메뉴 건너뛰기




Volumn 23, Issue 2, 2014, Pages 183-198

Novel investigational agents for the treatment of scleroderma

Author keywords

Drugs; Immunomodulatory; Novel therapies; Scleroderma; Systemic sclerosis; Treatment

Indexed keywords

ABATACEPT; ANGIOTENSIN 2 RECEPTOR; BELIMUMAB; BETA CATENIN; BLEOMYCIN; BORTEZOMIB; CONNECTIVE TISSUE GROWTH FACTOR; DERMATOLOGICAL AGENT; IMMUNOGLOBULIN; IMMUNOMODULATING AGENT; INTERLEUKIN 13 ANTIBODY; INTERLEUKIN 4; JANUS KINASE 2 INHIBITOR; LYSOPHOSPHATIDIC ACID; OSTEOPONTIN; PEROXISOME PROLIFERATOR ACTIVATED RECEPTOR; PEROXISOME PROLIFERATOR ACTIVATED RECEPTOR GAMMA; PEROXISOME PROLIFERATOR ACTIVATED RECEPTOR GAMMA AGONIST; PIRFENIDONE; PLACEBO; PROTEIN TYROSINE KINASE INHIBITOR; RAPAMYCIN; RHO KINASE INHIBITOR; RITUXIMAB; SONIC HEDGEHOG PROTEIN; THALIDOMIDE DERIVATIVE; THROMBIN; TOCILIZUMAB; TRANSFORMING GROWTH FACTOR BETA; UNINDEXED DRUG;

EID: 84892403410     PISSN: 13543784     EISSN: 17447658     Source Type: Journal    
DOI: 10.1517/13543784.2014.848852     Document Type: Review
Times cited : (11)

References (120)
  • 1
    • 49949114547 scopus 로고    scopus 로고
    • Molecular subsets in the gene expression signatures of scleroderma skin
    • Milano A, Pendergrass SA, Sargent JL, et al. Molecular subsets in the gene expression signatures of scleroderma skin. PLoS One 2008;3:e2696
    • (2008) PLoS One , vol.3
    • Milano, A.1    Pendergrass, S.A.2    Sargent, J.L.3
  • 3
    • 84655175061 scopus 로고    scopus 로고
    • Understanding fibrosis in systemic sclerosis: Shifting paradigms, emerging opportunities
    • Bhattacharyya S, Wei J, Varga J. Understanding fibrosis in systemic sclerosis: shifting paradigms, emerging opportunities. Nat Rev Rheumatol 2011;8:42-54
    • (2011) Nat Rev Rheumatol , vol.8 , pp. 42-54
    • Bhattacharyya, S.1    Wei, J.2    Varga, J.3
  • 4
    • 0034604110 scopus 로고    scopus 로고
    • Role of transforming growth factor β in human disease
    • DOI 10.1056/NEJM200005043421807
    • Blobe GC, Schiemann WP, Lodish HF. Role of transforming growth factor beta in human disease. N Engl J Med 2000; 342:1350-8 (Pubitemid 30408720)
    • (2000) New England Journal of Medicine , vol.342 , Issue.18 , pp. 1350-1358
    • Blobe, G.C.1    Schiemann, W.P.2    Lodish, H.F.3
  • 5
    • 67649649822 scopus 로고    scopus 로고
    • Overview of pathogenesis of systemic sclerosis
    • Abraham DJ, Krieg T, Distler J, et al. Overview of pathogenesis of systemic sclerosis. Rheumatology (Oxford) 2009; 48(Suppl 3):iii3-7
    • (2009) Rheumatology (Oxford) , vol.48 , Issue.SUPPL. 3
    • Abraham, D.J.1    Krieg, T.2    Distler, J.3
  • 6
    • 0026604019 scopus 로고
    • Sequential dermal microvascular and perivascular changes in the development of scleroderma
    • Prescott RJ, Freemont AJ, Jones CJ, et al. Sequential dermal microvascular and perivascular changes in the development of scleroderma. J Pathol 1992;166:255-63
    • (1992) J Pathol , vol.166 , pp. 255-263
    • Prescott, R.J.1    Freemont, A.J.2    Jones, C.J.3
  • 7
    • 0021185764 scopus 로고
    • Microvascular changes in progressive systemic sclerosis: Immunohistochemical and ultrastructural study
    • Trotta F, Biagini G, Cenacchi G, et al. Microvascular changes in progressive systemic sclerosis: immunohistochemical and ultrastructural study. Clin Exp Rheumatol 1984;2:209-15 (Pubitemid 14016362)
    • (1984) Clinical and Experimental Rheumatology , vol.2 , Issue.3 , pp. 209-215
    • Trotta, F.1    Biagini, G.2    Cenacchi, G.3
  • 8
    • 80155139387 scopus 로고    scopus 로고
    • Vascular involvement in systemic sclerosis (scleroderma)
    • Pattanaik D, Brown M, Postlethwaite AE. Vascular involvement in systemic sclerosis (scleroderma). J Inflamm Res 2011;4:105-25
    • (2011) J Inflamm Res , vol.4 , pp. 105-125
    • Pattanaik, D.1    Brown, M.2    Postlethwaite, A.E.3
  • 9
    • 84857042496 scopus 로고    scopus 로고
    • Angiogenesis and vasculopathy in systemic sclerosis: Evolving concepts
    • Rabquer BJ, Koch AE. Angiogenesis and vasculopathy in systemic sclerosis: evolving concepts. Curr Rheumatol Rep 2012;14:56-63
    • (2012) Curr Rheumatol Rep , vol.14 , pp. 56-63
    • Rabquer, B.J.1    Koch, A.E.2
  • 10
    • 40349108592 scopus 로고    scopus 로고
    • The pathology of scleroderma vascular disease
    • DOI 10.1016/j.rdc.2008.01.001, PII S0889857X08000021
    • Fleming JN, Schwartz SM. The pathology of scleroderma vascular disease. Rheum Dis Clin North Am 2008; 34:41-55; vi (Pubitemid 351337630)
    • (2008) Rheumatic Disease Clinics of North America , vol.34 , Issue.1 , pp. 41-55
    • Fleming, J.N.1    Schwartz, S.M.2
  • 11
    • 84876512983 scopus 로고    scopus 로고
    • My approach to the treatment of scleroderma
    • Shah AA, Wigley FM. My approach to the treatment of scleroderma. Mayo Clin Proc 2013;88:377-93
    • (2013) Mayo Clin Proc , vol.88 , pp. 377-393
    • Shah, A.A.1    Wigley, F.M.2
  • 12
    • 84855256381 scopus 로고    scopus 로고
    • The role of intravenous immunoglobulin preparations in the treatment of systemic sclerosis
    • Baleva M, Nikolov K. The role of intravenous immunoglobulin preparations in the treatment of systemic sclerosis. Int J Rheumatol 2011; 2011:829751
    • (2011) Int J Rheumatol , vol.2011 , pp. 829751
    • Baleva, M.1    Nikolov, K.2
  • 13
    • 0033017344 scopus 로고    scopus 로고
    • Anti-sclerotic effect of transforming growth factor-β antibody in a mouse model of bleomycin-induced scleroderma
    • DOI 10.1006/clim.1999.4720
    • Yamamoto T, Takagawa S, Katayama I, et al. Anti-sclerotic effect of transforming growth factor-beta antibody in a mouse model of bleomycin-induced scleroderma. Clin Immunol 1999; 92:6-13 (Pubitemid 29315981)
    • (1999) Clinical Immunology , vol.92 , Issue.1 , pp. 6-13
    • Yamamoto, T.1    Takagawa, S.2    Katayama, I.3    Nishioka, K.4
  • 14
    • 0036277168 scopus 로고    scopus 로고
    • The role of intravenous immunoglobulin therapy in mediating skin fibrosis in tight skin mice
    • DOI 10.1002/art.10363
    • Blank M, Levy Y, Amital H, et al. The role of intravenous immunoglobulin therapy in mediating skin fibrosis in tight skin mice. Arthritis Rheum 2002; 46:1689-90 (Pubitemid 34620218)
