ライフサイエンスコーパス: scleroderma

1 teristic feature of systemic sclerosis (SSc; scleroderma).

2 hosocial aspects of systemic sclerosis (SSc; scleroderma).

3 ing, and replicates key fibrotic features of scleroderma.

4 ome (SSS), a rare autosomal dominant form of scleroderma.

5 e, 28% with limited, and 100% with localized scleroderma.

6 mportant cause of morbidity and mortality in scleroderma.

7 nt implications for clinical trial design in scleroderma.

8 gous Stem cell Transplantation International Scleroderma.

9 model for the development of paraneoplastic scleroderma.

10 ibuted to cytotoxic therapies or damage from scleroderma.

11 er diagnosis and the development of clinical scleroderma.

12 tractive alternative approach to therapy for scleroderma.

13 uspicion should be maintained, even in early scleroderma.

14 onal tissue from mice with bleomycin-induced scleroderma.

15 systemic sclerosis and in a murine model of scleroderma.

16 evelopments leading to novel therapeutics in scleroderma.

17 ovel approach to strategies directed against scleroderma.

18 oximate cause of death in most patients with scleroderma.

19 rotic cells in various conditions, including scleroderma.

20 musculoskeletal impairments in persons with scleroderma.

21 t rosiglitazone was used in a mouse model of scleroderma.

22 y profile in a select group of patients with scleroderma.

23 tes mellitus, may be potential therapies for scleroderma.

24 f rehabilitation techniques for persons with scleroderma.

25 he treatment of certain disorders, including scleroderma.

26 ases idiopathic pulmonary fibrosis (IPF) and scleroderma.

27 involvement remains a major complication of scleroderma.

28 n-induced dermal fibrosis, a murine model of scleroderma.

29 easure to skin scoring in clinical trials of scleroderma.

30 dramatically in fibrotic conditions such as scleroderma.

31 roblasts from lesional skin of patients with scleroderma.

32 to immune activation and/or inflammation in scleroderma.

33 in the management of children with localized scleroderma.

34 diabetes, idiopathic pulmonary fibrosis, and scleroderma.

35 provide a rationale for IFNAR1 inhibition in scleroderma.

36 fibroblasts such as in systemic sclerosis or scleroderma.

37 d) or to prevent cutaneous fibrosis, such as scleroderma.

38 (+) MFBs is a viable therapy for fibrosis in scleroderma.

39 what the true entity(ies) of myopathy is in scleroderma.

40 -normal skin architecture in mouse models of scleroderma.

41 itis (RA), systemic lupus erythematosus, and scleroderma.

42 in conditions such as Sjogren's syndrome and scleroderma.

43 t for the full spectrum of muscle disease in scleroderma.

44 nal skin and lung tissues from patients with scleroderma.

45 tment of dermal fibrosis in diseases such as scleroderma.

46 rythematosus (0.79%), psoriasis (0.79%), and scleroderma (0.6%).

47 In 41 children with localized scleroderma, 111 lesions were assessed on 2 separate occ

48 with systemic lupus erythematosus, 121 with scleroderma, 86 with polymyositis/dermatomyositis [PM/DM

49 Study participants had a diagnosis of scleroderma, a diagnosis of cancer, cancer, an available

50 l Chinese Medicine, had a curative effect on scleroderma, a typical fibrotic disease.

51 ave been described in patients with systemic scleroderma, an auto-immune disorder with clinical fibro

52 C levels were quantified in 54 patients with scleroderma and 18 healthy control subjects by colony-fo

53 As patients with scleroderma and antibodies against RPC1 are at increased

54 Several inflammatory diseases including scleroderma and atopic dermatitis display dermal thicken

55 omatosis, juvenile dermatomyositis, juvenile scleroderma and autoinflammatory syndromes.

56 locally within target tissue that results in scleroderma and bronchiolitis obliterans, diagnostic fea

57 racterized by tissue fibrosis manifesting as scleroderma and bronchiolitis obliterans.

58 development of fibrosis in murine models of scleroderma and cirrhosis.

59 latest epidemiologic data linking cancer and scleroderma and explore a model for the development of p

60 a decline in lung function in patients with scleroderma and interstitial lung disease (ILD).

