Current and Future Outlook on Disease Modification and Defining Low Disease Activity in Systemic Sclerosis

Vivek Nagaraja; Marco Matucci-Cerinic; Daniel E Furst; Masataka Kuwana; Yannick Allanore; Christopher P Denton; Ganesh Raghu; Vallerie Mclaughlin; Panduranga S Rao; James R Seibold; John D Pauling; Michael L Whitfield; Dinesh Khanna

doi:10.1002/art.41246

Current and Future Outlook on Disease Modification and Defining Low Disease Activity in Systemic Sclerosis

Vivek Nagaraja, Marco Matucci-Cerinic, Daniel E Furst, Masataka Kuwana, Yannick Allanore, Christopher P Denton, Ganesh Raghu, Vallerie Mclaughlin, Panduranga S Rao, James R Seibold, John D Pauling, Michael L Whitfield, Dinesh Khanna^*

^*Corresponding author for this work

Bristol Medical School (PHS)

Research output: Contribution to journal › Article (Academic Journal) › peer-review

36 Citations (Scopus)

Abstract

Systemic sclerosis (SSc) is an autoimmune rheumatic disease with heterogeneous clinical manifestations and a variable course in which the severity of the pathology dictates the disease prognosis and course. Among autoimmune rheumatic diseases, SSc has the highest mortality rate among all rheumatic diseases, though there are exciting new therapeutic targets that appear to halt the progression of SSc manifestations such as skin or lung fibrosis. In selected patients, high-intensity regimens with autologous stem cell transplantation can favorably modify the course. In what was once thought to be an untreatable disease, targeted therapies have now changed the outlook of SSc to a treatable disorder. Herein, we discuss the targeted therapies modifying the outlook on selected organ involvement and creating opportunities for future treatment. We also present a framework for defining low disease activity in SSc.

Original language	English
Pages (from-to)	1049-1058
Number of pages	10
Journal	Arthritis and Rheumatology
Volume	72
Issue number	7
Early online date	5 Mar 2020
DOIs	https://doi.org/10.1002/art.41246
Publication status	Published - 1 Jul 2020

Bibliographical note

Publisher Copyright:
© 2020, American College of Rheumatology.

Keywords

Acute Disease
Antihypertensive Agents/therapeutic use
Disease Progression
Endothelin Receptor Antagonists/therapeutic use
Fibrosis
Fingers
Heart Diseases/etiology
Hematopoietic Stem Cell Transplantation
Humans
Immunosuppressive Agents/therapeutic use
Kidney Diseases/etiology
Lung Diseases, Interstitial/etiology
Myocardium/pathology
Outcome Assessment, Health Care
Prostaglandins I/therapeutic use
Pulmonary Arterial Hypertension/etiology
Raynaud Disease/etiology
Scleroderma, Systemic/complications
Severity of Illness Index
Skin Ulcer/etiology

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Nagaraja, V., Matucci-Cerinic, M., Furst, D. E., Kuwana, M., Allanore, Y., Denton, C. P., Raghu, G., Mclaughlin, V., Rao, P. S., Seibold, J. R., Pauling, J. D., Whitfield, M. L., & Khanna, D. (2020). Current and Future Outlook on Disease Modification and Defining Low Disease Activity in Systemic Sclerosis. Arthritis and Rheumatology, 72(7), 1049-1058. https://doi.org/10.1002/art.41246

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title = "Current and Future Outlook on Disease Modification and Defining Low Disease Activity in Systemic Sclerosis",

abstract = "Systemic sclerosis (SSc) is an autoimmune rheumatic disease with heterogeneous clinical manifestations and a variable course in which the severity of the pathology dictates the disease prognosis and course. Among autoimmune rheumatic diseases, SSc has the highest mortality rate among all rheumatic diseases, though there are exciting new therapeutic targets that appear to halt the progression of SSc manifestations such as skin or lung fibrosis. In selected patients, high-intensity regimens with autologous stem cell transplantation can favorably modify the course. In what was once thought to be an untreatable disease, targeted therapies have now changed the outlook of SSc to a treatable disorder. Herein, we discuss the targeted therapies modifying the outlook on selected organ involvement and creating opportunities for future treatment. We also present a framework for defining low disease activity in SSc.",

keywords = "Acute Disease, Antihypertensive Agents/therapeutic use, Disease Progression, Endothelin Receptor Antagonists/therapeutic use, Fibrosis, Fingers, Heart Diseases/etiology, Hematopoietic Stem Cell Transplantation, Humans, Immunosuppressive Agents/therapeutic use, Kidney Diseases/etiology, Lung Diseases, Interstitial/etiology, Myocardium/pathology, Outcome Assessment, Health Care, Prostaglandins I/therapeutic use, Pulmonary Arterial Hypertension/etiology, Raynaud Disease/etiology, Scleroderma, Systemic/complications, Severity of Illness Index, Skin Ulcer/etiology",

