Scalp and serum profiling of frontal fibrosing alopecia reveals scalp immune and fibrosis dysregulation with no systemic involvement.


Journal

Journal of the American Academy of Dermatology
ISSN: 1097-6787
Titre abrégé: J Am Acad Dermatol
Pays: United States
ID NLM: 7907132

Informations de publication

Date de publication:
03 2022
Historique:
received: 02 04 2021
accepted: 11 05 2021
pubmed: 28 5 2021
medline: 15 4 2022
entrez: 27 5 2021
Statut: ppublish

Résumé

Frontal fibrosing alopecia (FFA) is a progressive, scarring alopecia of the frontotemporal scalp that poses a substantial burden on quality of life. Large-scale global profiling of FFA is lacking, preventing the development of effective therapeutics. To characterize FFA compared to normal and alopecia areata using broad molecular profiling and to identify biomarkers linked to disease severity. This cross-sectional study assessed 33,118 genes in scalp using RNA sequencing and 350 proteins in serum using OLINK high-throughput proteomics. Disease biomarkers were also correlated with clinical severity and a fibrosis gene set. Genes differentially expressed in lesional FFA included markers related to Th1 (IFNγ/CXCL9/CXCL10), T-cell activation (CD2/CD3/CCL19/ICOS), fibrosis (CXCR3/FGF14/FGF22/VIM/FN1), T-regulatory (FOXP3/TGFB1/TGFB3), and Janus kinase/JAK (JAK3/STAT1/STAT4) (Fold changes [FCH]>1.5, FDR<.05 for all). Only one protein, ADM, was differentially expressed in FFA serum compared to normal (FCH>1.3, FDR<.05). Significant correlations were found between scalp biomarkers (IL-36RN/IL-25) and FFA severity, as well as between JAK/STAT and fibrosis gene-sets (r>.6; P <.05). This study was limited by a small sample size and predominantly female FFA patients. Our data characterize FFA as an inflammatory condition limited to scalp, involving Th1/JAK skewing, with associated fibrosis and elevated T-regulatory markers, suggesting the potential for disease reversibility with JAK/STAT inhibition.

Sections du résumé

BACKGROUND
Frontal fibrosing alopecia (FFA) is a progressive, scarring alopecia of the frontotemporal scalp that poses a substantial burden on quality of life. Large-scale global profiling of FFA is lacking, preventing the development of effective therapeutics.
OBJECTIVE
To characterize FFA compared to normal and alopecia areata using broad molecular profiling and to identify biomarkers linked to disease severity.
METHODS
This cross-sectional study assessed 33,118 genes in scalp using RNA sequencing and 350 proteins in serum using OLINK high-throughput proteomics. Disease biomarkers were also correlated with clinical severity and a fibrosis gene set.
RESULTS
Genes differentially expressed in lesional FFA included markers related to Th1 (IFNγ/CXCL9/CXCL10), T-cell activation (CD2/CD3/CCL19/ICOS), fibrosis (CXCR3/FGF14/FGF22/VIM/FN1), T-regulatory (FOXP3/TGFB1/TGFB3), and Janus kinase/JAK (JAK3/STAT1/STAT4) (Fold changes [FCH]>1.5, FDR<.05 for all). Only one protein, ADM, was differentially expressed in FFA serum compared to normal (FCH>1.3, FDR<.05). Significant correlations were found between scalp biomarkers (IL-36RN/IL-25) and FFA severity, as well as between JAK/STAT and fibrosis gene-sets (r>.6; P <.05).
LIMITATIONS
This study was limited by a small sample size and predominantly female FFA patients.
CONCLUSION
Our data characterize FFA as an inflammatory condition limited to scalp, involving Th1/JAK skewing, with associated fibrosis and elevated T-regulatory markers, suggesting the potential for disease reversibility with JAK/STAT inhibition.

Identifiants

pubmed: 34044102
pii: S0190-9622(21)00999-3
doi: 10.1016/j.jaad.2021.05.016
pii:
doi:

Substances chimiques

Biomarkers 0

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

551-562

Informations de copyright

Copyright © 2021 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.

