STAT6 Variants Associate With Relapse of Eosinophilic Esophagitis in Patients Receiving Long-term Proton Pump Inhibitor Therapy. Mougey, E. B., Nguyen, V., Gutiérrez-Junquera, C., Fernández-Fernández, S., Cilleruelo, M. L., Rayo, A., Borrell, B., Román, E., González-Lois, C., Chao, M., Al-Atrash, H., & Franciosi, J. P. Clinical Gastroenterology and Hepatology: The Official Clinical Practice Journal of the American Gastroenterological Association, 19(10):2046–2053.e2, October, 2021.
doi  abstract   bibtex   
BACKGROUND & AIMS: Based on histologic features, variants in STAT6 are associated with a poor initial response to proton pump inhibitor (PPI) therapy in pediatric patients with eosinophilic esophagitis (EoE). We investigated whether these genetic variants are associated with a poor long-term response in children with EoE who initially responded to PPI therapy. METHODS: We performed a prospective longitudinal cohort study of children ages 2 to 16 years who met the diagnostic criteria for EoE (≥15 eosinophils/high-power field [eos/hpf]), responded to 8 weeks of treatment with 2 mg/kg/d PPI (\textless15 eos/hpf), and whose dose then was reduced to 1 mg/kg/d PPI (maintenance therapy) for 1 year, at which point biopsy specimens were collected by endoscopy. Genomic DNA was isolated from formalin-fixed paraffin-embedded biopsy tissue and was genotyped for variants of STAT6. Remission of inflammation was assessed at eos/hpf thresholds of \textless15 and ≤5. RESULTS: Among 73 patients who received 1 mg/kg/d PPI maintenance therapy for 1 year, 13 patients (18%) had 6 to 14 eos/hpf, 36 patients (49%) had 5 or fewer eos/hpf, and 24 patients (33%) relapsed to EoE (≥15 eos/hpf). Carriage of any of 3 STAT6 variants in linkage disequilibrium (r2 ≥0.8; rs324011, rs167769, or rs12368672) was associated with a 2.3- to 2.8-fold increase in the odds of EoE relapse, and with a 2.8- to 4.1-fold increase in the odds of having 6 to 14 eos/hpf. For rs324011, the odds ratio [95% CI] for relapse was 2.77 [1.11, 6.92]; P = .029, and the odds ratio [95% CI] for having 6 to 14 eos/hpf was 3.06 [1.27, 7.36]; P = .012. CONCLUSIONS: Pediatric EoE patients who initially respond to PPI therapy and carry STAT6 variants rs324011, rs167769, or rs12368672 are at increased risk of relapse after 1 year of PPI maintenance therapy.
@article{mougey_stat6_2021,
	title = {{STAT6} {Variants} {Associate} {With} {Relapse} of {Eosinophilic} {Esophagitis} in {Patients} {Receiving} {Long}-term {Proton} {Pump} {Inhibitor} {Therapy}},
	volume = {19},
	issn = {1542-7714},
	doi = {10.1016/j.cgh.2020.08.020},
	abstract = {BACKGROUND \& AIMS: Based on histologic features, variants in STAT6 are associated with a poor initial response to proton pump inhibitor (PPI) therapy in pediatric patients with eosinophilic esophagitis (EoE). We investigated whether these genetic variants are associated with a poor long-term response in children with EoE who initially responded to PPI therapy.
METHODS: We performed a prospective longitudinal cohort study of children ages 2 to 16 years who met the diagnostic criteria for EoE (≥15 eosinophils/high-power field [eos/hpf]), responded to 8 weeks of treatment with 2 mg/kg/d PPI ({\textless}15 eos/hpf), and whose dose then was reduced to 1 mg/kg/d PPI (maintenance therapy) for 1 year, at which point biopsy specimens were collected by endoscopy. Genomic DNA was isolated from formalin-fixed paraffin-embedded biopsy tissue and was genotyped for variants of STAT6. Remission of inflammation was assessed at eos/hpf thresholds of {\textless}15 and ≤5.
RESULTS: Among 73 patients who received 1 mg/kg/d PPI maintenance therapy for 1 year, 13 patients (18\%) had 6 to 14 eos/hpf, 36 patients (49\%) had 5 or fewer eos/hpf, and 24 patients (33\%) relapsed to EoE (≥15 eos/hpf). Carriage of any of 3 STAT6 variants in linkage disequilibrium (r2 ≥0.8; rs324011, rs167769, or rs12368672) was associated with a 2.3- to 2.8-fold increase in the odds of EoE relapse, and with a 2.8- to 4.1-fold increase in the odds of having 6 to 14 eos/hpf. For rs324011, the odds ratio [95\% CI] for relapse was 2.77 [1.11, 6.92]; P = .029, and the odds ratio [95\% CI] for having 6 to 14 eos/hpf was 3.06 [1.27, 7.36]; P = .012.
CONCLUSIONS: Pediatric EoE patients who initially respond to PPI therapy and carry STAT6 variants rs324011, rs167769, or rs12368672 are at increased risk of relapse after 1 year of PPI maintenance therapy.},
	language = {eng},
	number = {10},
	journal = {Clinical Gastroenterology and Hepatology: The Official Clinical Practice Journal of the American Gastroenterological Association},
	author = {Mougey, Edward B. and Nguyen, Vivian and Gutiérrez-Junquera, Carolina and Fernández-Fernández, Sonia and Cilleruelo, Maria Luz and Rayo, Ana and Borrell, Belén and Román, Enriqueta and González-Lois, Carmen and Chao, Montserrat and Al-Atrash, Hadeel and Franciosi, James P.},
	month = oct,
	year = {2021},
	keywords = {Adolescent, Biomarker, Child, Child, Preschool, Eosinophilic Esophagitis, Esophagus, Humans, Immune Response, Longitudinal Studies, Prospective Studies, Proton Pump Inhibitors, Recurrence, Response to Treatment, STAT6 Transcription Factor},
	pages = {2046--2053.e2},
}

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