author = "Vivek Nagaraja and Marco Matucci-Cerinic and Furst, \{Daniel E\} and Masataka Kuwana and Yannick Allanore and Denton, \{Christopher P\} and Ganesh Raghu and Vallerie Mclaughlin and Rao, \{Panduranga S\} and Seibold, \{James R\} and Pauling, \{John D\} and Whitfield, \{Michael L\} and Dinesh Khanna",

note = "Publisher Copyright: {\textcopyright} 2020, American College of Rheumatology.",

year = "2020",

month = jul,

day = "1",

doi = "10.1002/art.41246",

language = "English",

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Nagaraja, V, Matucci-Cerinic, M, Furst, DE, Kuwana, M, Allanore, Y, Denton, CP, Raghu, G, Mclaughlin, V, Rao, PS, Seibold, JR, Pauling, JD, Whitfield, ML & Khanna, D 2020, 'Current and Future Outlook on Disease Modification and Defining Low Disease Activity in Systemic Sclerosis', Arthritis and Rheumatology, vol. 72, no. 7, pp. 1049-1058. https://doi.org/10.1002/art.41246

TY - JOUR

T1 - Current and Future Outlook on Disease Modification and Defining Low Disease Activity in Systemic Sclerosis

AU - Nagaraja, Vivek

AU - Matucci-Cerinic, Marco

AU - Furst, Daniel E

AU - Kuwana, Masataka

AU - Allanore, Yannick

AU - Denton, Christopher P

AU - Raghu, Ganesh

AU - Mclaughlin, Vallerie

AU - Rao, Panduranga S

AU - Seibold, James R

AU - Pauling, John D

AU - Whitfield, Michael L

AU - Khanna, Dinesh

PY - 2020/7/1

Y1 - 2020/7/1

N2 - Systemic sclerosis (SSc) is an autoimmune rheumatic disease with heterogeneous clinical manifestations and a variable course in which the severity of the pathology dictates the disease prognosis and course. Among autoimmune rheumatic diseases, SSc has the highest mortality rate among all rheumatic diseases, though there are exciting new therapeutic targets that appear to halt the progression of SSc manifestations such as skin or lung fibrosis. In selected patients, high-intensity regimens with autologous stem cell transplantation can favorably modify the course. In what was once thought to be an untreatable disease, targeted therapies have now changed the outlook of SSc to a treatable disorder. Herein, we discuss the targeted therapies modifying the outlook on selected organ involvement and creating opportunities for future treatment. We also present a framework for defining low disease activity in SSc.

AB - Systemic sclerosis (SSc) is an autoimmune rheumatic disease with heterogeneous clinical manifestations and a variable course in which the severity of the pathology dictates the disease prognosis and course. Among autoimmune rheumatic diseases, SSc has the highest mortality rate among all rheumatic diseases, though there are exciting new therapeutic targets that appear to halt the progression of SSc manifestations such as skin or lung fibrosis. In selected patients, high-intensity regimens with autologous stem cell transplantation can favorably modify the course. In what was once thought to be an untreatable disease, targeted therapies have now changed the outlook of SSc to a treatable disorder. Herein, we discuss the targeted therapies modifying the outlook on selected organ involvement and creating opportunities for future treatment. We also present a framework for defining low disease activity in SSc.

KW - Acute Disease

KW - Antihypertensive Agents/therapeutic use

KW - Disease Progression

KW - Endothelin Receptor Antagonists/therapeutic use

KW - Fibrosis

KW - Fingers

KW - Heart Diseases/etiology

KW - Hematopoietic Stem Cell Transplantation

KW - Humans

KW - Immunosuppressive Agents/therapeutic use

KW - Kidney Diseases/etiology

KW - Lung Diseases, Interstitial/etiology

KW - Myocardium/pathology

KW - Outcome Assessment, Health Care

KW - Prostaglandins I/therapeutic use

KW - Pulmonary Arterial Hypertension/etiology

KW - Raynaud Disease/etiology

KW - Scleroderma, Systemic/complications

KW - Severity of Illness Index

KW - Skin Ulcer/etiology

U2 - 10.1002/art.41246

DO - 10.1002/art.41246

M3 - Article (Academic Journal)

C2 - 32134199

SN - 2326-5191

VL - 72

SP - 1049

EP - 1058

JO - Arthritis and Rheumatology

JF - Arthritis and Rheumatology

IS - 7

ER -

Current and Future Outlook on Disease Modification and Defining Low Disease Activity in Systemic Sclerosis

Abstract

Bibliographical note

Keywords

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