    • (2002) Arthritis and Rheumatism , vol.46 , Issue.6 , pp. 1689-1690
    • Blank, M.1    Levy, Y.2    Amital, H.3    Shoenfeld, Y.4    Pines, M.5    Genina, O.6
  • 18
    • 0025506355 scopus 로고
    • Efficacy of intravenous immunoglobulins in sclerodermatomyositis
    • Bodemer C, Teillac D, Le Bourgeois M, et al. Efficacy of intravenous immunoglobulins in sclerodermatomyositis. Br J Dermatol 1990;123:545-6
    • (1990) Br J Dermatol , vol.123 , pp. 545-546
    • Bodemer, C.1    Teillac, D.2    Le Bourgeois, M.3
  • 19
    • 84892430215 scopus 로고    scopus 로고
    • IVIG treatment in systemic sclerosis [Internet]. Washington, D.C. National Institutes of Health Available from [Updated 2 May 2013; Cited 28 January 2013]
    • IVIG treatment in systemic sclerosis. [Internet]. Washington, D.C.: National Institutes of Health. 2013. Available from: http://www.clinicaltrials. gov/ct2/show/NCT01785056term=NCT017850 56&rank=1 [Updated 2 May 2013; Cited 28 January 2013]
    • (2013)
  • 22
    • 80051607473 scopus 로고    scopus 로고
    • B cells in systemic sclerosis: A possible target for therapy
    • Bosello S, De Luca G, Tolusso B, et al. B cells in systemic sclerosis: a possible target for therapy. Autoimmun Rev 2011;10:624-30
    • (2011) Autoimmun Rev , vol.10 , pp. 624-630
    • Bosello, S.1    De Luca, G.2    Tolusso, B.3
  • 23
    • 2642511623 scopus 로고    scopus 로고
    • Altered blood B lymphocyte homeostasis in systemic sclerosis: Expanded naive B cells and diminished but activated memory B cells
    • DOI 10.1002/art.20274
    • Sato S, Fujimoto M, Hasegawa M, et al. Altered blood B lymphocyte homeostasis in systemic sclerosis: expanded naive B cells and diminished but activated memory B cells. Arthritis Rheum 2004; 50:1918-27 (Pubitemid 38725103)
    • (2004) Arthritis and Rheumatism , vol.50 , Issue.6 , pp. 1918-1927
    • Sato, S.1    Fujimoto, M.2    Hasegawa, M.3    Takehara, K.4
  • 24
    • 80053467638 scopus 로고    scopus 로고
    • B-cell depletion therapy in systemic sclerosis: Experimental rationale and update on clinical evidence
    • Daoussis D, Liossis SN, Yiannopoulos G, et al. B-cell depletion therapy in systemic sclerosis: experimental rationale and update on clinical evidence. Int J Rheumatol 2011;2011:214013
    • (2011) Int J Rheumatol , vol.2011 , pp. 214013
    • Daoussis, D.1    Liossis, S.N.2    Yiannopoulos, G.3
  • 25
    • 77950443096 scopus 로고    scopus 로고
    • Experience with rituximab in scleroderma: Results from a 1-year, proof-of-principle study
    • Daoussis D, Liossis SN, Tsamandas AC, et al. Experience with rituximab in scleroderma: results from a 1-year, proof-of-principle study. Rheumatology (Oxford) 2010;49:271-80
    • (2010) Rheumatology (Oxford) , vol.49 , pp. 271-280
    • Daoussis, D.1    Liossis, S.N.2    Tsamandas, A.C.3
  • 26
    • 84867383069 scopus 로고    scopus 로고
    • Effect of long-term treatment with rituximab on pulmonary function and skin fibrosis in patients with diffuse systemic sclerosis
    • Daoussis D, Liossis SN, Tsamandas AC, et al. Effect of long-term treatment with rituximab on pulmonary function and skin fibrosis in patients with diffuse systemic sclerosis. Clin Exp Rheumatol 2012;30:S17-22
    • (2012) Clin Exp Rheumatol , vol.30
    • Daoussis, D.1    Liossis, S.N.2    Tsamandas, A.C.3
  • 27
    • 84865814629 scopus 로고    scopus 로고
    • Severe interstitial lung disease in connective tissue disease: Rituximab as rescue therapy
    • Keir GJ, Maher TM, Hansell DM, et al. Severe interstitial lung disease in connective tissue disease: rituximab as rescue therapy. Eur Respir J 2012; 40:641-8
    • (2012) Eur Respir J , vol.40 , pp. 641-648
    • Keir, G.J.1    Maher, T.M.2    Hansell, D.M.3
  • 28
    • 80052640206 scopus 로고    scopus 로고
    • Cyclophosphamide-refractory scleroderma-associated interstitial lung disease: Remarkable clinical and radiological response to a single course of rituximab combined with high-dose corticosteroids
    • Haroon M, McLaughlin P, Henry M, et al. Cyclophosphamide-refractory scleroderma-associated interstitial lung disease: remarkable clinical and radiological response to a single course of rituximab combined with high-dose corticosteroids. Ther Adv Respir Dis 2011;5:299-304
    • (2011) Ther Adv Respir Dis , vol.5 , pp. 299-304
    • Haroon, M.1    McLaughlin, P.2    Henry, M.3
  • 29
    • 84892380407 scopus 로고    scopus 로고
    • [Internet]. Washington, D.C.: National Institutes of Health Available from [Updated 1 August2013; Cited 10 December 2012]
    • Rituximab in systemic sclerosis. [Internet]. Washington, D.C.: National Institutes of Health. 2013. Available from: http://www.clinicaltrials.gov/ct2/ show/NCT01748084term=NCT01748084&rank=1 [Updated 1 August2013; Cited 10 December 2012]
    • (2013) Rituximab in Systemic Sclerosis
  • 30
    • 84892417255 scopus 로고    scopus 로고
    • [Internet]. Washington, D.C.: National Institutes of Health Available from [Updated 13 May 2013; Cited 15 August2012]
    • Belimumab for the treatment of diffuse cutaneous systemic sclerosis. [Internet]. Washington, D.C.: National Institutes of Health. 2013. Available from: http://www.clinicaltrials.gov/ct2/show/NCT01670565term= NCT01670565&rank=1 [Updated 13 May 2013; Cited 15 August2012]
    • (2013) Belimumab for the Treatment of Diffuse Cutaneous Systemic Sclerosis
  • 32
    • 84878404903 scopus 로고    scopus 로고
    • Outcomes of patients with systemic sclerosis-associated polyarthritis and myopathy treated with tocilizumab or abatacept: A EUSTAR observational study
    • Elhai M, Meunier M, Matucci-Cerinic M, et al. Outcomes of patients with systemic sclerosis-associated polyarthritis and myopathy treated with tocilizumab or abatacept: a EUSTAR observational study. Ann Rheum Dis 2013;72:1217-20
    • (2013) Ann Rheum Dis , vol.72 , pp. 1217-1220
    • Elhai, M.1    Meunier, M.2    Matucci-Cerinic, M.3
  • 34
    • 84892403934 scopus 로고    scopus 로고
    • Washington, D.C.: National Institutes of Health Available from [Updated 10 July 2012; Cited 24 July 2007]