61 tinib, dasatinib, and nilotinib treatment of scleroderma and normal fibroblasts leads to decreased pr

62 mproving mesenchymal stromal cell therapy in scleroderma and other diseases.

63 correlated with fibrotic disorders, such as scleroderma and progressive systemic sclerosis, the dire

64 sults offer insight into the pathogenesis of scleroderma and provide support for the idea that acquir

65 ltiple autoimmune diseases, including lupus, scleroderma and Sjogren's syndrome, and had a prominent

66 poatrophy via PPAR-gamma in a mouse model of scleroderma and suggest that pharmacological PPAR-gamma

67 e a major therapeutic target for fibrosis in scleroderma and treatment with blocking MFBs could produ

68 well as fibrotic skin lesions from localized scleroderma and uninvolved skin (n = 6).

69 ity and severity of systemic sclerosis (SSc, scleroderma) and are responsible for a greater health bu

70 nown etiology manifested by dermal fibrosis (scleroderma) and excessive connective tissue deposition

71 asian patients with systemic sclerosis (SSc; scleroderma) and in healthy individuals, particularly th

72 ty and mortality in systemic sclerosis (SSc; scleroderma), and interstitial lung disease (ILD) is the

73 ients with idiopathic pulmonary fibrosis and scleroderma, and investigate the pathological consequenc

74 tations of cutaneous lupus, dermatomyositis, scleroderma, and rheumatoid arthritis.

75 highlight the importance of muscle edema in scleroderma, and that aldolase may be a useful biomarker

76 nderstanding the key pathogenetic aspects of scleroderma, and these have led to potential targeted th

77 asurements of skin hardness in patients with scleroderma are reliable, simple, accurate, demonstrate

78 gressive forms of tissue fibrosis, including scleroderma, are characterized by an accumulation of act

79 bust GC reaction, but not in cGVHD mice with scleroderma as the prominent manifestation.

80 guishing features between scleromyxedema and scleroderma, as a common mimic.

81 ical paradigms and therapeutic strategies in scleroderma-associated interstitial lung disease and in

82 inclusion, which leads to the generation of scleroderma-associated LH2(long) messenger RNA (mRNA).

83 itis, including the antisynthetase syndrome, scleroderma-associated myopathy, antisignal recognition

84 eventeen of the 25 patients had PAH: 11 with scleroderma-associated PAH, and six with idiopathic PAH.

85 Scleroderma-associated pulmonary arterial hypertension (

86 insight into likely pathogenic mechanisms in scleroderma-associated pulmonary fibrosis.

87 atification, pathogenesis, and management of scleroderma-associated renal disease.

88 gous Stem Cell Transplantation International Scleroderma (ASTIS) trial, a phase 3, multicenter, rando

89 Malignancies are reported in scleroderma at an increased rate, but the magnitude of t

90 The median duration of scleroderma at cancer diagnosis differed significantly b

91 However, the scleroderma autoantibody profile is very different betwe

92 disorder that shares clinical features with scleroderma but has important distinguishing features in

93 ctively; P < .01 for each) and subjects with scleroderma but not PAH (median, 6.5 seconds; 25th-75th

94 [29 women; median age, 55.4 years], 11 with scleroderma but not PAH [seven women; median age, 58.9 y

95 ure confers an increased risk for developing scleroderma, but this exposure accounts for a very small

96 lucidate possible pathogenetic mechanisms in scleroderma by analysis of gene expression patterns of p

97 made, validated strategies for assessment of scleroderma cardiac disease are not yet well established

98 ibrosis-associated LH2(long) mRNA in primary scleroderma cells may suggest a novel approach to strate

99 ented patients followed at the Johns Hopkins Scleroderma Center.

100 al relationship between cancer diagnosis and scleroderma clinical onset has focused attention on the

101 t (the University of California, Los Angeles Scleroderma Clinical Trial Consortium GIT 2.0 [UCLA SCTC

102 d outcome measures have been validated (UCLA Scleroderma Clinical Trial Consortium GIT 2.0 and NIH PR

103 LAR Scleroderma Trials and Research, and the Scleroderma Clinical Trials Consortium confirm angiotens

104 isease (ILD) - a systemic sclerosis (SSc, or scleroderma) clinical phenotype which is the leading cau

105 herapeutically for any conditions other than scleroderma, close monitoring of blood pressure and rena

106 and prognostic markers in large contemporary scleroderma cohorts.

