Small molecules in idiopathic inflammatory myopathies: a systematic review and a multicenter case series about Janus kinase inhibitors and apremilast

Objective. Idiopathic inflammatory myopathies (IIM) are rare autoimmune diseases that primarily affect striated muscles; skin, joints, and lungs may be involved with different degrees of severity. Traditional treatment relies on high-dose glucocorticoids and conventional synthetic disease-modifying...

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Main Authors: Chiara Rizzo, Silvia Grazzini, Edoardo Conticini, Hector Chinoy, Roberto D’Alessandro, Federica Camarda, Luca Cantarini, Bruno Frediani, Giuliana Guggino, Lidia La Barbera
Format: Article
Language:English
Published: PAGEPress Publications 2025-01-01
Series:Reumatismo
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Online Access:https://www.reumatismo.org/reuma/article/view/1718
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author Chiara Rizzo
Silvia Grazzini
Edoardo Conticini
Hector Chinoy
Roberto D’Alessandro
Federica Camarda
Luca Cantarini
Bruno Frediani
Giuliana Guggino
Lidia La Barbera
author_facet Chiara Rizzo
Silvia Grazzini
Edoardo Conticini
Hector Chinoy
Roberto D’Alessandro
Federica Camarda
Luca Cantarini
Bruno Frediani
Giuliana Guggino
Lidia La Barbera
author_sort Chiara Rizzo
collection DOAJ
description Objective. Idiopathic inflammatory myopathies (IIM) are rare autoimmune diseases that primarily affect striated muscles; skin, joints, and lungs may be involved with different degrees of severity. Traditional treatment relies on high-dose glucocorticoids and conventional synthetic disease-modifying antirheumatic drugs. Methods. A growing amount of evidence is demonstrating the potential role of novel treatments in the management of IIM. We report our experience with Janus kinase inhibitors (JAKi) in these conditions and review the current evidence for the use of small molecules in real-life clinical practice. Results. A total of 41 papers were retrieved from PubMed, 37 papers concerning IIM and JAKi, and 4 papers concerning IIM and apremilast. Conclusions. An overall good efficacy was evidenced in IIM-associated skin lesions, including rash, ulcers, and calcinosis. If present, muscle and joint involvement demonstrated a good response to therapy, while it was not possible to draw any conclusion about dysphagia. No life-threatening adverse events were reported.
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institution Kabale University
issn 0048-7449
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language English
publishDate 2025-01-01
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record_format Article
series Reumatismo
spelling doaj-art-2ecaf44b3b5d4be88f33bf0cc617e61b2025-01-14T02:30:59ZengPAGEPress PublicationsReumatismo0048-74492240-26832025-01-0110.4081/reumatismo.2025.1718Small molecules in idiopathic inflammatory myopathies: a systematic review and a multicenter case series about Janus kinase inhibitors and apremilastChiara Rizzo0https://orcid.org/0000-0003-4838-5854Silvia Grazzini1https://orcid.org/0000-0001-6541-2469Edoardo Conticini2https://orcid.org/0000-0002-3974-6606Hector Chinoy3https://orcid.org/0000-0001-6492-1288Roberto D’Alessandro4Federica Camarda5https://orcid.org/0000-0002-2548-0659Luca Cantarini6Bruno Frediani7Giuliana Guggino8https://orcid.org/0000-0003-2479-6958Lidia La Barbera9https://orcid.org/0009-0005-4208-5828Rheumatology Unit, Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties, University of PalermoRheumatology Unit, Department of Medicine, Surgery and Neurosciences, University of SienaRheumatology Unit, Department of Medicine, Surgery and Neurosciences, University of SienaDepartment of Rheumatology, Salford Royal Hospital, Northern Care Alliance NHS Foundation Trust, Manchester Academic Health Science Centre, Salford; Division of Musculoskeletal and Dermatological Sciences, Faculty of Biology, Medicine and Health, University of ManchesterRheumatology Unit, Department of Medicine, Surgery and Neurosciences, University of SienaRheumatology Unit, Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties, University of PalermoRheumatology Unit, Department of Medicine, Surgery and Neurosciences, University of SienaRheumatology Unit, Department of Medicine, Surgery and Neurosciences, University of SienaRheumatology Unit, Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties, University of PalermoRheumatology Unit, Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties, University of Palermo Objective. Idiopathic inflammatory myopathies (IIM) are rare autoimmune diseases that primarily affect striated muscles; skin, joints, and lungs may be involved with different degrees of severity. Traditional treatment relies on high-dose glucocorticoids and conventional synthetic disease-modifying antirheumatic drugs. Methods. A growing amount of evidence is demonstrating the potential role of novel treatments in the management of IIM. We report our experience with Janus kinase inhibitors (JAKi) in these conditions and review the current evidence for the use of small molecules in real-life clinical practice. Results. A total of 41 papers were retrieved from PubMed, 37 papers concerning IIM and JAKi, and 4 papers concerning IIM and apremilast. Conclusions. An overall good efficacy was evidenced in IIM-associated skin lesions, including rash, ulcers, and calcinosis. If present, muscle and joint involvement demonstrated a good response to therapy, while it was not possible to draw any conclusion about dysphagia. No life-threatening adverse events were reported. https://www.reumatismo.org/reuma/article/view/1718Idiopathic inflammatory myopathiesmyositistreatmentJAK inhibitorsapremilasttsDMARDs
spellingShingle Chiara Rizzo
Silvia Grazzini
Edoardo Conticini
Hector Chinoy
Roberto D’Alessandro
Federica Camarda
Luca Cantarini
Bruno Frediani
Giuliana Guggino
Lidia La Barbera
Small molecules in idiopathic inflammatory myopathies: a systematic review and a multicenter case series about Janus kinase inhibitors and apremilast
Reumatismo
Idiopathic inflammatory myopathies
myositis
treatment
JAK inhibitors
apremilast
tsDMARDs
title Small molecules in idiopathic inflammatory myopathies: a systematic review and a multicenter case series about Janus kinase inhibitors and apremilast
title_full Small molecules in idiopathic inflammatory myopathies: a systematic review and a multicenter case series about Janus kinase inhibitors and apremilast
title_fullStr Small molecules in idiopathic inflammatory myopathies: a systematic review and a multicenter case series about Janus kinase inhibitors and apremilast
title_full_unstemmed Small molecules in idiopathic inflammatory myopathies: a systematic review and a multicenter case series about Janus kinase inhibitors and apremilast
title_short Small molecules in idiopathic inflammatory myopathies: a systematic review and a multicenter case series about Janus kinase inhibitors and apremilast
title_sort small molecules in idiopathic inflammatory myopathies a systematic review and a multicenter case series about janus kinase inhibitors and apremilast
topic Idiopathic inflammatory myopathies
myositis
treatment
JAK inhibitors
apremilast
tsDMARDs
url https://www.reumatismo.org/reuma/article/view/1718
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