ライフサイエンスコーパス: IPF

1 IPF has been associated with aberrant vascular remodelli

2 IPF lung fibroblasts transfected with anti-miR-144-3p ha

3 IPF-RC treatment further impairs ATII differentiation by

4 e patients were pneumonia (six [5%] of 111), IPF exacerbation (four [4%]), IPF progression (four [4%]

5 s isolated from the lungs of 3 control and 3 IPF patients at the single-cell level.

6 We conducted transcriptome profiling on 38 IPF subjects with time points available at 0, 12, and 24

7 [5%] of 111), IPF exacerbation (four [4%]), IPF progression (four [4%]), and chest pain (two [2%]).

8 eplication in two independent studies (1,456 IPF cases and 11,874 controls) and functional analyses (

9 wide association study of IPF to date (2,668 IPF cases and 8,591 controls).

10 subjects enrolled in the PANTHER-IPF and ACE-IPF, 62% (49/79) and 56% (28/50) had an LTL less than th

11 Replication cohorts included ACE-IPF (Anticoagulant Effectiveness in Idiopathic Pulmonary

12 ing was replicated in the placebo arm of ACE-IPF for those exposed to immunosuppression (hazard ratio

13 ith decreased pulmonary function in advanced IPF; (2) identifies IGF-1's C1 promoter as mediating the

14 lantation outcomes in patients with advanced IPF.

15 73T) mice provide a detailed ontogeny for AE-IPF driven by alveolar epithelial dysfunction that induc

16 We treated this system with an IPF-relevant cocktail (IPF-RC) to mimic the pro-fibrotic

17 promising strategy for treatment of ALI and IPF.

18 ulators of proliferation in both control and IPF fibroblasts but had no effect upon the fibrotic resp

19 le-cell RNA-sequencing data from control and IPF lungs.Measurements and Main Results: DSP is expresse

20 sion of already well-known genes of COPD and IPF across multiple experiments and the results of two d

21 lecular interaction regions between COPD and IPF and shed light on the congruent and incongruent biol

22 Applying NetPathScore to the COPD and IPF disease modules enabled the determination of concord

23 Concordant pathways between COPD and IPF included extracellular matrix remodeling, Mitogen-ac

24 pendent gene expression data set of COPD and IPF lung tissue and showed statistically significant ove

25 roach, we built disease modules for COPD and IPF using genome-wide association studies-implicated gen

26 ies have identified shared loci for COPD and IPF, including several loci with opposite directions of

27 ver pathways at the intersection of COPD and IPF, we developed a metric, NetPathScore, which prioriti

28 xperiments were done using healthy donor and IPF lungs.

29 gression of TGFbeta-induced fibrogenesis and IPF have remained unexplored.

30 ed miR-144-3p expression in both healthy and IPF lung fibroblasts in a SMAD family 2/3 (SMAD2/3)-depe

31 ER stress and fibrosis in Grp78 KO mouse and IPF lung slice cultures.Conclusions: These results suppo

32 e the role of MAP3K19 in cultured normal and IPF fibroblasts and in a humanized SCID mouse model of I

33 ed by CRISPR-Cas9 gene editing in normal and IPF fibroblasts, as well as in lung cancer-associated fi

34 map epithelial cell types of the normal and IPF human airways.

35 ly isolated epithelial cells from normal and IPF lungs to discern disease-dependent changes within ba

36 s that demonstrate a sex prevalence, such as IPF.

37 that a potential limitation to relaxin-based IPF therapy is decreased expression of a relaxin recepto

38 versity of Texas Southwestern Medical Center-IPF, n = 170).

39 -C) gene (SFTPC) has been linked to clinical IPF.

40 d this system with an IPF-relevant cocktail (IPF-RC) to mimic the pro-fibrotic cytokine milieu presen

41 4 (76%) of 111 patients received concomitant IPF therapy (pirfenidone n=55 or nintedanib n=29).

42 y six individuals, 17 of which had confirmed IPF.

43 Currently, IPF has few treatment options and no effective therapies

44 vance was demonstrated in live precision-cut IPF lung slices.

45 idence for an effect of previously described IPF loci; however, novel ILA associations were not assoc

46 tate, we found that the least differentiated IPF MPCs displayed the largest differences in their tran

47 blood and lung proteins that differentiated IPF progression status by the end of 80 weeks of follow-

48 ral proteomic signatures that differentiated IPF progressors and non-progressors.

