ライフサイエンスコーパス: IPF

1 IPF and normal lung fibroblasts differentially expressed

2 IPF made up 88 (18%) of all 490 MDTM first-choice diagno

3 IPF reduced false site-localization by more than sevenfo

4 Transcriptomic analysis of 134 IPF patients revealed that low expression of Twist1 was

5 se mortality (20 [7%] vs 22 [7%], p=0.8947), IPF-related mortality (11 [4%] vs 17 [5%]; p=0.4251), ab

6 (IPF) are more frequent than those for acute IPF exacerbations and are associated with poor outcomes.

7 Coarseness of fibrosing ILD (P = .011) and IPF diagnosis (P = .016) were independently associated w

8 ged infections and exacerbations in COPD and IPF.

9 e addressing the association between GER and IPF, and also identify future research priorities that c

10 ked TGFbeta1 induction of CDH2 in normal and IPF fibroblasts, and antagomiR-630 abrogated the effect

11 pulmonary fibrosis clinically classified as IPF according to American Thoracic Society/European Resp

12 sceptibility to age-related diseases such as IPF.

13 MI; in kg/m(2)); 31.7; 90% men] had baseline IPF and EPF (mean +/- SD) volumes of 172.4 +/- 53.3 mL a

14 ced weight loss substantially decreased both IPF and EPF volumes.

15 EMT failed to reach the values exhibited by IPF myofibroblasts in all parameters examined.

16 n the peripheral blood of well-characterized IPF patients and normal volunteers.

17 lveolar lavage obtained as part of the COMET-IPF (Correlating Outcomes with Biochemical Markers to Es

18 rom 30 to 65%, P = 0.0016 of high confidence IPF diagnosis, in the BLC group and SLB group, respectiv

19 ited patients aged 45-85 years with definite IPF diagnosed prior to 3 years of screening from 183 hos

20 changes predisposing individuals to develop IPF.

21 inimal protein signature that differentiated IPF patients from normal controls, which may allow for a

22 re of chronic obstructive pulmonary disease, IPF, or normal histology lungs using RNA-seq (n = 87).

23 ve insight into biological processes driving IPF.

24 .We sought to evaluate intrapericardial-fat (IPF) and extrapericardial-fat (EPF) changes during weigh

25 of murine lung macrophages and fibroblasts, IPF lung fibroblasts, and its contribution to experiment

26 , in comparison with donor lung fibroblasts, IPF lung fibroblasts exhibited decreased sensitivity to

27 patients with idiopathic pulmonary fibrosis (IPF) (men, 366; women, 90; median age, 72 years [range,

28 al exposures, idiopathic pulmonary fibrosis (IPF) and chronic obstructive pulmonary disease are usual

29 ng symptom of idiopathic pulmonary fibrosis (IPF) and is difficult to treat.

30 ting diseases idiopathic pulmonary fibrosis (IPF) and scleroderma.

31 ) features of idiopathic pulmonary fibrosis (IPF) and to gain insight into the way IPF proceeds throu

32 patients with idiopathic pulmonary fibrosis (IPF) are more frequent than those for acute IPF exacerba

33 outcome, and idiopathic pulmonary fibrosis (IPF) can be regarded as an exemplar.

34 iagnosed with idiopathic pulmonary fibrosis (IPF) in clinical practice could participate in the INPUL

35 done to treat idiopathic pulmonary fibrosis (IPF) in Europe.

36 RATIONALE: Idiopathic pulmonary fibrosis (IPF) involves the accumulation of alpha-smooth muscle ac

37 Idiopathic pulmonary fibrosis (IPF) is a chronic age-related lung disease with high mor

38 RATIONALE: Idiopathic pulmonary fibrosis (IPF) is a chronic fatal lung disease with dismal prognos

39 Idiopathic pulmonary fibrosis (IPF) is a chronic progressive lung disease with high mor

40 Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive and fatal fibrotic lung d

41 Idiopathic pulmonary fibrosis (IPF) is a deadly chronic lung disease.

42 Idiopathic pulmonary fibrosis (IPF) is a devastating disease that remains refractory to

43 Idiopathic pulmonary fibrosis (IPF) is a devastating lung disorder with increasing inci

44 Idiopathic pulmonary fibrosis (IPF) is a disease characterized by the accumulation of a

45 Idiopathic pulmonary fibrosis (IPF) is a fatal disease characterized by interstitial re

46 Idiopathic pulmonary fibrosis (IPF) is a form of progressive interstitial lung disease

47 Idiopathic pulmonary fibrosis (IPF) is a lethal, chronic, progressive disease character

48 Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal interstitial lung diseas

49 Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal interstitial pneumonia.