Déclaration de conflit d'intérêts

Conflicts of interest Dr Guttman-Yassky is an employee of Mount Sinai and has received research funds (grants paid to the institution) from Abbvie, Almirall, AnaptysBio, Asana Biosciences, Boerhinger-Ingelhiem, Dermavant, DS Biopharma, Eli Lilly, Ichnos Sciences, Innovaderm, Janssen, Kiniska, Kyowa Kirin, Leo Pharma, Novan, Pfizer, Ralexar, Regeneron Pharmaceuticals, Inc, Sienna Biopharma, UCB, and Union Therapeutics, and is a consultant for Abbvie, Aditum Bio, Almirall, Amgen, Asana Biosciences, AstraZeneca, Boerhinger-Ingelhiem, Cara Therapeutics, Celgene, Concert, DBV, Dermira, DS Biopharma, Eli Lilly, EMD Serono, Escalier, Galderma, Ichnos Sciences, Incyte Kyowa Kirin, Leo Pharma, Mitsubishi Tanabe, Pandion Therapeutics, Pfizer, RAPT Therapeutics, Regeneron Pharmaceuticals, Inc, Sanofi, Sienna Biopharma, Target PharmaSolutions and Union Therapeutics. Dr Krueger is an employee of Rockefeller University and has received research funds (grants paid to the institution) from Novartis, Pfizer, Amgen, Eli Lilly, Boehringer, Innovaderm, BMS, Janssen, Abbvie, Paraxel, Leo Pharma, Vitae, Akros, Regeneron, Allergan, Novan, Biogen MA, Sienna, UCB, Celgene, Botanix, Incyte, Avillion, and Exicure, and is a consultant for Novartis, Pfizer, Amgen, Eli Lilly, Boehringer, BiogenIdec, Abbvie, Leo Pharma, Escalier, Valeant, Aurigne, Allergan, Asana, UCB, Sienna, Celgene, Nimbus, Menlo, Aristea, Sanofi, Sun Pharma, Almirall, Arena, BMS, Ventyx, Aclaris, Galapagos. The remaining authors have no conflicts to disclose.

Auteurs

Celina Dubin (C)

Department of Dermatology and Laboratory of Inflammatory Skin Diseases, Icahn School of Medicine at Mount Sinai, New York, New York.

Jacob W Glickman (JW)

Department of Dermatology and Laboratory of Inflammatory Skin Diseases, Icahn School of Medicine at Mount Sinai, New York, New York.

Ester Del Duca (E)

Department of Dermatology and Laboratory of Inflammatory Skin Diseases, Icahn School of Medicine at Mount Sinai, New York, New York; Department of Dermatology, University of Magna Graecia, Catanzaro, Italy.

Sumanth Chennareddy (S)

Department of Dermatology and Laboratory of Inflammatory Skin Diseases, Icahn School of Medicine at Mount Sinai, New York, New York.

Joseph Han (J)

Department of Dermatology and Laboratory of Inflammatory Skin Diseases, Icahn School of Medicine at Mount Sinai, New York, New York.

Dante Dahabreh (D)

Department of Dermatology and Laboratory of Inflammatory Skin Diseases, Icahn School of Medicine at Mount Sinai, New York, New York.

Yeriel D Estrada (YD)

Department of Dermatology and Laboratory of Inflammatory Skin Diseases, Icahn School of Medicine at Mount Sinai, New York, New York.

Ning Zhang (N)

Department of Dermatology and Laboratory of Inflammatory Skin Diseases, Icahn School of Medicine at Mount Sinai, New York, New York.

Grace W Kimmel (GW)

Department of Dermatology and Laboratory of Inflammatory Skin Diseases, Icahn School of Medicine at Mount Sinai, New York, New York.

Giselle Singer (G)

Department of Dermatology and Laboratory of Inflammatory Skin Diseases, Icahn School of Medicine at Mount Sinai, New York, New York.

Mashkura Chowdhury (M)

Department of Dermatology and Laboratory of Inflammatory Skin Diseases, Icahn School of Medicine at Mount Sinai, New York, New York.

Andrew Y Zheng (AY)

Macaulay Honors College at City University of New York (CUNY) Hunter College, New York, New York.

Michael Angelov (M)

Department of Dermatology and Laboratory of Inflammatory Skin Diseases, Icahn School of Medicine at Mount Sinai, New York, New York.

Jesús Gay-Mimbrera (J)

Immune-Mediated Inflammatory Skin Diseases Research Group, IMIBIC/Reina Sofia University Hospital/University of Cordoba, Cordoba, Spain.

Juan Ruano Ruiz (J)

Department of Dermatology, Reina Sofia University Hospital, Cordoba, Spain.

James G Krueger (JG)

Laboratory of Investigative Dermatology, Rockefeller University, New York, New York.

Ana B Pavel (AB)

Department of Dermatology and Laboratory of Inflammatory Skin Diseases, Icahn School of Medicine at Mount Sinai, New York, New York; Department of Biomedical Engineering, The University of Mississippi, Oxford, Mississippi. Electronic address: ana.pavel@mssm.edu.

Emma Guttman-Yassky (E)

Department of Dermatology and Laboratory of Inflammatory Skin Diseases, Icahn School of Medicine at Mount Sinai, New York, New York; Laboratory of Investigative Dermatology, Rockefeller University, New York, New York. Electronic address: emma.guttman@mountsinai.org.

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