49 ifferentially expressed genes distinguishing IPF MPCs from control (CD44, cell surface; and MARCKS, i

50 tical strategies to raise tissue PGE2 during IPF have been limited.

51 ntifibrotic agent that may lead to effective IPF treatments.

52 D44 as a characteristic of the most entropic IPF MPCs.

53 and Integrative LASSO with Penalty Factors (IPF-lasso).

54 s), including idiopathic pulmonary fibrosis (IPF) and sarcoidosis.

55 icipants with idiopathic pulmonary fibrosis (IPF) and to evaluate the reproducibility of these metric

56 new drugs for idiopathic pulmonary fibrosis (IPF) are in development.

57 ury (ALI) and idiopathic pulmonary fibrosis (IPF) are severe lung diseases causing irreversible lung

58 se (COPD) and idiopathic pulmonary fibrosis (IPF) are two pathologically distinct chronic lung diseas

59 Patients with idiopathic pulmonary fibrosis (IPF) can experience life-threatening episodes of acute w

60 Rationale: Idiopathic pulmonary fibrosis (IPF) causes considerable global morbidity and mortality,

61 Idiopathic pulmonary fibrosis (IPF) has a poor prognosis and limited treatment options.

62 se (COPD) and Idiopathic Pulmonary Fibrosis (IPF) have contrasting clinical and pathological characte

63 the origin of idiopathic pulmonary fibrosis (IPF) in patients mutant for telomere maintenance genes.

64 patients with idiopathic pulmonary fibrosis (IPF) in the PANTHER-IPF (Evaluating the Effectiveness of

65 Idiopathic pulmonary fibrosis (IPF) is a chronic and fatal disease of unknown etiology;

66 Idiopathic pulmonary fibrosis (IPF) is a chronic progressive interstitial lung disease

67 Idiopathic pulmonary fibrosis (IPF) is a chronic progressive lung disease with limited

68 Rationale: Idiopathic pulmonary fibrosis (IPF) is a complex lung disease characterized by scarring

69 Rationale: Idiopathic pulmonary fibrosis (IPF) is a deadly disease with increasingly impaired heal

70 Rationale: Idiopathic pulmonary fibrosis (IPF) is a devastating progressive disease with limited t

71 Idiopathic Pulmonary Fibrosis (IPF) is a disease with a devastating prognosis character

72 Idiopathic pulmonary fibrosis (IPF) is a disease with high 5-year mortality and few the

73 Idiopathic pulmonary fibrosis (IPF) is a fatal and incurable form of interstitial lung

74 Idiopathic pulmonary fibrosis (IPF) is a fatal disease of unknown cause that is charact

75 Idiopathic pulmonary fibrosis (IPF) is a fatal disease with a variable and unpredictabl

76 Idiopathic pulmonary fibrosis (IPF) is a lung parenchymal disease of unknown cause usua

77 Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal lung disease, and its mo

78 Idiopathic pulmonary fibrosis (IPF) is a progressive and heterogeneous interstitial lun

79 Idiopathic pulmonary fibrosis (IPF) is a progressive disease characterized by interstit

80 Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial lung disease with a m

81 Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease with a poor prognosis

82 Idiopathic pulmonary fibrosis (IPF) is a rare and devastating chronic lung disease of u

83 Idiopathic pulmonary fibrosis (IPF) is a severe form of lung fibrosis with a high morta

84 Idiopathic pulmonary fibrosis (IPF) is characterized by altered epithelial cell phenoty

85 Idiopathic pulmonary fibrosis (IPF) is characterized by exuberant deposition of extrace

86 e proteins in idiopathic pulmonary fibrosis (IPF) is not known.

87 s of sporadic idiopathic pulmonary fibrosis (IPF) is not known.Objectives: To identify the prevalence

88 ased in human idiopathic pulmonary fibrosis (IPF) lung tissue.