50 Idiopathic pulmonary fibrosis (IPF) is a progressive clinical syndrome of fatal outcome

51 Idiopathic pulmonary fibrosis (IPF) is a progressive disease with a prevalence of 1 mil

52 Idiopathic pulmonary fibrosis (IPF) is a progressive parenchymal lung disease of comple

53 Idiopathic pulmonary fibrosis (IPF) is a progressive, fatal disorder with a variable di

54 Idiopathic pulmonary fibrosis (IPF) is a progressive, fatal interstitial lung disease (

55 RATIONALE: Idiopathic pulmonary fibrosis (IPF) is an increasingly recognized, often fatal lung dis

56 Idiopathic pulmonary fibrosis (IPF) is progressive and rapidly fatal.

57 cal course of idiopathic pulmonary fibrosis (IPF) is unpredictable.

58 of normal and idiopathic pulmonary fibrosis (IPF) lung fibroblasts.

59 sed as having idiopathic pulmonary fibrosis (IPF) or chronic (fibrotic) hypersensitivity pneumonitis,

60 ity influence idiopathic pulmonary fibrosis (IPF) progression.

61 diagnosis of idiopathic pulmonary fibrosis (IPF) versus not IPF for MDTMs, clinicians, and radiologi

62 Idiopathic pulmonary fibrosis (IPF), a chronic and progressive fibrosing interstitial p

63 with COPD or idiopathic pulmonary fibrosis (IPF), as well as in cigarette smoke-exposed mice.

64 diagnosis of idiopathic pulmonary fibrosis (IPF), based on a systematic search of the medical litera

65 rogression of idiopathic pulmonary fibrosis (IPF), it remains a debilitating and lethal condition.

66 patients with idiopathic pulmonary fibrosis (IPF), whereas PIAS4 protein levels are significantly red

67 patients with idiopathic pulmonary fibrosis (IPF), which is a very aggressive fibrotic disorder.

68 rogression in idiopathic pulmonary fibrosis (IPF).

69 eases such as idiopathic pulmonary fibrosis (IPF).

70 thogenesis of idiopathic pulmonary fibrosis (IPF).

71 y progressive idiopathic pulmonary fibrosis (IPF).

72 rogression of idiopathic pulmonary fibrosis (IPF).

73 thogenesis of idiopathic pulmonary fibrosis (IPF).

74 thogenesis of idiopathic pulmonary fibrosis (IPF).

75 thogenesis of idiopathic pulmonary fibrosis (IPF); however, the repertoire of autoantigens involved i

76 This study provides a basis for IPF therapeutic intervention by modulating PIAS4 protein

77 ation of AKAP13 as a susceptibility gene for IPF increases the prospect of successfully targeting Rho

78 ement on diagnostic likelihoods was good for IPF (kappaw=0.71 [IQR 0.64-0.77]) and connective tissue

79 al or CT findings that are indeterminate for IPF.

80 lacebo-controlled studies of pirfenidone for IPF (the two CAPACITY [Clinical Studies Assessing Pirfen

81 R pathway may have therapeutic potential for IPF.

82 o analytes were elevated, the odds ratio for IPF increased to 5.0 (95% confidence interval, 2.2-11.5;

83 ment of efficient therapeutic strategies for IPF is an important future endeavor.

84 provide a potential therapeutic strategy for IPF.

85 stigated as a novel therapeutic strategy for IPF.

86 nce of some agents as novel therapeutics for IPF.

87 eases, may represent potential therapies for IPF.

88 ed the development of targeted therapies for IPF.

89 The MAFs were similar to those for IPF (UCSF 33.3%, p=0.09; UTSW 32.0%, p=0.95).

90 ng diseases, including its most common form, IPF, remains poorly understood.