107 Measurement of skin disease in scleroderma continues to be a challenge, and there is a

108 lidated outcome measures (i.e. the localized scleroderma cutaneous assessment tool) as well as consen

109 The myeloablative Scleroderma Cyclophosphamide versus Transplant instead i

110 us far, specific therapeutic targets include scleroderma, cystic fibrosis, dermatomyositis, and lupus

111 ween 1972 and 2007 as part of the Pittsburgh Scleroderma Database.

112 common and aetiologically mysterious form of scleroderma (defined as pathological fibrosis of the ski

113 RK procontractile pathway is dysregulated in scleroderma dermal fibroblasts.

114 eduled for idiopathic pulmonary fibrosis and scleroderma dermal fibrosis.

115 promote fibrosis in systemic sclerosis (SSc; scleroderma) dermal fibroblasts, and such cells in scler

116 to examine the temporal relationship between scleroderma development and malignancy, and to evaluate

117 transcript of LH2 (LH2[long]), are linked to scleroderma disease.

118 ronic progressive fibrotic diseases, such as scleroderma, due to lack of effective therapies.

119 GVHD) approximates an inflammatory subset of scleroderma estimated at 17% to 36% of patients analyzed

120 Seven hundred ten SSc families from the Scleroderma Family Registry and DNA Repository (Sclerode

121 w of 1,379 patients with SSc enrolled in the Scleroderma Family Registry and DNA Repository and/or th

122 phea in Adults and Children (MAC) cohort and Scleroderma Family Registry and DNA Repository.

123 educes collagen induction in fibroblasts and scleroderma fibroblasts have lower constitutive levels o

124 In scleroderma fibroblasts, a double TIA-1/TIAL1 knockdown

125 proteins that normally are overexpressed by scleroderma fibroblasts, including integrin alpha4 and i

126 H2 (long) is reported to be overexpressed in scleroderma fibroblasts, the regulation of LH2 splicing

127 is significantly increased in subjects with scleroderma following PAH development.

128 The Scleroderma Gastrointestinal Tract 1.0 (SSC-GIT 1.0) sur

129 ecently published breast and lung cancer and scleroderma GWASs to explore the association between the

130 e studies using large case-control series in scleroderma have been reported.

131 Other outcome data collected included the Scleroderma Health Assessment Questionnaire.

132 Early detection of scleroderma heart disease will allow exploration of nove

133 ated pulmonary hypertension in patients with scleroderma, however the mechanisms underlying this asso

134 g these include H syndrome, characterized by scleroderma, hyperpigmentation, hypertrichosis, hepatome

135 termine the advances made in the genetics of scleroderma in candidate gene association studies.

136 c that has been previously used in models of scleroderma in mice.

137 relationship between the onset of cancer and scleroderma in patients with antibodies to RNA polymeras

138 Bleomycin-induced scleroderma in the mouse was accompanied by increased Eg

139 el to account for persistent fibrogenesis in scleroderma, in which activation of fibroblast TLR4 sign

140 Using a novel mouse model of scleroderma induced by immunization with topoisomerase-I

141 ing pathogenesis of systemic sclerosis (SSc; scleroderma) involves a complex interplay of inflammatio

142 lood flow cytometry data (Immune Response In Scleroderma, IRIS) from consented patients followed at t

143 Scleroderma is a chronic autoimmune rheumatic disease as

144 Scleroderma is a group of skin-fibrosing diseases for wh

145 Scleroderma is a lethal autoimmune disease characterized

146 Scleroderma is an autoimmune connective tissue disease i

147 Scleroderma is an autoimmune rheumatic disorder accompan

148 A feature of the skin fibrosis typical of scleroderma is atrophy of the dermal white adipose tissu

149 Thus, an inflammatory subset of scleroderma is driven by IL-13 and may benefit from IL-1

150 One hallmark of scleroderma is excessive accumulation of collagen accomp

151 Systemic sclerosis (SSc; scleroderma) is a systemic connective tissue disease wit

152 Systemic sclerosis (SSc or scleroderma) is an auto-immune disease characterized by

153 Systemic sclerosis (SSc; scleroderma) is characterized by a unique widespread vas

154 The lung in systemic sclerosis (scleroderma) is susceptible to fibrosis and the ensuing

155 OF REVIEW: Morphea, also known as localized scleroderma, is a disorder of excessive collagen deposit

156 Systemic sclerosis (SSc), or scleroderma, is a heterogeneous and complex autoimmune d

157 Systemic sclerosis (SSc), also known as scleroderma, is a rare connective tissue disease charact

158 Systemic sclerosis, also called scleroderma, is an immune-mediated rheumatic disease tha

159 Morphea, also known as localized scleroderma, is characterized by predominant skin involv

160 signatures in autoimmune diseases, including scleroderma, led us to investigate the pathological role