89 Patients with idiopathic pulmonary fibrosis (IPF) often experience precipitous deteriorations, termed

90 diagnosis of idiopathic pulmonary fibrosis (IPF) requires a pattern of usual interstitial pneumonia

91 Patients with idiopathic pulmonary fibrosis (IPF) treated with PRM-151, a recombinant human pentraxin

92 n observed in idiopathic pulmonary fibrosis (IPF), a disease of aging.Objectives: To investigate a ca

93 d severity of idiopathic pulmonary fibrosis (IPF), a fatal and irreversible interstitial lung disease

94 f hormones in idiopathic pulmonary fibrosis (IPF), a predominantly male lung disease, is unknown.Obje

95 evelopment of Idiopathic Pulmonary Fibrosis (IPF), a progressive and fatal lung disease.

96 es, including idiopathic pulmonary fibrosis (IPF), have some of the worst prognoses and affect millio

97 reatments for idiopathic pulmonary fibrosis (IPF), new insights into the deleterious mechanisms contr

98 In Idiopathic Pulmonary Fibrosis (IPF), there is unrelenting scarring of the lung mediated

99 e observed in idiopathic pulmonary fibrosis (IPF), with key risk factors yet to be identified.

100 therapies for idiopathic pulmonary fibrosis (IPF).

101 rotic foci of idiopathic pulmonary fibrosis (IPF).

102 l therapy for idiopathic pulmonary fibrosis (IPF).

103 ns, including idiopathic pulmonary fibrosis (IPF).

104 patients with idiopathic pulmonary fibrosis (IPF).

105 patients with idiopathic pulmonary fibrosis (IPF).

106 evelopment of idiopathic pulmonary fibrosis (IPF).

107 eases such as idiopathic pulmonary fibrosis (IPF).

108 ociation with idiopathic pulmonary fibrosis (IPF).

109 ses including idiopathic pulmonary fibrosis (IPF).

110 evelopment of idiopathic pulmonary fibrosis (IPF).Objectives: We sought to decipher the transcriptome

111 MAP3K19 was required for IPF myofibroblast differentiation, and targeting its act

112 ntify targets and therapeutic strategies for IPF.

113 in of IPF and that a potential treatment for IPF based on telomerase activation would be of interest

114 ical trials, to be a potential treatment for IPF.

115 Treatments for IPF are limited to anti-inflammatory and immunomodulator

116 lagen secretion in organotypic cultures from IPF patients, thus suggesting that targeting of REVERBal

117 polyplexes in primary lung fibroblasts from IPF mice showed high cellular uptake and promising antif

118 Using lung tissues from IPF and control subjects, we showed that expression of T

119 CD44 expression, we determined that CD44(hi) IPF MPCs manifested an increased capacity for anchorage-

120 n and extracellular matrix in cultured human IPF fibroblasts.

121 s to alphavbeta6 with high affinity in human IPF lung and reduces downstream pro-fibrotic TGFbeta sig

122 levels are significantly increased in human IPF patients with acute exacerbation compared to patient

123 tures reveals significant overlap with human IPF patient data.

124 In IPF lung tissue, pathological MPCs resided in the highly

125 Balpha inhibited myofibroblast activation in IPF fibroblasts and collagen secretion in organotypic cu

126 nsferrin receptor 1 (CD71)-expressing AMs in IPF is not known.

127 rogenitors of distal lung epithelium, and in IPF lung slice cultures.Measurements and Main Results: G

128 a murine model of pulmonary fibrosis and in IPF tissue samples.

129 ht represent a novel therapeutic approach in IPF.

130 Atypical alveolar epithelial cells in IPF express molecular markers of airway epithelium.

131 d expression in alveolar epithelial cells in IPF lungs.

132 s a potential role for airway basal cells in IPF.

133 omplement activation and oxidative damage in IPF patients and provides haptoglobin-related protein as

134 The short telomere defect in IPF-LTRs was associated with significantly impaired CMV-

135 trations of BAL transferrin were enhanced in IPF-BAL, and furthermore, CD71(-) AMs had an impaired ab

136 ition currently under clinical evaluation in IPF.

137 global downregulation of the TOLLIP gene in IPF lungs may predispose injured lung epithelial cells t

138 le role of mitotic spindle-assembly genes in IPF susceptibility.

139 inflammatory response, which was greater in IPF compared to control fibroblasts.

140 ain Results: ERalpha expression increased in IPF lung tissue, myofibroblasts, or BLM mice.

141 evant target for therapeutic intervention in IPF.

142 in the lung parenchyma is globally lower in IPF compared to controls.

143 on, supporting a role for lung microbiota in IPF.