    • Imatinib in systemic sclerosis. [Internet]. Washington, D.C.: National Institutes of Health. 2012. Available from: http://www.clinicaltrials.gov/ct2/ show/NCT00506831term=NCT00506831&rank=1 [Updated 10 July 2012; Cited 24 July 2007]
    • (2012) Imatinib in Systemic Sclerosis. [Internet]
  • 35
    • 79958834107 scopus 로고    scopus 로고
    • Are tyrosine kinase inhibitors promising for the treatment of systemic sclerosis and other fibrotic diseases
    • Beyer C, Distler JH, Distler O. Are tyrosine kinase inhibitors promising for the treatment of systemic sclerosis and other fibrotic diseases Swiss Med Wkly 2010;140:w13050
    • (2010) Swiss Med Wkly , vol.140
    • Beyer, C.1    Distler, J.H.2    Distler, O.3
  • 36
    • 80155206225 scopus 로고    scopus 로고
    • Imatinib in active diffuse cutaneous systemic sclerosis: Results of a six-month, randomized, double-blind, placebo-controlled, proof-of-concept pilot study at a single center
    • Pope J, McBain D, Petrlich L, et al. Imatinib in active diffuse cutaneous systemic sclerosis: results of a six-month, randomized, double-blind, placebo-controlled, proof-of-concept pilot study at a single center. Arthritis Rheum 2011;63:3547-51
    • (2011) Arthritis Rheum , vol.63 , pp. 3547-3551
    • Pope, J.1    McBain, D.2    Petrlich, L.3
  • 37
    • 84856049288 scopus 로고    scopus 로고
    • Proteasomal inhibition after injury prevents fibrosis by modulating TGFbeta( 1) signalling
    • Mutlu GM, Budinger GR, Wu M, et al. Proteasomal inhibition after injury prevents fibrosis by modulating TGFbeta( 1) signalling. Thorax 2012;67:139-46
    • (2012) Thorax , vol.67 , pp. 139-146
    • Mutlu, G.M.1    Budinger, G.R.2    Wu, M.3
  • 38
    • 33645805628 scopus 로고    scopus 로고
    • Proteasome blockade exerts an antifibrotic activity by coordinately down-regulating type I collagen and tissue inhibitor of metalloproteinase-1 and up-regulating metalloproteinase-1 production in human dermal fibroblasts
    • DOI 10.1096/fj.05-4870fje
    • Fineschi S, Reith W, Guerne PA, et al. Proteasome blockade exerts an antifibrotic activity by coordinately down-regulating type I collagen and tissue inhibitor of metalloproteinase-1 and up-regulating metalloproteinase-1 production in human dermal fibroblasts. FASEB J 2006;20:562-4 (Pubitemid 46671250)
    • (2006) FASEB Journal , vol.20 , Issue.3 , pp. 562-564
    • Fineschi, S.1    Reith, W.2    Guerne, P.A.3    Dayer, J.-M.4    Chizzolini, C.5
  • 39
    • 53349129568 scopus 로고    scopus 로고
    • In vivo investigations on anti-fibrotic potential of proteasome inhibition in lung and skin fibrosis
    • Fineschi S, Bongiovanni M, Donati Y, et al. In vivo investigations on anti-fibrotic potential of proteasome inhibition in lung and skin fibrosis. Am J Respir Cell Mol Biol 2008;39:458-65
    • (2008) Am J Respir Cell Mol Biol , vol.39 , pp. 458-465
    • Fineschi, S.1    Bongiovanni, M.2    Donati, Y.3
  • 40
    • 84863809258 scopus 로고    scopus 로고
    • Proteasome inhibition prevents development of experimental dermal fibrosis
    • Koca SS, Ozgen M, Dagli F, et al. Proteasome inhibition prevents development of experimental dermal fibrosis. Inflammation 2012;35:810-7
    • (2012) Inflammation , vol.35 , pp. 810-817
    • Koca, S.S.1    Ozgen, M.2    Dagli, F.3
  • 41
    • 79954579275 scopus 로고    scopus 로고
    • Angiotensin II type 2 receptor signaling attenuates aortic aneurysm in mice through ERK antagonism
    • Habashi JP, Doyle JJ, Holm TM, et al. Angiotensin II type 2 receptor signaling attenuates aortic aneurysm in mice through ERK antagonism. Science 2011;332:361-5
    • (2011) Science , vol.332 , pp. 361-365
    • Habashi, J.P.1    Doyle, J.J.2    Holm, T.M.3
  • 43
    • 84892374609 scopus 로고    scopus 로고
    • [Internet]. Washington, D.C.: National Institutes of Health. Available from [Updated 4 March 2013; Cited 30 September 2008]
    • Study of the efficacy of losartan on aortic dilatation in patients with marfan syndrome (MARFANSARTAN). [Internet]. Washington, D.C.: National Institutes of Health. 2013. Available from: http://www. clinicaltrials.gov/ct2/ show/NCT00763893term=NCT00763893&rank=1 [Updated 4 March 2013; Cited 30 September 2008]
    • (2013) Study of the Efficacy of Losartan on Aortic Dilatation in Patients with Marfan Syndrome (MARFANSARTAN)
  • 45
    • 84862763855 scopus 로고    scopus 로고
    • Matrix-dependent perturbation of TGFbeta signaling and disease
    • Doyle JJ, Gerber EE, Dietz HC. Matrix-dependent perturbation of TGFbeta signaling and disease. FEBS Lett 2012;586:2003-15
    • (2012) FEBS Lett , vol.586 , pp. 2003-2015
    • Doyle, J.J.1    Gerber, E.E.2    Dietz, H.C.3
  • 46
    • 84870319857 scopus 로고    scopus 로고
    • Focal adhesion kinase and reactive oxygen species contribute to the persistent fibrotic phenotype of lesional scleroderma fibroblasts
    • Shi-wen X, Thompson K, Khan K, et al. Focal adhesion kinase and reactive oxygen species contribute to the persistent fibrotic phenotype of lesional scleroderma fibroblasts. Rheumatology (Oxford) 2012;51:2146-54
    • (2012) Rheumatology (Oxford) , vol.51 , pp. 2146-2154
    • Shi-Wen, X.1    Thompson, K.2    Khan, K.3
  • 47
    • 84871347177 scopus 로고    scopus 로고
    • MiR-150 down-regulation contributes to the constitutive type i collagen overexpression in scleroderma dermal fibroblasts via the induction of integrin beta3
    • Honda N, Jinnin M, Kira-Etoh T, et al. miR-150 down-regulation contributes to the constitutive type I collagen overexpression in scleroderma dermal fibroblasts via the induction of integrin beta3. Am J Pathol 2013;182:206-16
    • (2013) Am J Pathol , vol.182 , pp. 206-216
    • Honda, N.1    Jinnin, M.2    Kira-Etoh, T.3
  • 48
    • 38049002642 scopus 로고    scopus 로고
    • Partial inhibition of integrin alpha(v) beta6 prevents pulmonary fibrosis without exacerbating inflammation
    • Horan GS, Wood S, Ona V, et al. Partial inhibition of integrin alpha(v) beta6 prevents pulmonary fibrosis without exacerbating inflammation. Am J Respir Crit Care Med 2008;177:56-65
    • (2008) Am J Respir Crit Care Med , vol.177 , pp. 56-65
    • Horan, G.S.1    Wood, S.2    Ona, V.3
  • 49
    • 66049111238 scopus 로고    scopus 로고