91 We found IPF plasma to be altered and enriched for proteins invol

92 Consistent with induction of p53, AECs from IPF lungs or mice with diverse types of lung injuries sh

93 t in vitro proliferation of CD4 T cells from IPF patients and enhanced the production of IL-4, IL-17,

94 und in epithelial cells and fibroblasts from IPF lungs compared with age-matched cells from normal lu

95 Compared to normals, plasma from IPF patients showed significantly higher hemolytic activ

96 ffering new hope for patients suffering from IPF.

97 pathogenesis of pulmonary fibrosis in human IPF and in an experimental mouse model.

98 In vivo, injection of human IPF MPCs converted a self-limited bleomycin-induced mous

99 perimental lung injury and in cases of human IPF.

100 In a pooled analysis of three phase III IPF clinical trials, patients receiving pirfenidone had

101 eeks using data derived from three phase III IPF clinical trials.

102 In IPF tissue, fibroblastic foci contained cells expressing

103 s 2.3-fold increased (95% CI = 1.91-2.71) in IPF lung tissue (P < 0.0001).

104 o clear information on the role of VEGF-A in IPF.

105 that regulate ADORA2B expression on AAMs in IPF is not well understood.

106 XRalpha in experimental lung fibrosis and in IPF lung fibroblasts reduced the exacerbated fibrotic re

107 use lung fibroblasts and macrophages, and in IPF lung fibroblasts, using loss-and-gain of function as

108 evated abundance of senescence biomarkers in IPF lung, with p16 expression increasing with disease se

109 associated with dysregulation of T cells in IPF patients.

110 for a more granular insight into changes in IPF progression.

111 tudy suggests that the mechanism of cough in IPF might be disease specific.

112 to predict disease progression and death in IPF.

113 und that RXFP1 is significantly decreased in IPF.

114 isoform expression and functional effects in IPF.

115 plicated in mediating adenosine's effects in IPF.

116 UCHL5 is highly expressed in IPF lungs.

117 s in regulating inflammation and fibrosis in IPF.

118 s NUMB and PDGFA occurred more frequently in IPF or emphysema compared with control and validated the

119 ults in progressive loss of lung function in IPF.

120 ive expression of these profibrotic genes in IPF fibroblasts.

121 IgG autoantibody levels were much greater in IPF subjects from the University of Alabama at Birmingha

122 ial DNA (mtDNA), have not been identified in IPF.

123 mtDNA concentrations are increased in IPF BAL and plasma, and in the latter compartment, they

124 DSP expression is increased in IPF lung and concentrated in the airway epithelia, sugge

125 rapulmonary vimentin levels are increased in IPF patients.

126 vbeta6 integrin is dramatically increased in IPF.

127 nt stimuli for repetitive alveolar injury in IPF.

128 immune response and microbial interaction in IPF as they relate to progression-free survival (PFS), f

129 o explore the host-microbial interactions in IPF.

130 ates that this fate switching is involved in IPF pathogenesis, opening potential therapeutic avenues

131 dea that telomere dysfunction is involved in IPF pathogenesis.

132 Anti-vimentin autoantibody levels in IPF patients were HLA biased and inversely correlated wi

133 of disease and improving quality of life in IPF patients.

134 AK(Y397) may rescue normal mechanobiology in IPF.

135 differentiation, and its loss as observed in IPF facilitates profibrotic phenotypic changes.

136 ably, this pattern mirrored that observed in IPF lung tissues.

137 Y [Clinical Studies Assessing Pirfenidone in IPF: Research of Efficacy and Safety Outcomes] trials an

138 Although hypoxic conditions are present in IPF, hypoxia's role as a direct modulator of macrophage

139 anti-vimentin autoreactivity is prevalent in IPF patients and is strongly associated with disease man

140 hat target profibrotic genes up-regulated in IPF fibroblasts.

141 on between mtDNA and fibroblast responses in IPF.

142 with stiff substrates, and spontaneously in IPF fibroblasts, is associated with excessive levels of

143 a therapeutic target has not been studied in IPF.

144 n vitro, we found that the loss of Twist1 in IPF lung fibroblasts increased expression of CXCL12 down

145 ficantly less RXFP1 was detected in vitro in IPF fibroblasts than in donor controls.