144 role of DNM3OS and its associated miRNAs in IPF pathogenesis.

145 e a BAL signature that predicts mortality in IPF and improves the accuracy of outcome prediction base

146 IL-1beta and caspase-1 has been observed in IPF patients, indicating inflammasome activation associa

147 ermed bronchiolization, is often observed in IPF.

148 paving the way for a new treatment option in IPF.

149 se findings highlight the role of 15-PGDH in IPF and suggest 15-PGDH inhibition as a promising therap

150 to investigate the expression of 15-PGDH in IPF and the therapeutic potential of a specific inhibito

151 rlining the importance of this population in IPF and as a potential therapeutic target.

152 the pro-fibrotic cytokine milieu present in IPF lungs.

153 2) levels and reduces collagen production in IPF precision cut lung slices and in the bleomycin model

154 he results of this first-ever eHealth RCT in IPF showed that a comprehensive home monitoring program

155 we found that miR-144-3p is up-regulated in IPF fibroblasts compared with lung fibroblasts from heal

156 UTR of RXFP1 was significantly repressed in IPF lung fibroblasts, whereas a reporter carrying a muta

157 eates AM subsets that play distinct roles in IPF and furthermore show that CD71(-) AMs may be an impo

158 ting that miR-144-3p down-regulates RXFP1 in IPF lung fibroblasts by targeting its 3' UTR.

159 The mechanism that down-regulates RXFP1 in IPF remains unclear.

160 n-like family peptide receptor 1 (RXFP1), in IPF fibroblasts.

161 sent new effective therapeutic strategies in IPF.

162 in future low dose CO therapeutic studies in IPF.

163 of the secretory primed basal cell subset in IPF.

164 However, the functional role of TOLLIP in IPF is unknown.

165 cally interacts with nintedanib treatment in IPF fibroblasts.

166 K19) was recently shown to be upregulated in IPF and this MAPK has a key role in target gene transcri

167 LINC00960 and LINC01140 were upregulated in IPF fibroblasts.

168 high prevalence of relapsing CMV viremia in IPF-LTRs compared with non-IPF-LTRs (69% vs. 31%; odds r

169 e our understanding of factors that increase IPF susceptibility by identifying previously unreported

170 d the BAL transcriptome of three independent IPF cohorts: Freiburg (Germany), Siena (Italy), and Leuv

171 ptoms and impacts) of the Living with IPF (L-IPF) questionnaire.

172 ng levels of IPF severity.Conclusions: The L-IPF is a new questionnaire that assesses symptoms, disea

173 The L-IPF yields five scales demonstrating good psychometric p

174 endent colony formation compared to CD44(lo) IPF MPCs.

175 eration, invasion, and migration in multiple IPF fibroblast cells in vitro as well as to reduce pulmo

176 orter time to viremia versus age-matched non-IPF control subjects (P < 0.001).

177 tudied IPF-LTRs (n = 42) and age-matched non-IPF-LTRs (n = 42) and assessed CMV outcomes.

178 ng CMV viremia in IPF-LTRs compared with non-IPF-LTRs (69% vs. 31%; odds ratio, 4.98; 95% confidence

179 n model system that recapitulates aspects of IPF-associated bronchiolization of the lung epithelium i

180 ated protein as a new candidate biomarker of IPF.

181 ase mutations but also for sporadic cases of IPF associated with physiological aging.

182 cells to apoptosis and to the development of IPF.

183 plinary teams in ascertaining a diagnosis of IPF, particularly for patients without a clear radiologi

184 ne expression, and testing for enrichment of IPF susceptibility signals in regulatory regions) to det

185 The etiology of IPF is not completely understood but involves pathologic

186 Although the etiology of IPF is unknown, it is characterized by extensive alveola

187 However, the etiology of IPF remains unknown.

188 their disease, termed acute exacerbation of IPF, which may be caused by bacterial and/or viral infec

189 orisin are involved in acute exacerbation of IPF, yielding insights to the molecular basis for the el

190 bility of old and male mice, all features of IPF.

191 lapping associations in independent GWASs of IPF.Measurements and Main Results: Genome-wide genotypin

192 In situ hybridization of IPF lung biopsies revealed that miR-144-3p is expressed

193 te between patients with differing levels of IPF severity.Conclusions: The L-IPF is a new questionnai

194 levels were found decreased in the lungs of IPF patients and of mice upon bleomycin-induced pulmonar

195 of a proactive approach to the management of IPF comorbidities, including gastro-oesophageal reflux,

196 llagen deposition and serum C3M, a marker of IPF disease progression.