    • Regulation of pulmonary inflammation and fibrosis through expression of integrins alphaVbeta3 and alphaVbeta5 on pulmonary T lymphocytes
    • Luzina IG, Todd NW, Nacu N, et al. Regulation of pulmonary inflammation and fibrosis through expression of integrins alphaVbeta3 and alphaVbeta5 on pulmonary T lymphocytes. Arthritis Rheum 2009;60:1530-9
    • (2009) Arthritis Rheum , vol.60 , pp. 1530-1539
    • Luzina, I.G.1    Todd, N.W.2    Nacu, N.3
  • 50
    • 33144458448 scopus 로고    scopus 로고
    • Increased expression of integrin αvβ5 induces the myofibroblastic differentiation of dermal fibroblasts
    • DOI 10.2353/ajpath.2006.041306
    • Asano Y, Ihn H, Yamane K, et al. Increased expression of integrin alphavbeta5 induces the myofibroblastic differentiation of dermal fibroblasts. Am J Pathol 2006;168:499-510 (Pubitemid 43271148)
    • (2006) American Journal of Pathology , vol.168 , Issue.2 , pp. 499-510
    • Asano, Y.1    Ihn, H.2    Yamane, K.3    Jinnin, M.4    Tamaki, K.5
  • 51
    • 33746141265 scopus 로고    scopus 로고
    • Involvement of αvβ5 integrin in the establishment of autocrine TGF-β signaling in dermal fibroblasts derived from localized scleroderma
    • DOI 10.1038/sj.jid.5700331, PII 5700331
    • Asano Y, Ihn H, Jinnin M, et al. Involvement of alphavbeta5 integrin in the establishment of autocrine TGF-beta signaling in dermal fibroblasts derived from localized scleroderma. J Invest Dermatol 2006;126:1761-9 (Pubitemid 44086510)
    • (2006) Journal of Investigative Dermatology , vol.126 , Issue.8 , pp. 1761-1769
    • Asano, Y.1    Ihn, H.2    Jinnin, M.3    Mimura, Y.4    Tamaki, K.5
  • 52
    • 69449108113 scopus 로고    scopus 로고
    • Loss of beta1 integrin in mouse fibroblasts results in resistance to skin scleroderma in a mouse model
    • Liu S, Kapoor M, Denton CP, et al. Loss of beta1 integrin in mouse fibroblasts results in resistance to skin scleroderma in a mouse model. Arthritis Rheum 2009;60:2817-21
    • (2009) Arthritis Rheum , vol.60 , pp. 2817-2821
    • Liu, S.1    Kapoor, M.2    Denton, C.P.3
  • 53
    • 25444501226 scopus 로고    scopus 로고
    • Involvement of αvβ5 integrin-mediated activation of latent transforming growth factor β1 in autocrine transforming growth factor β signaling in systemic sclerosis fibroblasts
    • DOI 10.1002/art.21246
    • Asano Y, Ihn H, Yamane K, et al. Involvement of alphavbeta5 integrin-mediated activation of latent transforming growth factor beta1 in autocrine transforming growth factor beta signaling in systemic sclerosis fibroblasts. Arthritis Rheum 2005;52:2897-905 (Pubitemid 41369121)
    • (2005) Arthritis and Rheumatism , vol.52 , Issue.9 , pp. 2897-2905
    • Asano, Y.1    Ihn, H.2    Yamane, K.3    Jinnin, M.4    Mimura, Y.5    Tamaki, K.6
  • 55
    • 77952974791 scopus 로고    scopus 로고
    • Mutations in fibrillin-1 cause congenital scleroderma: Stiff skin syndrome
    • Loeys BL, Gerber EE, Riegert-Johnson D, et al. Mutations in fibrillin-1 cause congenital scleroderma: stiff skin syndrome. Sci Transl Med 2010;2:23ra20
    • (2010) Sci Transl Med , vol.2
    • Loeys, B.L.1    Gerber, E.E.2    Riegert-Johnson, D.3
  • 56
    • 78349281192 scopus 로고    scopus 로고
    • PPARgamma downregulation by TGFss in fibroblast and impaired expression and function in systemic sclerosis: A novel mechanism for progressive fibrogenesis
    • Wei J, Ghosh AK, Sargent JL, et al. PPARgamma downregulation by TGFss in fibroblast and impaired expression and function in systemic sclerosis: a novel mechanism for progressive fibrogenesis. PLoS One 2010;5:e13778
    • (2010) PLoS One , vol.5
    • Wei, J.1    Ghosh, A.K.2    Sargent, J.L.3
  • 57
    • 77958025377 scopus 로고    scopus 로고
    • Peroxisome proliferator-activated receptor gamma: Innate protection from excessive fibrogenesis and potential therapeutic target in systemic sclerosis
    • Wei J, Bhattacharyya S, Varga J. Peroxisome proliferator-activated receptor gamma: innate protection from excessive fibrogenesis and potential therapeutic target in systemic sclerosis. Curr Opin Rheumatol 2010;22:671-6
    • (2010) Curr Opin Rheumatol , vol.22 , pp. 671-676
    • Wei, J.1    Bhattacharyya, S.2    Varga, J.3
  • 58
    • 1842733168 scopus 로고    scopus 로고
    • Disruption of Transforming Growth Factor β Signaling and Profibrotic Responses in Normal Skin Fibroblasts by Peroxisome Proliferator-Activated Receptor γ
    • DOI 10.1002/art.20104
    • Ghosh AK, Bhattacharyya S, Lakos G, et al. Disruption of transforming growth factor beta signaling and profibrotic responses in normal skin fibroblasts by peroxisome proliferator-activated receptor gamma. Arthritis Rheum 2004; 50:1305-18 (Pubitemid 38480842)
    • (2004) Arthritis and Rheumatism , vol.50 , Issue.4 , pp. 1305-1318
    • Ghosh, A.K.1    Bhattacharyya, S.2    Lakos, G.3    Chen, S.-J.4    Mori, Y.5    Varga, J.6
  • 59
    • 79251563128 scopus 로고    scopus 로고
    • PPAR-gamma ligands repress TGFbeta-induced myofibroblast differentiation by targeting the PI3K/Akt pathway: Implications for therapy of fibrosis
    • Kulkarni AA, Thatcher TH, Olsen KC, et al. PPAR-gamma ligands repress TGFbeta-induced myofibroblast differentiation by targeting the PI3K/Akt pathway: implications for therapy of fibrosis. PLoS One 2011;6:e15909
    • (2011) PLoS One , vol.6
    • Kulkarni, A.A.1    Thatcher, T.H.2    Olsen, K.C.3
  • 60
    • 69449094871 scopus 로고    scopus 로고
    • Loss of peroxisome proliferator-activated receptor gamma in mouse fibroblasts results in increased susceptibility to bleomycin-induced skin fibrosis
    • Kapoor M, McCann M, Liu S, et al. Loss of peroxisome proliferator- activated receptor gamma in mouse fibroblasts results in increased susceptibility to bleomycin-induced skin fibrosis. Arthritis Rheum 2009;60:2822-9
    • (2009) Arthritis Rheum , vol.60 , pp. 2822-2829
    • Kapoor, M.1    McCann, M.2    Liu, S.3
  • 61
    • 84860272867 scopus 로고    scopus 로고
    • Levels of adiponectin a marker for PPAR-gamma activity correlate with skin fibrosis in systemic sclerosis: Potential utility as biomarker
    • Lakota K, Wei J, Carns M, et al. Levels of adiponectin, a marker for PPAR-gamma activity, correlate with skin fibrosis in systemic sclerosis: potential utility as biomarker Arthritis Res Ther 2012;14:R102
