JAK inhibitors and systemic sclerosis: A systematic review of the literature.


Journal

Autoimmunity reviews
ISSN: 1873-0183
Titre abrégé: Autoimmun Rev
Pays: Netherlands
ID NLM: 101128967

Informations de publication

Date de publication:
Oct 2022
Historique:
received: 29 07 2022
accepted: 03 08 2022
pubmed: 10 8 2022
medline: 24 9 2022
entrez: 9 8 2022
Statut: ppublish

Résumé

Systemic sclerosis (SSc) is a systemic autoimmune disease with heterogeneous clinical presentation and prognosis. JAK inhibitors reduced cutaneous and pulmonary fibrosis in mice models of SSc. Clinical data regarding the efficacy and safety of JAK inhibitors in SSc patients are scarce. We performed a systematic literature review of patients with SSc defined by the 2013 ACR/EULAR criteria and treated with JAK inhibitors, searching in Medline, Cochrane library and Embase databases. Fifty-nine patients (mean age 47 ± 15 years) were included. Median treatment duration was 12 [6-12] months. JAK inhibitors (tofacitinib in 47 patients and baricitinib in 12 patients) were prescribed as first line therapy in 35 patients (59%). A significant cutaneous response (decrease in the mRSS - modified Rodnan skin score - of >5 points and ≥ 25% from baseline) was reported in 52 patients (88%). Among patients with interstitial lung disease (ILD) (n = 31), 28/29 patients had no ILD progression during follow-up time (missing data in 2 patients). Only 2 patients had a disease progression during treatment (including one patient with progressive skin fibrosis). Cutaneous response was more frequently observed in treatment naïve SSc patients. Decrease of the mRSS after treatment initiation was more significant in treatment naïve SSc patients. Eighteen non-serious side-effects were noted in 12 patients (20%), without treatment interruption: 6 infections, 6 gastrointestinal disorders, 4 hepatitis and 3 dyslipidemias. JAK inhibitors could represent a safe and effective treatment option for skin fibrosis and ILD in systemic sclerosis.

Sections du résumé

BACKGROUND BACKGROUND
Systemic sclerosis (SSc) is a systemic autoimmune disease with heterogeneous clinical presentation and prognosis. JAK inhibitors reduced cutaneous and pulmonary fibrosis in mice models of SSc. Clinical data regarding the efficacy and safety of JAK inhibitors in SSc patients are scarce.
METHODS METHODS
We performed a systematic literature review of patients with SSc defined by the 2013 ACR/EULAR criteria and treated with JAK inhibitors, searching in Medline, Cochrane library and Embase databases.
RESULTS RESULTS
Fifty-nine patients (mean age 47 ± 15 years) were included. Median treatment duration was 12 [6-12] months. JAK inhibitors (tofacitinib in 47 patients and baricitinib in 12 patients) were prescribed as first line therapy in 35 patients (59%). A significant cutaneous response (decrease in the mRSS - modified Rodnan skin score - of >5 points and ≥ 25% from baseline) was reported in 52 patients (88%). Among patients with interstitial lung disease (ILD) (n = 31), 28/29 patients had no ILD progression during follow-up time (missing data in 2 patients). Only 2 patients had a disease progression during treatment (including one patient with progressive skin fibrosis). Cutaneous response was more frequently observed in treatment naïve SSc patients. Decrease of the mRSS after treatment initiation was more significant in treatment naïve SSc patients. Eighteen non-serious side-effects were noted in 12 patients (20%), without treatment interruption: 6 infections, 6 gastrointestinal disorders, 4 hepatitis and 3 dyslipidemias.
CONCLUSION CONCLUSIONS
JAK inhibitors could represent a safe and effective treatment option for skin fibrosis and ILD in systemic sclerosis.

Identifiants

pubmed: 35944611
pii: S1568-9972(22)00138-0
doi: 10.1016/j.autrev.2022.103168
pii:
doi:

Substances chimiques

Janus Kinase Inhibitors 0

Types de publication

Journal Article Review Systematic Review

Langues

eng

Sous-ensembles de citation

IM

Pagination

103168

Informations de copyright

Copyright © 2022 Elsevier B.V. All rights reserved.

Déclaration de conflit d'intérêts

Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

Auteurs

Clothilde Moriana (C)

Department of internal medicine and clinical immunology, Centre de compétence des maladies auto-immunes systémiques rares, CHU de Nancy, Université de Lorraine, 5 Rue du Morvan, Vandoeuvre-lès-Nancy 54500, France.

Thomas Moulinet (T)

Department of internal medicine and clinical immunology, Centre de compétence des maladies auto-immunes systémiques rares, CHU de Nancy, Université de Lorraine, 5 Rue du Morvan, Vandoeuvre-lès-Nancy 54500, France; UMR 7365, IMoPA, Université de Lorraine, CNRS, Nancy, France.

Roland Jaussaud (R)

Department of internal medicine and clinical immunology, Centre de compétence des maladies auto-immunes systémiques rares, CHU de Nancy, Université de Lorraine, 5 Rue du Morvan, Vandoeuvre-lès-Nancy 54500, France.

Paul Decker (P)

Department of internal medicine and clinical immunology, Centre de compétence des maladies auto-immunes systémiques rares, CHU de Nancy, Université de Lorraine, 5 Rue du Morvan, Vandoeuvre-lès-Nancy 54500, France. Electronic address: p.decker@chru-nancy.fr.

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Classifications MeSH