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Systemic sclerosis

Volkmann, Elizabeth R. ; Andréasson, Kristofer LU and Smith, Vanessa (2023) In The Lancet 401(10373). p.304-318
Abstract

Systemic sclerosis, also known as scleroderma, is a rare and complex autoimmune connective-tissue disease. Once considered an untreatable and unpredictable condition, research advancements have improved our understanding of its disease pathogenesis and clinical phenotypes and expanded our treatment armamentarium. Early and accurate diagnosis is essential, while ongoing efforts to risk stratify patients have a central role in predicting both organ involvement and disease progression. A holistic approach is required when choosing the optimal therapeutic strategy, balancing the side-effect profile with efficacy and tailoring the treatment according to the goals of care of the patient. This Seminar reviews the multiple clinical dimensions... (More)

Systemic sclerosis, also known as scleroderma, is a rare and complex autoimmune connective-tissue disease. Once considered an untreatable and unpredictable condition, research advancements have improved our understanding of its disease pathogenesis and clinical phenotypes and expanded our treatment armamentarium. Early and accurate diagnosis is essential, while ongoing efforts to risk stratify patients have a central role in predicting both organ involvement and disease progression. A holistic approach is required when choosing the optimal therapeutic strategy, balancing the side-effect profile with efficacy and tailoring the treatment according to the goals of care of the patient. This Seminar reviews the multiple clinical dimensions of systemic sclerosis, beginning at a precursor very early stage of disease, with a focus on timely early detection of organ involvement. This Seminar also summarises management considerations according to the pathological hallmarks of systemic sclerosis (eg, inflammation, fibrosis, and vasculopathy) and highlights unmet needs and opportunities for future research and discovery.

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author
; and
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
The Lancet
volume
401
issue
10373
pages
15 pages
publisher
Elsevier
external identifiers
  • scopus:85146891440
  • pmid:36442487
ISSN
0140-6736
DOI
10.1016/S0140-6736(22)01692-0
language
English
LU publication?
yes
id
12d0bd75-296a-4432-8316-b57d4f4554c1
date added to LUP
2023-02-10 15:43:34
date last changed
2024-06-14 07:26:56
@article{12d0bd75-296a-4432-8316-b57d4f4554c1,
  abstract     = {{<p>Systemic sclerosis, also known as scleroderma, is a rare and complex autoimmune connective-tissue disease. Once considered an untreatable and unpredictable condition, research advancements have improved our understanding of its disease pathogenesis and clinical phenotypes and expanded our treatment armamentarium. Early and accurate diagnosis is essential, while ongoing efforts to risk stratify patients have a central role in predicting both organ involvement and disease progression. A holistic approach is required when choosing the optimal therapeutic strategy, balancing the side-effect profile with efficacy and tailoring the treatment according to the goals of care of the patient. This Seminar reviews the multiple clinical dimensions of systemic sclerosis, beginning at a precursor very early stage of disease, with a focus on timely early detection of organ involvement. This Seminar also summarises management considerations according to the pathological hallmarks of systemic sclerosis (eg, inflammation, fibrosis, and vasculopathy) and highlights unmet needs and opportunities for future research and discovery.</p>}},
  author       = {{Volkmann, Elizabeth R. and Andréasson, Kristofer and Smith, Vanessa}},
  issn         = {{0140-6736}},
  language     = {{eng}},
  number       = {{10373}},
  pages        = {{304--318}},
  publisher    = {{Elsevier}},
  series       = {{The Lancet}},
  title        = {{Systemic sclerosis}},
  url          = {{http://dx.doi.org/10.1016/S0140-6736(22)01692-0}},
  doi          = {{10.1016/S0140-6736(22)01692-0}},
  volume       = {{401}},
  year         = {{2023}},
}