    • (2012) Arthritis Res Ther , vol.14
    • Lakota, K.1    Wei, J.2    Carns, M.3
  • 62
    • 84892374809 scopus 로고    scopus 로고
    • A synthetic PPAR-gamma agonist triterpenoid ameliorates experimental fibrosis: PPAR-gamma-independent suppression of fibrotic responses
    • Epub ahead of print
    • Wei J, Zhu H, Komura K, et al. A synthetic PPAR-gamma agonist triterpenoid ameliorates experimental fibrosis: PPAR-gamma-independent suppression of fibrotic responses. Ann Rheum Dis 2013; Epub ahead of print
    • (2013) Ann Rheum Dis
    • Wei, J.1    Zhu, H.2    Komura, K.3
  • 63
    • 0031911610 scopus 로고    scopus 로고
    • Serum levels of interleukin 6 (IL-6), oncostatin M, soluble IL-6 receptor, and soluble gp130 in patients with systemic sclerosis
    • Hasegawa M, Sato S, Fujimoto M, et al. Serum levels of interleukin 6 (IL-6), oncostatin M, soluble IL-6 receptor, and soluble gp130 in patients with systemic sclerosis. J Rheumatol 1998; 25:308-13 (Pubitemid 28088260)
    • (1998) Journal of Rheumatology , vol.25 , Issue.2 , pp. 308-313
    • Hasegawa, M.1    Sato, S.2    Fujimoto, M.3    Ihn, H.4    Kikuchi, K.5    Takehara, K.6
  • 64
    • 84861805021 scopus 로고    scopus 로고
    • Interleukin 6 in systemic sclerosis and potential implications for targeted therapy
    • Muangchan C, Pope JE. Interleukin 6 in systemic sclerosis and potential implications for targeted therapy. J Rheumatol 2012;39:1120-4
    • (2012) J Rheumatol , vol.39 , pp. 1120-1124
    • Muangchan, C.1    Pope, J.E.2
  • 65
    • 78049421175 scopus 로고    scopus 로고
    • The skin of patients with systemic sclerosis softened during the treatment with anti-IL-6 receptor antibody tocilizumab
    • Shima Y, Kuwahara Y, Murota H, et al. The skin of patients with systemic sclerosis softened during the treatment with anti-IL-6 receptor antibody tocilizumab. Rheumatology (Oxford) 2010;49:2408-12
    • (2010) Rheumatology (Oxford) , vol.49 , pp. 2408-2412
    • Shima, Y.1    Kuwahara, Y.2    Murota, H.3
  • 66
    • 84892386944 scopus 로고    scopus 로고
    • [Internet]. Washington, D.C.: National Institutes of Health Available from [Updated 19 August 2013; Cited 10 February 2012]
    • A study of RoActemra/Actemra (tocilizumab) versus placebo in patients with systemic sclerosis. [Internet]. Washington, D.C.: National Institutes of Health. 2013. Available from: http://www. clinicaltrials.gov/ct2/show/NCT 01532869term=NCT01532869&rank=1 [Updated 19 August 2013; Cited 10 February 2012]
    • (2013) A Study of RoActemra/Actemra (Tocilizumab) Versus Placebo in Patients with Systemic Sclerosis
  • 67
    • 40749107501 scopus 로고    scopus 로고
    • Regulation and function of connective tissue growth factor/CCN2 in tissue repair, scarring and fibrosis
    • DOI 10.1016/j.cytogfr.2008.01.002, PII S135961010800004X
    • Shi-Wen X, Leask A, Abraham D. Regulation and function of connective tissue growth factor/CCN2 in tissue repair, scarring and fibrosis. Cytokine Growth Factor Rev 2008;19:133-44 (Pubitemid 351382279)
    • (2008) Cytokine and Growth Factor Reviews , vol.19 , Issue.2 , pp. 133-144
    • Shi-Wen, X.1    Leask, A.2    Abraham, D.3
  • 68
    • 79951713169 scopus 로고    scopus 로고
    • Cooperative interaction of CTGF and TGF-beta in animal models of fibrotic disease
    • Wang Q, Usinger W, Nichols B, et al. Cooperative interaction of CTGF and TGF-beta in animal models of fibrotic disease. Fibrogenesis Tissue Repair 2011; 4:4, 1536-4-4
    • (2011) Fibrogenesis Tissue Repair , vol.4 , Issue.4 , pp. 1536-1544
    • Wang, Q.1    Usinger, W.2    Nichols, B.3
  • 69
    • 0034535271 scopus 로고    scopus 로고
    • Connective tissue growth factor (CTGF) stimulates vascular smooth muscle cell growth and migration in vitro
    • Fan WH, Pech M, Karnovsky MJ. Connective tissue growth factor (CTGF) stimulates vascular smooth muscle cell growth and migration in vitro. Eur J Cell Biol 2000;79:915-23 (Pubitemid 32000725)
    • (2000) European Journal of Cell Biology , vol.79 , Issue.12 , pp. 915-923
    • Fan, W.-H.1    Pech, M.2    Karnovsky, M.J.3
  • 70
    • 48749112311 scopus 로고    scopus 로고
    • Neutralizing monoclonal antibody to human connective tissue growth factor ameliorates transforming growth factor-beta-induced mouse fibrosis
    • Ikawa Y, Ng PS, Endo K, et al. Neutralizing monoclonal antibody to human connective tissue growth factor ameliorates transforming growth factor-beta-induced mouse fibrosis. J Cell Physiol 2008;216:680-7
    • (2008) J Cell Physiol , vol.216 , pp. 680-687
    • Ikawa, Y.1    Ng, P.S.2    Endo, K.3
  • 71
    • 67650069660 scopus 로고    scopus 로고
    • Pivotal role of connective tissue growth factor in lung fibrosis: MAPK-dependent transcriptional activation of type i collagen
    • Ponticos M, Holmes AM, Shi-wen X, et al. Pivotal role of connective tissue growth factor in lung fibrosis: MAPK-dependent transcriptional activation of type I collagen. Arthritis Rheum 2009;60:2142-55
    • (2009) Arthritis Rheum , vol.60 , pp. 2142-2155
    • Ponticos, M.1    Holmes, A.M.2    Shi-Wen, X.3
  • 72
    • 84871768637 scopus 로고    scopus 로고
    • Interleukin-13-producing CD8+ T cells mediate dermal fibrosis in patients with systemic sclerosis
    • Fuschiotti P, Larregina AT, Ho J, et al. Interleukin-13-producing CD8+ T cells mediate dermal fibrosis in patients with systemic sclerosis. Arthritis Rheum 2013;65:236-46
    • (2013) Arthritis Rheum , vol.65 , pp. 236-246
    • Fuschiotti, P.1    Larregina, A.T.2    Ho, J.3
  • 73
    • 31644431508 scopus 로고    scopus 로고
    • Beneficial acute effects of Rho-kinase inhibitor in patients with pulmonary arterial hypertension
    • DOI 10.1253/circj.70.174
    • Ishikura K, Yamada N, Ito M, et al. Beneficial acute effects of rho-kinase inhibitor in patients with pulmonary arterial hypertension. Circ J 2006; 70:174-8 (Pubitemid 43170128)
    • (2006) Circulation Journal , vol.70 , Issue.2 , pp. 174-178
    • Ishikura, K.1    Yamada, N.2    Ito, M.3    Ota, S.4    Nakamura, M.5    Isaka, N.6    Nakano, T.7
  • 74
    • 79960432567 scopus 로고    scopus 로고
