Emerging targets of disease-modifying therapy for systemic sclerosis

Nature Reviews. Rheumatology
Elizabeth R Volkmann, J Varga

Abstract

Systemic sclerosis (SSc) has the highest cause-specific mortality of all the connective tissue diseases, and the aetiology of this complex and heterogeneous condition remains an enigma. Current disease-modifying therapies for SSc predominantly target inflammatory and vascular pathways but have variable and unpredictable clinical efficacy, and none is curative. Moreover, many of these therapies possess undesirable safety profiles and have no appreciable effect on long-term mortality. This Review describes the most promising of the existing therapeutic targets for SSc and places them in the context of our evolving understanding of the pathophysiology of this disease. As well as taking an in-depth look at the immune, inflammatory, vascular and fibrotic pathways implicated in the pathogenesis of SSc, this Review discusses emerging treatment targets and therapeutic strategies. The article concludes with an overview of important unanswered questions in SSc research that might inform the design of future studies of treatments aimed at modifying the course of this disease.

References

Nov 22, 2000·The Journal of Immunology : Official Journal of the American Association of Immunologists·S SatoK Takehara
Jul 28, 2004·The American Journal of Pathology·Noriko AsanoShinichi Sato
May 13, 2006·The Journal of Investigative Dermatology·Ahalia M FerreiraLuisa A DiPietro
Mar 3, 2007·The Journal of Clinical Investigation·John Varga, David Abraham
Sep 1, 2007·Arthritis and Rheumatism·Robert LafyatisEric Matteson
Apr 2, 2009·Nature Reviews. Rheumatology·John Varga, Boris Pasche
May 8, 2009·The New England Journal of Medicine·Armando GabrielliThomas Krieg
Oct 9, 2009·The Journal of Investigative Dermatology·Jennifer L SargentMichael L Whitfield
Feb 18, 2010·European Journal of Medical Research·Thilo GambichlerA Kreuter
Aug 25, 2010·The Journal of Cell Biology·Fei LiuDaniel J Tschumperlin
Nov 12, 2010·Science Translational Medicine·Glenda TrujilloCory M Hogaboam
Dec 15, 2010·The Journal of Immunology : Official Journal of the American Association of Immunologists·Anagha A DivekarRam Raj Singh
Aug 2, 2011·The Clinical Respiratory Journal·Maria De SantisGianfranco Ferraccioli
Sep 17, 2011·Cytokine·Patrizia Fuschiotti
Oct 26, 2011·Nature Reviews. Rheumatology·Swati BhattacharyyaJohn Varga
Nov 9, 2011·The American Journal of Pathology·Shun KitabaIchiro Katayama
Feb 23, 2012·Arthritis and Rheumatism·Angelika HornJörg H W Distler
Mar 1, 2012·The European Respiratory Journal·Gérald SimonneauIrene M Lang
Mar 6, 2012·The American Journal of Pathology·Boris HinzGiulio Gabbiani
Apr 12, 2012·Annals of the Rheumatic Diseases·Estrella Garcia GonzalezJörg H W Distler

