. Waldner, P. Preis, et al.
Treatment of lymphangioleiomyomatosis by ovariectomy, interferon alpha 2b and tamoxifen-a case report.
Arch Gynecol Obstet, 252 (1992), pp. 99-102
[134.]
J.J. Bissler, F.X. McCormack, L.R. Young, J.M. Elwing, G. Chuck, J.M. Leonard, et al.
Sirolimus for angiomyolipoma in tuberous sclerosis complex or lymphangioleiomyomatosis.
N Engl J Med, 358 (2008), pp. 140-151
[135.]
E. Paul, E. Thiele.
Efficacy of sirolimus in treating tuberous sclerosis and lymphangioleiomyomatosis.
N Engl J Med, 358 (2008), pp. 190-192
[136.]
J.J. Egan, K.F. Remund, P. Corris.
Sirolimus for lymphangioleiomyomatosis lesions.
N Engl J Med, 358 (2008), pp. 1963-1964
[137.]
J.R. Sampson.
Therapeutic targeting of mTOR in tuberous sclerosis.
Biochem Soc Trans, 37 (2009), pp. 259-264
[138.]
J.B. Orens, M. Estenne, S. Arcasoy, J.V. Conte, P. Corris, J.J. Egan, et al.
International guidelines for the selection of lung transplant candidates: 2006 update-a consensus report from the Pulmonary Scientific Council of the International Society for Heart and Lung Transplantation.
J Heart Lung Transplant, 25 (2006), pp. 745-755
[139.]
J.D. Christie, L.B. Edwards, P. Aurora, F. Dobbels, R. Kirk, A.O. Rahmel, et al.
The registry of the international society for heart and lung transplantation: twenty-sixth official adult lung and heart-lung transplantation Report-2009.
J Heart Lung Transplant, 28 (2009), pp. 1031-1049
[140.]
J.S. Nine, S.A. Yousem, I.L. Paradis, R. Keenan, B.P. Griffith.
Lymphangioleiomyomatosis: recurrence after lung transplantation.
J Heart Lung Transplant, 13 (1994), pp. 714-719
[141.]
I. Bittmann, B. Rolf, G. Amann, U. Lohrs.
Recurrence of lymphangioleiomyomatosis after single lung transplantation: new insights into pathogenesis.
Hum Pathol, 34 (2003), pp. 95-98
[142.]
T.T. Pechet, B.F. Meyers, T.J. Guthrie, R.J. Battafarano, E.P. Trulock, J.D. Cooper, et al.
Lung transplantation for lymphangioleiomyomatosis.
J Heart Lung Transplant, 23 (2004), pp. 301-308
[143.]
M. Reynaud-Gaubert, J.F. Mornex, H. Mal, M. Treilhaud, C. Dromer, S. Quetant, et al.
Lung transplantation for lymphangioleiomyomatosis: the French experience.
Transplantation, 86 (2008), pp. 515-520
[144.]
J. Kpodonu, M.G. Massad, R.A. Chaer, A. Caines, A. Evans, N.J. Snow, et al.
The US experience with lung transplantation for pulmonary lymphangioleiomyomatosis.
J Heart Lung Transplant, 24 (2005), pp. 1247-1253
[145.]
C. Benden, F. Rea, J. Behr, P.A. Corris, M. Reynaud-Gaubert, M. Stern, et al.
Lung transplantation for lymphangioleiomyomatosis: the European experience.
J Heart Lung Transplant, 28 (2009), pp. 1-7
[146.]
J.R. Maurer, J. Ryu, G. Beck, J. Moss, J.C. Lee, G. Finlay, et al.
Lung transplantation in the management of patients with lymphangioleiomyomatosis: baseline data from the NHLBI LAM Registry.
J Heart Lung Transplant, 26 (2007), pp. 1209-1293
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