    • Intraradial administration of fasudil inhibits augmented rho kinase activity to effectively dilate the spastic radial artery during coronary artery bypass grafting surgery
    • Takagi T, Okamoto Y, Tomita S, et al. Intraradial administration of fasudil inhibits augmented rho kinase activity to effectively dilate the spastic radial artery during coronary artery bypass grafting surgery. J Thorac Cardiovasc Surg 2011;142:e59-65
    • (2011) J Thorac Cardiovasc Surg , vol.142
    • Takagi, T.1    Okamoto, Y.2    Tomita, S.3
  • 75
    • 84863202226 scopus 로고    scopus 로고
    • Efficacy of rho kinase inhibitor fasudil in secondary raynaud's phenomenon
    • Fava A, Wung PK, Wigley FM, et al. Efficacy of rho kinase inhibitor fasudil in secondary raynaud's phenomenon. Arthritis Care Res (Hoboken) 2012; 64:925-9
    • (2012) Arthritis Care Res (Hoboken) , vol.64 , pp. 925-929
    • Fava, A.1    Wung, P.K.2    Wigley, F.M.3
  • 76
    • 84892397128 scopus 로고    scopus 로고
    • [Internet]. Washington, D.C.: National Institutes of Health Available from [Updated 29 March 2012; Cited 6 July 2007]
    • A rho-kinase inhibitor (fasudil) in the treatment of raynaud's phenomenon. [Internet]. Washington, D.C.: National Institutes of Health. 2012. Available from: http://www.clinicaltrials.gov/ct2/show/NCT00498615term= NCT00498615&rank=1 [Updated 29 March 2012; Cited 6 July 2007]
    • (2012) A Rho-kinase Inhibitor (Fasudil) in the Treatment of Raynaud's Phenomenon
  • 78
    • 19944430437 scopus 로고    scopus 로고
    • Molecular mechanisms whereby immunomodulatory drugs activate natural killer cells: Clinical application
    • Hayashi T, Hideshima T, Akiyama M, et al. Molecular mechanisms whereby immunomodulatory drugs activate natural killer cells: clinical application. Br J Haematol 2005;128:192-203
    • (2005) Br J Haematol , vol.128 , pp. 192-203
    • Hayashi, T.1    Hideshima, T.2    Akiyama, M.3
  • 79
    • 84872186323 scopus 로고    scopus 로고
    • Novel immunomodulatory compounds in multiple myeloma
    • Saini N, Mahindra A. Novel immunomodulatory compounds in multiple myeloma. Expert Opin Investig Drugs 2013;22:207-15
    • (2013) Expert Opin Investig Drugs , vol.22 , pp. 207-215
    • Saini, N.1    Mahindra, A.2
  • 80
    • 40449135275 scopus 로고    scopus 로고
    • Alternate day pomalidomide retains anti-myeloma effect with reduced adverse events and evidence of in vivo immunomodulation
    • DOI 10.1111/j.1365-2141.2008.07013.x
    • Streetly MJ, Gyertson K, Daniel Y, et al. Alternate day pomalidomide retains anti-myeloma effect with reduced adverse events and evidence of in vivo immunomodulation. Br J Haematol 2008;141:41-51 (Pubitemid 351350609)
    • (2008) British Journal of Haematology , vol.141 , Issue.1 , pp. 41-51
    • Streetly, M.J.1    Gyertson, K.2    Daniel, Y.3    Zeldis, J.B.4    Kazmi, M.5    Schey, S.A.6
  • 82
    • 84867404501 scopus 로고    scopus 로고
    • Pomalidomide is effective for prevention and treatment of experimental skin fibrosis
    • Weingartner S, Zerr P, Tomcik M, et al. Pomalidomide is effective for prevention and treatment of experimental skin fibrosis. Ann Rheum Dis 2012; 71:1895-9
    • (2012) Ann Rheum Dis , vol.71 , pp. 1895-1899
    • Weingartner, S.1    Zerr, P.2    Tomcik, M.3
  • 83
    • 84892389732 scopus 로고    scopus 로고
    • Study of pomalidomide (CC-4047) to evaluate safety, tolerability, pharmacokinetics, pharmacodynamics and effectiveness for subjects with systemic sclerosis with int5erstitial lung disease [Internet] Washington, D.C.: National Institutes of Health Available from [Updated 6 April 2013; Cited 19 March 2012]
    • Study of pomalidomide (CC-4047) to evaluate safety, tolerability, pharmacokinetics, pharmacodynamics and effectiveness for subjects with systemic sclerosis with int5erstitial lung disease. [Internet]. Washington, D.C.: National Institutes of Health. 2013. Available from: http://www.clinicaltrials. gov/ct2/show/NCT01559129 term=NCT01559129&rank=1 [Updated 6 April 2013; Cited 19 March 2012]
    • (2013)
  • 84
    • 78649502678 scopus 로고    scopus 로고
    • Immunotherapy of systemic sclerosis
    • Manno R, Boin F. Immunotherapy of systemic sclerosis. Immunotherapy 2010;2:863-78
    • (2010) Immunotherapy , vol.2 , pp. 863-878
    • Manno, R.1    Boin, F.2
  • 85
    • 77955349716 scopus 로고    scopus 로고
    • Treatment with rapamycin prevents fibrosis in tight-skin and bleomycininduced mouse models of systemic sclerosis
    • Yoshizaki A, Yanaba K, Yoshizaki A, et al. Treatment with rapamycin prevents fibrosis in tight-skin and bleomycininduced mouse models of systemic sclerosis. Arthritis Rheum 2010; 62:2476-87
    • (2010) Arthritis Rheum , vol.62 , pp. 2476-2487
    • Yoshizaki, A.1    Yanaba, K.2    Yoshizaki, A.3
  • 86
    • 73249139173 scopus 로고    scopus 로고
    • Rapamycin versus methotrexate in early diffuse systemic sclerosis: Results from a randomized, single-blind pilot study
    • Su TI, Khanna D, Furst DE, et al. Rapamycin versus methotrexate in early diffuse systemic sclerosis: results from a randomized, single-blind pilot study. Arthritis Rheum 2009;60:3821-30
    • (2009) Arthritis Rheum , vol.60 , pp. 3821-3830
    • Su, T.I.1    Khanna, D.2    Furst, D.E.3
  • 87
    • 84892425168 scopus 로고    scopus 로고
    • [Internet]. Washington, D.C.: National Institutes of Health Available from [Updated 28 December 2005; Cited 14 October 2005]
    • Rapamycin vs methotrexate in diffuse SSc. [Internet]. Washington, D.C.: National Institutes of Health. 2005. Available from: http://www. clinicaltrials.gov/ct2/show/NCT 00241189term=NCT00241189&rank=1 [Updated 28 December 2005; Cited 14 October 2005]
    • (2005) Rapamycin Vs Methotrexate in Diffuse SSc
  • 88
    • 84879399953 scopus 로고    scopus 로고
    • Pirfenidone for IPF. Pro/con debate; The'con' viewpoint
    • Raghu G, Thickett DR. Pirfenidone for IPF. Pro/con debate; the 'con' viewpoint. Thorax 2013;68:605-8
    • (2013) Thorax , vol.68 , pp. 605-608
    • Raghu, G.1    Thickett, D.R.2
  • 89
    • 0032926937 scopus 로고    scopus 로고
    • Treatment of idiopathic pulmonary fibrosis with a new antifibrotic agent, pirfenidone: Results of a prospective, open-label phase II study