❮ Previous
Next ❯

Citations

May 23, 2019·Current Rheumatology Reports·Maria E TevesJohn Varga
Jul 12, 2019·Der Internist·Michael Sticherling
Nov 11, 2019·Nature Reviews. Rheumatology·Yoshishige MiyabeAndrew D Luster
Jul 18, 2020·Expert Opinion on Pharmacotherapy·Zsuzsanna H McMahan, Elizabeth R Volkmann
Dec 28, 2019·Arthritis Research & Therapy·Yi-Ting LinPing-Hsun Wu
Dec 4, 2019·Nature Reviews. Rheumatology·Boris Hinz, David Lagares
Jul 9, 2020·Frontiers in Immunology·Aslıhan Avanoǧlu GülerMarco Matucci-Cerinic
Jul 12, 2020·Journal of Cell Science·Pardis PakshirBoris Hinz
Jun 21, 2020·Annals of the Rheumatic Diseases·Lorenzo BerettaUNKNOWN PRECISESADS Flow Cytometry study group
Jul 12, 2019·Frontiers in Immunology·Anne M StevensFrancesco Zulian
Oct 31, 2020·Cell Biochemistry and Biophysics·Ming LiJinfeng Wu
Oct 15, 2019·Journal of Clinical Rheumatology : Practical Reports on Rheumatic & Musculoskeletal Diseases·Ivana MikačićĐuro Plavljanić
Dec 19, 2020·Autoimmunity Reviews·Carla VarricaSandra Simões
Oct 16, 2020·Expert Opinion on Emerging Drugs·David RoofehDinesh Khanna
Mar 12, 2021·Journal of Scleroderma and Related Disorders·Elizabeth R Volkmann, Aryeh Fischer
Apr 10, 2021·International Journal of Rheumatic Diseases·Ahmet KaratasSuleyman Serdar Koca
Apr 15, 2021·Advanced Biology·Andrea De PieriKarin Wuertz-Kozak
May 15, 2021·Immunological Reviews·Ronen SchusterBoris Hinz
Jun 28, 2020·Reumatología clinica·Miguel Gomes GuerraPatrícia Pinto
May 27, 2021·Scientific Reports·Julien GuiotRenaud Louis
Jun 3, 2021·Arthritis Research & Therapy·Corrado Campochiaro, Yannick Allanore
Jun 4, 2021·Wound Repair and Regeneration : Official Publication of the Wound Healing Society [and] the European Tissue Repair Society·Mugdha SawantRonen Schuster
Jul 18, 2021·Immunity, Inflammation and Disease·Dan WenzelMehdi Rashighi
Aug 18, 2021·Clinical Immunology : the Official Journal of the Clinical Immunology Society·Alexandra Zanin-Zhorov, Bruce R Blazar
Sep 2, 2021·Clinical Reviews in Allergy & Immunology·Alain LescoatDinesh Khanna
Aug 18, 2021·Current Opinion in Rheumatology·Nicholas A Flavahan
Sep 9, 2021·Current Opinion in Rheumatology·Dibyendu BhattacharyyaJohn Varga
Aug 10, 2021·Blood·Ronjon Chakraverty, Takanori Teshima

❮ Previous
Next ❯

Methods Mentioned

BETA
biopsy
nuclear translocation
bronchoalveolar
lavage

Clinical Trials Mentioned

NCT02981082
NCT00043706
NCT01538719
NCT00946699
NCT03274076
NCT03365869
NCT02921971
NCT03222492
NCT03198689
NCT01639573

Related Concepts

Trending Feeds

COVID-19

Coronaviruses encompass a large family of viruses that cause the common cold as well as more serious diseases, such as the ongoing outbreak of coronavirus disease 2019 (COVID-19; formally known as 2019-nCoV). Coronaviruses can spread from animals to humans; symptoms include fever, cough, shortness of breath, and breathing difficulties; in more severe cases, infection can lead to death. This feed covers recent research on COVID-19.

Blastomycosis

Blastomycosis fungal infections spread through inhaling Blastomyces dermatitidis spores. Discover the latest research on blastomycosis fungal infections here.

Nuclear Pore Complex in ALS/FTD

Alterations in nucleocytoplasmic transport, controlled by the nuclear pore complex, may be involved in the pathomechanism underlying multiple neurodegenerative diseases including Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Here is the latest research on the nuclear pore complex in ALS and FTD.

Applications of Molecular Barcoding

The concept of molecular barcoding is that each original DNA or RNA molecule is attached to a unique sequence barcode. Sequence reads having different barcodes represent different original molecules, while sequence reads having the same barcode are results of PCR duplication from one original molecule. Discover the latest research on molecular barcoding here.

Chronic Fatigue Syndrome

Chronic fatigue syndrome is a disease characterized by unexplained disabling fatigue; the pathology of which is incompletely understood. Discover the latest research on chronic fatigue syndrome here.

Evolution of Pluripotency

Pluripotency refers to the ability of a cell to develop into three primary germ cell layers of the embryo. This feed focuses on the mechanisms that underlie the evolution of pluripotency. Here is the latest research.

Position Effect Variegation

Position Effect Variagation occurs when a gene is inactivated due to its positioning near heterochromatic regions within a chromosome. Discover the latest research on Position Effect Variagation here.

STING Receptor Agonists

Stimulator of IFN genes (STING) are a group of transmembrane proteins that are involved in the induction of type I interferon that is important in the innate immune response. The stimulation of STING has been an active area of research in the treatment of cancer and infectious diseases. Here is the latest research on STING receptor agonists.

Microbicide

Microbicides are products that can be applied to vaginal or rectal mucosal surfaces with the goal of preventing, or at least significantly reducing, the transmission of sexually transmitted infections. Here is the latest research on microbicides.

Related Papers

Nihon rinsho. Japanese journal of clinical medicine
Osamu Ishikawa, Kazunori Ohnishi
Seminars in Cutaneous Medicine and Surgery
J H Stone, F M Wigley
Current Pharmaceutical Biotechnology
Jingxiu XuanJulian L Ambrus
© 2022 Meta ULC. All rights reserved