146 for treatment of fibrotic diseases including IPF.

147 t many endstage fibrotic diseases, including IPF; scleroderma; myelofibrosis; kidney-, pancreas-, and

148 -free survival between these two independent IPF cohorts, serious adverse outcomes were most frequent

149 Whether the lung microbiome influences IPF host defense remains unknown.

150 Leveraging the bleomycin-injury IPF model, we demonstrate that early-intervention suicid

151 ical outcomes that provides new insight into IPF.

152 l as in plasma samples from two longitudinal IPF cohorts and demographically matched control subjects

153 After controlling for weight loss, IPF and EPF volume reduction paralleled changes in lipid

154 Unlike normal and COPD lungs, IPF lungs were characterized by significantly increased

155 Moreover, IPF alveolar macrophages had evidence of increased mitop

156 ficient to confer fibrotic properties to non-IPF MPCs.

157 tients with IPF compared with those with non-IPF ILD.

158 iopathic pulmonary fibrosis (IPF) versus not IPF for MDTMs, clinicians, and radiologists, using univa

159 markers by immunohistochemical assessment of IPF lung tissue.

160 etic risk associated with the development of IPF; however, mechanisms by which genetic risk factors p

161 because accurate and consistent diagnoses of IPF are needed if clinical outcomes are to be optimised.

162 establish confident and working diagnoses of IPF.

163 s, which may allow for accurate diagnosis of IPF based on easily-accessible peripheral blood.

164 res, and establishing a working diagnosis of IPF if lung tissue is not available.

165 A working diagnosis of IPF should be reviewed at regular intervals since the di

166 Furthermore, MDTMs made the diagnosis of IPF with higher confidence and more frequently than did

167 ole of CT is expanded to permit diagnosis of IPF without surgical lung biopsy in select cases when CT

168 that strongly associated with a diagnosis of IPF, BAL bacterial burden (determined by 16S quantitativ

169 signs of DPO may be helpful for diagnosis of IPF.

170 ase and may prove useful in the diagnosis of IPF.

171 improved knowledge of the natural history of IPF.

172 gh-confidence diagnoses (>65% likelihood) of IPF were given in 68 (77%) of 88 cases by MDTMs, 62 (65%

173 d the uPA receptor in AECs from the lungs of IPF patients, and in mice with bleomycin, cigarette smok

174 Optimal management of IPF therefore requires a comprehensive approach, which i

175 may underlie the aberrant mechanobiology of IPF fibroblasts.

176 UCHL5 may contribute to the pathogenesis of IPF and may be a potential therapeutic target.

177 otential role for DSP in the pathogenesis of IPF.

178 is considered central to the pathogenesis of IPF.

179 miR-155 reduced the profibrotic phenotype of IPF and miR-155(-/-) fibroblasts.

180 dings replicate the spontaneous phenotype of IPF fibroblasts.

181 definition 1 was an independent predictor of IPF diagnosis (P < .001) and male sex (P = .003).

182 f diagnostic subgroups on the progression of IPF and the effect of nintedanib.

183 ated with the development and progression of IPF are also associated with the development of fibrosis

184 ctors for the development and progression of IPF, but robust definitive data are few.

185 Vimentin supplementation of IPF PBMC cultures also resulted in HLA-DR-dependent prod

186 CCN1 mRNA were confirmed in lung tissues of IPF subjects undergoing lung transplantation, and CCN1 p

187 of antifibrotic agents for the treatment of IPF along with the patent literature from the past 10 ye

188 t evidence-based guidelines for treatment of IPF approved conditional recommendation of PPIs for all

189 A or ADORA2B antagonists in the treatment of IPF.

190 discussed as potential chronic treatments of IPF in 13.3 and 30.6% of the 98 websites that had been u

191 rade 3 or 4 adverse events were worsening of IPF, dyspnoea, and pneumonia.

192 espiratory tract infection, and worsening of IPF; and the most common grade 3 or 4 adverse events wer

193 Patient-directed online information on IPF is frequently incomplete, inaccurate, and outdated.

194 ites that provide appropriate information on IPF.

195 lveolar macrophages of patients with COPD or IPF.

196 Here, we present inference of peptidoforms (IPF), a fully automated algorithm that uses spectral lib

197 anisms by which genetic risk factors promote IPF remain unclear.