    • Raghu G, Johnson WC, Lockhart D, et al. Treatment of idiopathic pulmonary fibrosis with a new antifibrotic agent, pirfenidone: results of a prospective, open-label phase II study. Am J Respir Crit Care Med 1999;159:1061-9 (Pubitemid 29202536)
    • (1999) American Journal of Respiratory and Critical Care Medicine , vol.159 , Issue.4 , pp. 1061-1069
    • Raghu, G.1    Johnson, W.C.2    Lockhart, D.3    Mageto, Y.4
  • 90
    • 77951160564 scopus 로고    scopus 로고
    • Pirfenidone in idiopathic pulmonary fibrosis
    • Taniguchi H, Ebina M, Kondoh Y, et al. Pirfenidone in idiopathic pulmonary fibrosis. Eur Respir J 2010;35:821-9
    • (2010) Eur Respir J , vol.35 , pp. 821-829
    • Taniguchi, H.1    Ebina, M.2    Kondoh, Y.3
  • 91
    • 79960556065 scopus 로고    scopus 로고
    • The clinical significance of 5% change in vital capacity in patients with idiopathic pulmonary fibrosis: Extended analysis of the pirfenidone trial
    • Taniguchi H, Kondoh Y, Ebina M, et al. The clinical significance of 5% change in vital capacity in patients with idiopathic pulmonary fibrosis: extended analysis of the pirfenidone trial. Respir Res 2011;12:93, 9921-12-93
    • (2011) Respir Res , vol.12 , Issue.93 , pp. 992112-992193
    • Taniguchi, H.1    Kondoh, Y.2    Ebina, M.3
  • 92
    • 81255146512 scopus 로고    scopus 로고
    • Exploratory analysis of a phase III trial of pirfenidone identifies a subpopulation of patients with idiopathic pulmonary fibrosis as benefiting from treatment
    • Azuma A, Taguchi Y, Ogura T, et al. Exploratory analysis of a phase III trial of pirfenidone identifies a subpopulation of patients with idiopathic pulmonary fibrosis as benefiting from treatment. Respir Res 2011;12:143, 9921-12-143
    • (2011) Respir Res , vol.12 , Issue.143 , pp. 992112-992143
    • Azuma, A.1    Taguchi, Y.2    Ogura, T.3
  • 93
    • 84867131951 scopus 로고    scopus 로고
    • Effects of lecithinized superoxide dismutase and/or pirfenidone against bleomycin-induced pulmonary fibrosis
    • Tanaka K, Azuma A, Miyazaki Y, et al. Effects of lecithinized superoxide dismutase and/or pirfenidone against bleomycin-induced pulmonary fibrosis. Chest 2012;142:1011-9
    • (2012) Chest , vol.142 , pp. 1011-1019
    • Tanaka, K.1    Azuma, A.2    Miyazaki, Y.3
  • 94
    • 84892405113 scopus 로고    scopus 로고
    • [Internet]. Washington, D.C.: National Institutes of Health Available from [Updated 12 May 2011; Cited 6 February 2006]
    • Three-arm study of the safety and efficacy of pirfenidone in patients with idiopathic pulmonary fibrosis. [Internet]. Washington, D.C.: National Institutes of Health. 2011. Available from: http://www.clinicaltrials.gov/ct2/ show/NCT00287716term=NCT00287716&rank=1 [Updated 12 May 2011; Cited 6 February 2006]
    • (2011) Three-arm Study of the Safety and Efficacy of Pirfenidone in Patients with Idiopathic Pulmonary Fibrosis
  • 95
    • 79955559412 scopus 로고    scopus 로고
    • Amelioration of dermal fibrosis by genetic deletion or pharmacologic antagonism of lysophosphatidic acid receptor 1 in a mouse model of scleroderma
    • Castelino FV, Seiders J, Bain G, et al. Amelioration of dermal fibrosis by genetic deletion or pharmacologic antagonism of lysophosphatidic acid receptor 1 in a mouse model of scleroderma. Arthritis Rheum 2011; 63:1405-15
    • (2011) Arthritis Rheum , vol.63 , pp. 1405-1415
    • Castelino, F.V.1    Seiders, J.2    Bain, G.3
  • 96
    • 78549260067 scopus 로고    scopus 로고
    • Lysophosphatidic acid-activated clcurrent activity in human systemic sclerosis skin fibroblasts
    • Yin Z, Carbone LD, Gotoh M, et al. Lysophosphatidic acid-activated clcurrent activity in human systemic sclerosis skin fibroblasts. Rheumatology (Oxford) 2010;49:2290-7
    • (2010) Rheumatology (Oxford) , vol.49 , pp. 2290-2297
    • Yin, Z.1    Carbone, L.D.2    Gotoh, M.3
  • 98
    • 39349093685 scopus 로고    scopus 로고
    • - current activity and fibroblast-to-myofibroblast differentiation
    • DOI 10.1152/ajpcell.00291.2007
    • Yin Z, Tong Y, Zhu H, et al. ClC-3 is required for LPA-activated cl-current activity and fibroblast-to-myofibroblast differentiation. Am J Physiol Cell Physiol 2008;294:C535-42 (Pubitemid 351264403)
    • (2008) American Journal of Physiology - Cell Physiology , vol.294 , Issue.2
    • Yin, Z.1    Tong, Y.2    Zhu, H.3    Watsky, M.A.4
  • 99
    • 0028418608 scopus 로고
    • Scleroderma bronchoalveolar lavage fluid contains thrombin, a mediator of human lung fibroblast proliferation via induction of platelet-derived growth factor alpha-receptor
    • Ohba T, McDonald JK, Silver RM, et al. Scleroderma bronchoalveolar lavage fluid contains thrombin, a mediator of human lung fibroblast proliferation via induction of platelet-derived growth factor alpha-receptor. Am J Respir Cell Mol Biol 1994;10:405-12
    • (1994) Am J Respir Cell Mol Biol , vol.10 , pp. 405-412
    • Ohba, T.1    McDonald, J.K.2    Silver, R.M.3
  • 100
    • 84892419077 scopus 로고    scopus 로고
    • Proof of biological activity of SAR100842 in systemic sclerosis. [Internet]. Washington, D.C.: National Institutes of Health Available from [Updated 8 August 2013; Cited 24 July 2012]
    • Proof of biological activity of SAR100842 in systemic sclerosis. [Internet]. Washington, D.C.: National Institutes of Health. 2013. Available from: http://www.clinicaltrials.gov/ct2/show/NCT01651143 term= NCT01651143&rank=1 [Updated 8 August 2013; Cited 24 July 2012]
    • (2013)
  • 101
    • 84865637745 scopus 로고    scopus 로고
    • JAK-2 as a novel mediator of the profibrotic effects of transforming growth factor beta in systemic sclerosis
    • Dees C, Tomcik M, Palumbo-Zerr K, et al. JAK-2 as a novel mediator of the profibrotic effects of transforming growth factor beta in systemic sclerosis. Arthritis Rheum 2012;64:3006-15
    • (2012) Arthritis Rheum , vol.64 , pp. 3006-3015
    • Dees, C.1    Tomcik, M.2    Palumbo-Zerr, K.3
  • 102
    • 40349098060 scopus 로고    scopus 로고
    • Procoagulant signalling mechanisms in lung inflammation and fibrosis: Novel opportunities for pharmacological intervention?