161 teristic IFN response gene signature seen in scleroderma lesions might therefore signify a tissue-aut

162 features of interface dermatitis followed by scleroderma-like changes.

163 treatment of fibrosing lung diseases such as scleroderma lung disease and idiopathic pulmonary fibros

164 diseases associated with fibrosis, including scleroderma lung disease, are characterized by the accum

165 Rodnan skin score and a higher likelihood of scleroderma lung disease.

166 when treated with dabigatran (1 microg/ml), scleroderma lung myofibroblasts produced 6-fold less alp

167 lung fibroblasts resembling the phenotype of scleroderma lung myofibroblasts.

168 edicted in patients receiving placebo in the Scleroderma Lung Study and to evaluate possible factors

169 A retrospective analysis of the Scleroderma Lung Study data identified the severity of r

170 analyses were retrospectively applied to the Scleroderma Lung Study data in order to identify baselin

171 differences (MCID) for FVC% predicted in the Scleroderma Lung Study I and II.

172 Completion of the CYC arm of the Scleroderma Lung Study was associated with a placebo-adj

173 Among patients with SSc-ILD in the Scleroderma Lung Study, the rates of progression of lung

174 as focused attention on the possibility that scleroderma may be a paraneoplastic syndrome in a subset

175 itis, systemic lupus erythematosus, systemic scleroderma, mixed connective tissue disease, juvenile d

176 In a Th1-dependent and Th17-associated scleroderma model, GS-649443 effectively treated mice wi

177 t reduced active chronic GVHD in both BO and scleroderma models.

178 ic effects could be observed in one of these scleroderma models.

179 y endstage fibrotic diseases, including IPF; scleroderma; myelofibrosis; kidney-, pancreas-, and hear

180 Scleroderma myopathy is a heterogeneous group of muscle

181 significance of chronic renal impairment in scleroderma need to be better defined.

182 ) were significantly lower than those in the scleroderma non-PAH (median RV MPRI, 2.5 [25th-75th perc

183 nflammation and fibrosis in diseases such as scleroderma or atopic dermatitis.

184 thy control sera and sera from patients with scleroderma or rheumatoid arthritis.

185 rolled in the Genetics versus Environment in Scleroderma Outcome Study (GENISOS) cohort and 97 matche

186 ry and/or the Genetics versus Environment in Scleroderma Outcome Study cohort.

187 vement in the Genetics versus Environment in Scleroderma Outcome Study cohort.

188 Methotrexate is the treatment of choice for scleroderma overlap syndromes, whereas mycophenolate and

189 ed by early-life exposures may contribute to scleroderma pathogenesis, and warrant in-depth character

190 h factor-beta (TGF-beta) plays a key role in scleroderma pathogenesis.

191 hese causes of pulmonary hypertension in the scleroderma patient is essential because the initiation

192 n used as a screening procedure in a typical scleroderma patient population, it is projected that tho

193 enal impairment affects approximately 50% of scleroderma patients and may be associated with other va

194 e trial, 9 investigators examined the same 5 scleroderma patients by MRSS and durometry.

195 lthough approximately one-third of sera from scleroderma patients contained detectable autoantibodies

196 , we showed that IL-13 pathway activation in scleroderma patients correlated with clinical skin score

197 ch revealed markedly decreased EPC levels in scleroderma patients relative to healthy subjects.

198 were increased in EPCs freshly isolated from scleroderma patients relative to that obtained from heal

199 en identified in malignant tissue from these scleroderma patients suggesting that autoantigen express

200 RNA polymerase I/III, which is distinct from scleroderma patients with other autoantibody specificiti

201 Scleroderma patients with progressive skin fibrosis disp

202 those from idiopathic pulmonary fibrosis and scleroderma patients, demonstrate similar heterogeneity

203 oteins found to be significantly elevated in scleroderma patients, none of the large panel of plasma

204 in both the monocytes and CD4 lymphocytes of scleroderma patients, together with the detection of IFN

205 Studying cancers from scleroderma patients, we found genetic alterations of th

206 demonstrated an increased risk of cancer in scleroderma patients.