198 whole-body MRI and volumetrically quantified IPF and EPF among 80 participants to follow the 18-mo ch

199 Whether and how senescent cells regulate IPF or if their removal may be an efficacious interventi

200 on and thereby prevents cellular senescence, IPF and carcinoma formation.

201 osis-as significant contributors to sporadic IPF.

202 dy-wide significant contributors to sporadic IPF.

203 tively, 11.3% of case subjects with sporadic IPF carried a qualifying variant in one of these three g

204 banalysis of 186 case subjects with sporadic IPF confirmed TERT, RTEL1, and PARN as study-wide signif

205 wide transcriptional analysis, we found that IPF MPCs displayed increased expression of S100 calcium-

206 This might indicate that IPF fosters an environment for the development or perpet

207 Our findings indicate that IPF MPCs are intrinsically fibrogenic and that S100A4 co

208 In silico analyses indicated that IPF lung fibroblasts have increased expression of genes

209 Ex vivo analysis revealed that IPF MPCs had increased levels of nuclear S100A4, which i

210 After 18 mo, the IPF volume had reduced twice as much in the MED/LC group

211 s, is superior to an LF diet in terms of the IPF burden reduction.

212 Twist1 may shape the IPF phenotype and regulate inflammation in fibrotic lung

213 We previously discovered that the IPF lung harbors fibrogenic mesenchymal progenitor cells

214 ith improved glycemic profile variables: the IPF relative reduction was associated with a decrease in

215 scale unbiased protein discovery analysis to IPF and describes distinct biological processes that fur

216 Epigenetic changes contribute to IPF; therefore, microRNAs may reveal novel pathogenic pa

217 associated with increased susceptibility to IPF was also associated with increased expression of AKA

218 Due to a lack of effective treatment, IPF is associated with a high mortality rate.

219 tems biology approaches to identify a unique IPF proteome signature and give insight into biological

220 Using IPF, we quantified peptidoforms in DIA data acquired fro

221 CCN1 expression in ex vivo IPF lung fibroblasts correlated with gene expression of

222 rosis (IPF) and to gain insight into the way IPF proceeds through the lungs and progresses over time.

223 Whereas IPF fibroblasts are enriched for aerobic glycolysis and

224 gle-nucleotide polymorphisms associated with IPF (MUC5B rs35705950 and TOLLIP rs5743890) and telomere

225 to identify genetic variants associated with IPF susceptibility and provide mechanistic insight using

226 Sequence variants in DSP are associated with IPF, and rs2076295 genotype is associated with different

227 ts in the chromosome 6 locus associated with IPF.

228 -wide significant signal of association with IPF susceptibility near A-kinase anchoring protein 13 (A

229 Sixty patients diagnosed with IPF were prospectively enrolled together with 20 matched

230 comorbid conditions share risk factors with IPF, the likelihood for their presence or development in

231 Lungs from humans with IPF demonstrated increased expression of PHGDH and SHMT2

232 cted biomarkers to identify individuals with IPF who were at risk of progression or death.

233 Participants with IPF and chronic cough recruited from seven centres in th

234 2015, and Feb 2, 2016, 24 participants with IPF were randomly assigned to treatment groups.

235 pneumonitis suggest shared pathobiology with IPF, and might help to stratify risk.

236 1) was significantly higher in patients with IPF (28.5%) than in those without IPF (8.3%, P < .001).

237 ibility of a harmful effect in patients with IPF (adjusted rate of decline 125.6 mL/6 months for acet

238 tes measured in plasma from 86 patients with IPF (derivation cohort) and in 63 patients with IPF (val

239 ature identified two groups of patients with IPF (low-risk and high-risk), with significant differenc

240 large clinical trial cohort of patients with IPF (n = 1,113), we comprehensively screened multivariat

241 mes higher in lung tissue from patients with IPF (n=46) than that in lung tissue from controls (n=51)

242 (derivation cohort) and in 63 patients with IPF (validation cohort).

243 Patients with IPF aged 40-80 years and established on pirfenidone (at

244 602 patients with IPF and 3366 controls were selected for stage 1.