    • DOI 10.1038/sj.bjp.0707603, PII 0707603
    • Chambers RC. Procoagulant signalling mechanisms in lung inflammation and fibrosis: novel opportunities for pharmacological intervention Br J Pharmacol 2008;153(Suppl 1): S367-78 (Pubitemid 351340998)
    • (2008) British Journal of Pharmacology , vol.153 , Issue.SUPPL. 1
    • Chambers, R.C.1
  • 104
    • 0035976915 scopus 로고    scopus 로고
    • Thrombin differentiates normal lung fibroblasts to a myofibroblast phenotype via the proteolytically activated receptor-1 and a protein kinase C-dependent pathway
    • Bogatkevich GS, Tourkina E, Silver RM, et al. Thrombin differentiates normal lung fibroblasts to a myofibroblast phenotype via the proteolytically activated receptor-1 and a protein kinase C-dependent pathway. J Biol Chem 2001;276:45184-92
    • (2001) J Biol Chem , vol.276 , pp. 45184-45192
    • Bogatkevich, G.S.1    Tourkina, E.2    Silver, R.M.3
  • 105
    • 4444252776 scopus 로고    scopus 로고
    • Thrombin-mediated cellular events in pulmonary fibrosis associated with systemic sclerosis (scleroderma)
    • Ludwicka-Bradley A, Bogatkevich G, Silver RM. Thrombin-mediated cellular events in pulmonary fibrosis associated with systemic sclerosis (scleroderma). Clin Exp Rheumatol 2004;22:S38-46 (Pubitemid 39162034)
    • (2004) Clinical and Experimental Rheumatology , vol.22 , Issue.3
    • Ludwicka-Bradley, A.1    Bogatkevich, G.2    Silver, R.M.3
  • 106
    • 70350539686 scopus 로고    scopus 로고
    • Dabigatran, a direct thrombin inhibitor, demonstrates antifibrotic effects on lung fibroblasts
    • Bogatkevich GS, Ludwicka-Bradley A, Silver RM. Dabigatran, a direct thrombin inhibitor, demonstrates antifibrotic effects on lung fibroblasts. Arthritis Rheum 2009;60:3455-64
    • (2009) Arthritis Rheum , vol.60 , pp. 3455-3464
    • Bogatkevich, G.S.1    Ludwicka-Bradley, A.2    Silver, R.M.3
  • 107
    • 79960905171 scopus 로고    scopus 로고
    • Epigenetic modifications: Novel therapeutic strategies for systemic sclerosis
    • Jungel A, Distler JH, Gay S, et al. Epigenetic modifications: novel therapeutic strategies for systemic sclerosis Expert Rev Clin Immunol 2011;7:475-80
    • (2011) Expert Rev Clin Immunol , vol.7 , pp. 475-480
    • Jungel, A.1    Distler, J.H.2    Gay, S.3
  • 108
    • 33745918328 scopus 로고    scopus 로고
    • Association between enhanced type I collagen expression and epigenetic repression of the FLI1 gene in scleroderma fibroblasts
    • DOI 10.1002/art.21948
    • Wang Y, Fan PS, Kahaleh B. Association between enhanced type I collagen expression and epigenetic repression of the FLI1 gene in scleroderma fibroblasts. Arthritis Rheum 2006;54:2271-9 (Pubitemid 44051084)
    • (2006) Arthritis and Rheumatism , vol.54 , Issue.7 , pp. 2271-2279
    • Wang, Y.1    Fan, P.-S.2    Kahaleh, B.3
  • 109
    • 79961162197 scopus 로고    scopus 로고
    • Decreased catalytic function with altered sumoylation of DNA topoisomerase i in the nuclei of scleroderma fibroblasts
    • Zhou X, Lin W, Tan FK, et al. Decreased catalytic function with altered sumoylation of DNA topoisomerase I in the nuclei of scleroderma fibroblasts. Arthritis Res Ther 2011;13:R128
    • (2011) Arthritis Res Ther , vol.13
    • Zhou, X.1    Lin, W.2    Tan, F.K.3
  • 110
    • 84867399017 scopus 로고    scopus 로고
    • Inhibition of sumoylation prevents experimental fibrosis
    • Khodzhigorova A, Distler A, Lang V, et al. Inhibition of sumoylation prevents experimental fibrosis. Ann Rheum Dis 2012;71:1904-8
    • (2012) Ann Rheum Dis , vol.71 , pp. 1904-1908
    • Khodzhigorova, A.1    Distler, A.2    Lang, V.3
  • 111
    • 77952946050 scopus 로고    scopus 로고
    • MicroRNA-29, a key regulator of collagen expression in systemic sclerosis
    • Maurer B, Stanczyk J, Jungel A, et al. MicroRNA-29, a key regulator of collagen expression in systemic sclerosis. Arthritis Rheum 2010;62:1733-43
    • (2010) Arthritis Rheum , vol.62 , pp. 1733-1743
    • Maurer, B.1    Stanczyk, J.2    Jungel, A.3
  • 112
    • 84862829123 scopus 로고    scopus 로고
    • MicroRNA expression abnormalities in limited cutaneous scleroderma and diffuse cutaneous scleroderma
    • Zhu H, Li Y, Qu S, et al. MicroRNA expression abnormalities in limited cutaneous scleroderma and diffuse cutaneous scleroderma. J Clin Immunol 2012;32:514-22
    • (2012) J Clin Immunol , vol.32 , pp. 514-522
    • Zhu, H.1    Li, Y.2    Qu, S.3
  • 113
    • 84875979967 scopus 로고    scopus 로고
    • The downregulation of microRNA let-7a contributes to the excessive expression of type i collagen in systemic and localized scleroderma
    • Makino K, Jinnin M, Hirano A, et al. The downregulation of microRNA let-7a contributes to the excessive expression of type I collagen in systemic and localized scleroderma. J Immunol 2013; 190:3905-15
    • (2013) J Immunol , vol.190 , pp. 3905-3915
    • Makino, K.1    Jinnin, M.2    Hirano, A.3
  • 114
    • 84859500585 scopus 로고    scopus 로고
    • Beta-catenin is a central mediator of pro-fibrotic wnt signaling in systemic sclerosis
    • Beyer C, Schramm A, Akhmetshina A, et al. Beta-catenin is a central mediator of pro-fibrotic wnt signaling in systemic sclerosis. Ann Rheum Dis 2012; 71:761-7
    • (2012) Ann Rheum Dis , vol.71 , pp. 761-767
    • Beyer, C.1    Schramm, A.2    Akhmetshina, A.3
  • 115
    • 40649105572 scopus 로고    scopus 로고
    • Increased expression of Wnt2 and SFRP4 in Tsk mouse skin: Role of Wnt signaling in altered dermal fibrillin deposition and systemic sclerosis
    • DOI 10.1038/sj.jid.5701101, PII 5701101
    • Bayle J, Fitch J, Jacobsen K, et al. Increased expression of Wnt2 and SFRP4 in tsk mouse skin: role of wnt signaling in altered dermal fibrillin deposition and systemic sclerosis. J Invest Dermatol 2008;128:871-81 (Pubitemid 351374297)
    • (2008) Journal of Investigative Dermatology , vol.128 , Issue.4 , pp. 871-881
    • Bayle, J.1    Fitch, J.2    Jacobsen, K.3    Kumar, R.4    Lafyatis, R.5    Lemaire, R.6
  • 116
    • 84863923526 scopus 로고    scopus 로고
    • Wnt/betacatenin signaling is hyperactivated in systemic sclerosis and induces smad-dependent fibrotic responses in mesenchymal cells
    • Wei J, Fang F, Lam AP, et al. Wnt/betacatenin signaling is hyperactivated in systemic sclerosis and induces smad-dependent fibrotic responses in mesenchymal cells. Arthritis Rheum 2012;64:2734-45
    • (2012) Arthritis Rheum , vol.64 , pp. 2734-2745
    • Wei, J.1    Fang, F.2    Lam, A.P.3
  • 117
    • 84861182154 scopus 로고    scopus 로고
    • Osteopontin in systemic sclerosis and its role in dermal fibrosis
    • Wu M, Schneider DJ, Mayes MD, et al. Osteopontin in systemic sclerosis and its role in dermal fibrosis. J Invest Dermatol 2012;132:1605-14
    • (2012) J Invest Dermatol , vol.132 , pp. 1605-1614
    • Wu, M.1    Schneider, D.J.2    Mayes, M.D.3
  • 118
    • 80052961642 scopus 로고    scopus 로고
    • Association of osteopontin regulatory polymorphisms with systemic sclerosis
    • Barizzone N, Marchini M, Cappiello F, et al. Association of osteopontin regulatory polymorphisms with systemic sclerosis. Hum Immunol 2011;72:930-4
    • (2011) Hum Immunol , vol.72 , pp. 930-934
    • Barizzone, N.1    Marchini, M.2    Cappiello, F.3
  • 119
    • 84859260199 scopus 로고    scopus 로고
    • Hedgehog signaling controls fibroblast activation and tissue fibrosis in systemic sclerosis
    • Horn A, Palumbo K, Cordazzo C, et al. Hedgehog signaling controls fibroblast activation and tissue fibrosis in systemic sclerosis. Arthritis Rheum 2012;64:2724-33
    • (2012) Arthritis Rheum , vol.64 , pp. 2724-2733
    • Horn, A.1    Palumbo, K.2    Cordazzo, C.3
  • 120
    • 84859503196 scopus 로고    scopus 로고
    • Inhibition of hedgehog signalling prevents experimental fibrosis and induces regression of established fibrosis
    • Horn A, Kireva T, Palumbo-Zerr K, et al. Inhibition of hedgehog signalling prevents experimental fibrosis and induces regression of established fibrosis. Ann Rheum Dis 2012;71:785-9
    • (2012) Ann Rheum Dis , vol.71 , pp. 785-789
    • Horn, A.1    Kireva, T.2    Palumbo-Zerr, K.3


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.