207 ne response and drive disease in a subset of scleroderma patients.

208 Sera were obtained from healthy subjects and scleroderma patients.

209 XO3A was also observed in the vasculature of scleroderma patients.

210 D4+ T cells were differentially expressed in scleroderma patients.

211 for the decreased circulating EPC levels in scleroderma patients.

212 on that predicts changes in lung function in scleroderma patients.

213 ointestinal evaluation in morphea (localized scleroderma) patients.

214 cular interactions are characteristic of the scleroderma phenotype.

215 GF ligand and receptor was consistent with a scleroderma phenotype.

216 rinary tract infections (CLL/SLL), localized scleroderma, pneumonia, and gastrohepatic infections (ot

217 pproximately two-thirds of all patients with scleroderma present with three dominant autoantibody sub

218 During scleroderma progression, resident fibroblasts undergo ac

219 eroderma Family Registry and DNA Repository (Scleroderma Registry) were examined, and 18 multicase fa

220 are idiopathic pulmonary fibrosis (IPF) and scleroderma-related interstitial lung disease (SSc-ILD).

221 led patients from 14 US medical centres with scleroderma-related interstitial lung disease meeting de

222 e has been shown to alter the progression of scleroderma-related interstitial lung disease when compa

223 Treatment of scleroderma-related interstitial lung disease with mycop

224 de and mycophenolate mofetil for progressive scleroderma-related interstitial lung disease, and the p

225 ding that is recapitulated in the setting of scleroderma-related interstitial lung disease.

226 an survival time of 1 year for patients with scleroderma-related pulmonary arterial hypertension.

227 ay lend new insight into the pathogenesis of scleroderma-related pulmonary fibrosis.

228 complications in systemic sclerosis includes scleroderma renal crisis (SRC), normotensive renal crisi

229 Over half of cases of scleroderma renal crisis require dialysis; just under ha

230 recent articles have reported the course of scleroderma renal crisis, and examined risk factors, cli

231 Some manifestations of the disease, such as scleroderma renal crisis, pulmonary arterial hypertensio

232 ly impacted by older age of onset, male sex, scleroderma renal crisis, pulmonary fibrosis, pulmonary

233 Use of corticosteroids is associated with scleroderma renal crisis.

234 scleroderma (systemic sclerosis) and include scleroderma renal crisis.

235 rogressive bronchiolitis obliterans (BO) and scleroderma, respectively, for which new treatments are

236 ases, the cell type-specific analyses of our scleroderma samples showed expression of genes suggestin

237 d in EPCs after culturing in the presence of scleroderma sera compared with normal sera.

238 iguingly, depletion of the IgG fraction from scleroderma sera completely abolished the apoptotic effe

239 ng EPCs were reduced in scleroderma, whether scleroderma sera could induce EPC apoptosis, and, if so,

240 Furthermore, scleroderma sera inhibited the activation/phosphorylatio

241 Scleroderma serum-induced EPC apoptosis is mediated chie

242 XO3a and Bim knockdown substantially reduced scleroderma serum-induced EPC apoptosis.

243 Fibrotic diseases such as scleroderma, severe chronic asthma, pulmonary fibrosis,

244 n expression in adult normal human (ANF) and scleroderma (SF) fibroblasts.

245 of lesional skin and lung from patients with scleroderma showed increased Egr-1 levels, which were hi

246 PDGFR, these antibodies were not specific to scleroderma, since they were also detected in a similar

247 me (Standardized incidence ratio [SIR]8.14), scleroderma (SIR 7.00), rheumatoid arthritis (SIR5.96),

248 d individually (rheumatoid arthritis; lupus; scleroderma; Sjogren Syndrome; dermatomyositis/polymyosi

249 s were significantly elevated in a subset of scleroderma skin biopsies.

250 mRNA and protein were elevated in explanted scleroderma skin fibroblasts in vitro.

251 because of cell death, in 2 murine models of scleroderma skin fibrosis.