245 For stage 2, 2158 patients with IPF and 5195 controls were selected.

246 gulated in fibrotic lungs from patients with IPF and bleomycin-treated mice.

247 d, placebo-controlled trial in patients with IPF and chronic cough and a parallel study of similar de

248 and safety of inhaled PA101 in patients with IPF and chronic cough and, to explore the antitussive me

249 sma, and lung fibroblasts from patients with IPF and control subjects.

250 nts in independent datasets of patients with IPF and controls from two independent US samples from th

251 using lung tissue samples from patients with IPF and controls.

252 etermined in lung tissues from patients with IPF and mice with bleomycin-induced pulmonary fibrosis.

253 py did not improve outcomes in patients with IPF and might potentially be associated with an increase

254 nt option for chronic cough in patients with IPF and warrants further investigation.

255 servation suggests that either patients with IPF are somehow resistant to PPI-based intervention or P

256 nhanced diagnostic accuracy in patients with IPF compared with those with non-IPF ILD.

257 Patients with IPF diagnosed in clinical practice who had possible UIP

258 We enrolled patients with IPF diagnosis in a six-cohort study at Yale University (

259 ch significantly distinguished patients with IPF from patients with a-ILD, both individually and in a

260 ompared findings with those in patients with IPF from the UCSF and UTSW cohorts, and healthy controls

261 biomarkers that differentiated patients with IPF from those with a-ILD.

262 ed by pulmonary fibrosis, most patients with IPF have associated comorbid conditions, which might neg

263 indicate that the majority of patients with IPF have silent reflux with no symptoms of GER.

264 eir presence or development in patients with IPF is still greater than expected.

265 nome-wide association study in patients with IPF of European ancestry recruited from nine different c

266 gically normal human subjects, patients with IPF presented local induction of C5aR, local and systemi

267 recommendation of PPIs for all patients with IPF regardless of their GER status.

268 Fibrosis and cyst formation in patients with IPF seem to start at the periphery of the pulmonary lobu

269 ignificantly more prevalent in patients with IPF than in those with other fibrosing ILDs, and thus, c

270 Patients with IPF were recruited between Jan 31, 2013, and June 1, 201

271 fibrosis and lung samples from patients with IPF were used to examine the effects and interactions of

272 By contrast, patients with IPF who undergo Nissen fundoplication surgery are effect

273 Our data suggest that patients with IPF with the highest RXFP1 expression would be predicted

274 um and lymphoid follicles from patients with IPF, and AKAP13 mRNA expression was 1.42-times higher in

275 ne-2-receptor antagonists) for patients with IPF, in the absence of oesophageal reflux or symptoms.

276 In patients with IPF, PA101 reduced daytime cough frequency by 31.1% at d

277 In patients with IPF, the magnitude of RXFP1 gene expression correlated d

278 port the use of simtuzumab for patients with IPF.

279 ected in plasma of progressing patients with IPF.

280 re is predictive of outcome in patients with IPF.

281 n a well-defined population of patients with IPF.

282 ing RhoA pathway inhibitors in patients with IPF.

283 n a well-defined population of patients with IPF.

284 hepsins in fibrotic lesions of patients with IPF.

285 ising therapeutic strategy for patients with IPF.

286 ted with pulmonary function in patients with IPF.

287 lung fibroblasts obtained from patients with IPF.

288 nal antibody against LOXL2, in patients with IPF.

289 inal cohort of treatment-naive patients with IPF.

290 ation for providers caring for patients with IPF.

291 is, we examined samples from 206 people with IPF from among the remaining 212 patients recruited to P

292 omarkers to predict outcomes for people with IPF.

293 variants was conducted in 936 subjects with IPF and 936 control subjects.

294 Subjects with IPF were given handheld spirometers and instruction on h

295 For subjects with IPF, additional samples were taken at 1, 3, and 6 months

296 lar senescence markers are detectable within IPF lung tissue and senescent cell deletion rejuvenates

297 lavage (BAL) from subjects with and without IPF, as well as in plasma samples from two longitudinal

298 ients with IPF (28.5%) than in those without IPF (8.3%, P < .001).

299 , contusion, forearm fracture, and worsening IPF in the placebo group.

300 Yet IPF myofibroblasts exhibited higher stiffness and expres