197 obin-related protein as a possible marker of IPF when compared to control samples (Area under the cur

198 rrelation with existing clinical measures of IPF disease severity.

199 lasts and in a humanized SCID mouse model of IPF employing both short interfering (si) RNA and novel

200 markers for the detection and monitoring of IPF, is paramount.

201 ith physiological aging are at the origin of IPF and that a potential treatment for IPF based on telo

202 ein expression is lower in the parenchyma of IPF lungs but is expressed in the atypical epithelial ce

203 ever, a possible role in the pathogenesis of IPF has not been investigated.

204 efect has been linked to the pathogenesis of IPF in some cases, our data indicate that impaired CMV i

205 which may contribute to the pathogenesis of IPF.

206 ys and are implicated in the pathogenesis of IPF.

207 uction pathway is linked to the pathology of IPF.

208 microbiota contribute to the progression of IPF.

209 ut no difference in the fibrotic response of IPF fibroblasts.

210 uring differentiation increases secretion of IPF biomarkers and RNA sequencing (RNA-seq) of these cul

211 cy in ALI and in early fibrinogenic stage of IPF.

212 d PAI-1 in ALI and various disease stages of IPF.

213 the largest genome-wide association study of IPF to date (2,668 IPF cases and 8,591 controls).

214 d studies of the pathogenesis and therapy of IPF.

215 minimally effective therapies, two-thirds of IPF patients die within 2-5 years from this progressive

216 ells obtained from the distal lung tissue of IPF lungs.

217 in airway basal cells in BAL and tissues of IPF compared with control subjects, but not in chronic o

218 and well-tolerated antifibrotic treatment of IPF remains high.

219 epresent a novel target for the treatment of IPF.

220 ured from available DNA samples from PANTHER-IPF (interim analysis, n = 79; final analysis, n = 118).

221 In PANTHER-IPF, exposure to prednisone/azathioprine/N-acetylcystein

222 thic pulmonary fibrosis (IPF) in the PANTHER-IPF (Evaluating the Effectiveness of Prednisone, Azathio

223 lts: Of the subjects enrolled in the PANTHER-IPF and ACE-IPF, 62% (49/79) and 56% (28/50) had an LTL

224 levels of MARCKS phosphorylation in primary IPF fibroblasts.

225 Among 12 previously reported IPF GWAS loci, five (DPP9, DSP, FAM13A, IVD, and MUC5B)

226 Among patients with moderate or severe IPF, treatment with oral co-trimoxazole did not reduce a

227 of patients with both familial and sporadic IPF appear to be at similar risk.

228 relatives of patients with FPF and sporadic IPF.Methods: Undiagnosed first-degree relatives of patie

229 with FPF and the 59 relatives with sporadic IPF.

230 ival was observed in the model of late-stage IPF.

231 Using FACS to stratify IPF MPCs based on CD44 expression, we determined that CD

232 We studied IPF-LTRs (n = 42) and age-matched non-IPF-LTRs (n = 42)

233 Within this subset, IPF-LTRs who had short telomeres had the highest risk of

234 AEs were consistent with long-term IPF sequelae.

235 Our data support the concept that IPF fibroblasts acquire a cell-autonomous pathological p

236 82 genes were predictive of mortality in the IPF derivation cohort in univariate analyses adjusted fo

237 ssess the role of CD71-expressing AMs in the IPF lung.

238 drive downstream fibrotic remodeling in the IPF lung.

239 mononuclear cells (PBMC) derived from these IPF study subjects.

240 ss as a potential mechanism linking aging to IPF.

241 , a relentlessly progressive course, akin to IPF, is seen in a minority.

242 ed IPF susceptibility variants contribute to IPF susceptibility.Conclusions: The observation that dec

243 short telomeres in the lung, thus leading to IPF with aging.

244 ion and cell migration, which is relevant to IPF development.Objectives: To determine whether rs20762

245 P) are associated with the susceptibility to IPF and mortality.

246 associates with increased susceptibility to IPF supports recent studies demonstrating the importance

247 where along their differentiation trajectory IPF MPCs acquire fibrogenic properties, we analyzed the

248 eutic potential for PI3K inhibitors to treat IPF.