252 derma) dermal fibroblasts, and such cells in scleroderma skin lesions produce excessive reactive oxyg

253 levels in vascular and perivascular cells in scleroderma skin samples.

254 ation correlated with the modified Localized Scleroderma Skin Severity Index (r = 0.44, P = 0.0001),

255 a tool to manipulate cellular LH2 levels in scleroderma so that potential intervention therapies may

256 We propose that malignancy may initiate the scleroderma-specific immune response and drive disease i

257 in a homogeneous population of patients with scleroderma spectrum disorders at risk of developing pul

258 emodynamic response in at-risk patients with scleroderma spectrum disorders who did not have resting

259 determined by right heart catheterization in scleroderma spectrum disorders.

260 eritable PAH, and in PAH associated with the scleroderma spectrum of diseases or with anorexigen use.

261 ignaling is the major factor contributing to scleroderma (SSc) fibrosis.

262 The pathogenesis of scleroderma (SSc) includes components of autoimmunity, v

263 Scleroderma (SSc) is a complex and heterogeneous connect

264 Systemic sclerosis or scleroderma (SSc) is a complex autoimmune connective tis

265 Scleroderma (SSc) is a complex disease that involves act

266 Systemic scleroderma (SSc) is an autoimmune disease that affects

267 osis and vasculopathy in systemic sclerosis, scleroderma (SSc) largely mediated through the developme

268 eadly complication of fibrotic diseases like scleroderma (SSc).

269 leading cause of morbidity and mortality in scleroderma (SSc).

270 Cardiac involvement in systemic sclerosis (scleroderma [SSc]) adversely affects long-term prognosis

271 Systemic sclerosis (scleroderma, SSc) is a devastating fibrotic disease with

272 act (GIT) involvement in systemic sclerosis (scleroderma, SSc) is the most common internal complicati

273 nile dermatomyositis, and juvenile localized scleroderma stand out among the connective tissue diseas

274 and gene regions have now been identified as scleroderma susceptibility loci.

275 l as TBX21 and STAT4 interact with regard to scleroderma susceptibility.

276 e esophageal involvement in the two forms of scleroderma (systemic and localized), compare the same a

277 Scleroderma (systemic sclerosis [SSc]) is a complex conn

278 nd connective tissue growth factor (CTGF) in scleroderma (systemic sclerosis [SSc]) lung fibroblasts.

279 Scleroderma (systemic sclerosis [SSc]), is characterized

280 pathway in many chronic diseases, including scleroderma (systemic sclerosis [SSc]).

281 Renal complications are important in scleroderma (systemic sclerosis) and include scleroderma

282 isease and reducing functional disability in scleroderma (systemic sclerosis; SSc).

283 ssage fibroblasts derived from patients with scleroderma, the knockdown of Fox-2 protein significantl

284 reatment that is unequivocally effective for scleroderma, there have been some promising developments

285 ean League Against Rheumatism (EULAR), EULAR Scleroderma Trials and Research, and the Scleroderma Cli

286 red during a multicenter treatment trial for scleroderma using handheld digital durometers with a con

287 neration/repair and thus may protect against scleroderma vasculopathy.

288 nflammation, wound healing, pain, psoriasis, scleroderma, warts, and skin cancer.

289 n the skin, although clinical improvement of scleroderma was only seen in one model.

290 rstand the critical early events in GVHD and scleroderma, we are studying a murine model that uses di

291 Using the model of bleomycin-induced scleroderma, we found that the early influx of inflammat

292 nt manifestation in systemic sclerosis (SSc, scleroderma) where it portends a poor prognosis.

293 ine whether circulating EPCs were reduced in scleroderma, whether scleroderma sera could induce EPC a

294 cle disorders among patients with underlying scleroderma which requires robust studies to clarify the

295 ssion of Egr-1 was elevated in patients with scleroderma, which suggests that Egr-1 may be involved i

296 iscusses the characterization of myopathy in scleroderma with a focus on new developments in imaging,

297 y epitope discovery to examine patients with scleroderma with or without known autoantibody specifici

298 ent information about cardiac dysfunction in scleroderma, with special emphasis on its detection and

299 ing 3 (RNPC3) that is found in patients with scleroderma without known specificities and is absent in

300 The remaining eight patients had scleroderma without PAH.