249 ols and AKT activity occurred in MPS-treated IPF fibroblast cells.

250 ffect of many thousands of as yet unreported IPF susceptibility variants contribute to IPF susceptibi

251 and in situ hybridization as well as ex vivo IPF tissue culture experiments were done using healthy d

252 ern is commonly observed in SSc-ILD, whereas IPF is defined by usual interstitial pneumonia.

253 pared with histopathology results in 18 with IPF diagnoses (proportion of diagnoses that were confide

254 ew hypothesis for mechanisms associated with IPF progression and could readily be translated to other

255 ting inflammasome activation associated with IPF.

256 el ILA associations were not associated with IPF.

257 novel associations were not associated with IPF.

258 of variants not reported as associated with IPF.Measurements and Main Results: We identified and rep

259 (P < 5 x 10(-8)) signals of association with IPF susceptibility (associated with altered gene express

260 ronic lung disease sharing key features with IPF including progressive fibrosis and bronchiolization

261 es (symptoms and impacts) of the Living with IPF (L-IPF) questionnaire.

262 Biodistribution analysis in mice with IPF showed prolonged lung retention and widespread lung

263 this prospective study, 18 participants with IPF underwent (3)He DW MRI at 1.5 T and 11 participants

264 d Main Results: A total of 125 patients with IPF (62.4% men) completed the longitudinal study.

265 m control subjects (n = 6) and patients with IPF (n = 6), aging bleomycin (BLM)-treated mice (n = 7),

266 blood from healthy donors and patients with IPF and A549 cells.

267 or lacking CD71 in the BAL of patients with IPF and of healthy control subjects.

268 y lung tissue macrophages from patients with IPF and the signaling pathways controlling CXCL13 gene e

269 ortions of AMs lacking CD71 in patients with IPF compared with healthy control subjects.

270 The reason why some patients with IPF experience harm is unknown.Objectives: To determine

271 rogressive course, whereas all patients with IPF experience progression of disease.

272 e, we show that BAL cells from patients with IPF have a profibrotic phenotype secondary to increased

273 Patients with IPF have altered lung microbiota, with bacterial burden

274 study demonstrate that AM from patients with IPF produces CXCL13 and that the NF-kappaB and JAK/STAT

275 ndscape of transplantation for patients with IPF with regards to antifibrotic therapy, technological

276 oprine and N-Acetylcysteine in Patients with IPF) clinical trial.

277 in AT2 cells from old mice and patients with IPF, and ER stress inhibitor tauroursodeoxycholic acid a

278 68- and CD206-positive AM from patients with IPF, and the CXCL13 gene is induced in these macrophages

279 rallel randomized trial of 342 patients with IPF, breathlessness (Medical Research Council dyspnea sc

280 o increase life expectancy for patients with IPF, but it is associated with disease-specific challeng

281 In patients with IPF, lung bacterial burden predicts fibrosis progression

282 re significantly correlated in patients with IPF, suggesting that TNF-alpha contributes to CXCL13 pro

283 -5p, which is downregulated in patients with IPF, suppressed 15-PGDH expression in vivo and in vitro.

284 o limit acute exacerbations in patients with IPF.

285 s for prognosis and therapy in patients with IPF.

286 novel therapeutic strategy for patients with IPF.

287 clinical trials of 9-ING-41 to patients with IPF.

288 lticenter RCT in newly treated patients with IPF.

289 icrobiota in BAL fluid from 68 patients with IPF.

290 M-151 in larger populations of patients with IPF.

291 eas of increased expression in patients with IPF.

292 , disease impacts, and HRQL in patients with IPF.

293 om blood of healthy donors and patients with IPF.

294 in bronchoalveolar lavage from patients with IPF.

295 effect of immunosuppression in patients with IPF.Methods: LTL was measured from available DNA samples

296 s HRQOL and medication use for patients with IPF.Methods: We performed a multicenter RCT in newly tre

297 cts, and HRQL specifically for patients with IPF.Methods: Working with the U.S. Food and Drug Adminis

298 SSc-ILD shares similarities with IPF, although clear differences exist.

299 Stimulation with IPF-RC during differentiation increases secretion of IPF

300 g both animal models and human subjects with IPF.