A comprehensive framework for navigating patient care in systemic sclerosis: A global response to the need for improving the practice of diagnostic and preventive strategies in SSc.
Disability
Interstitial lung disease
Pulmonary fibrosis
Pulmonary hypertension
Quality of life
Renal crisis
Scleroderma
Symptom burden
Systemic sclerosis
Journal
Best practice & research. Clinical rheumatology
ISSN: 1532-1770
Titre abrégé: Best Pract Res Clin Rheumatol
Pays: Netherlands
ID NLM: 101121149
Informations de publication
Date de publication:
09 2021
09 2021
Historique:
pubmed:
21
9
2021
medline:
3
11
2021
entrez:
20
9
2021
Statut:
ppublish
Résumé
Systemic sclerosis (SSc), the most lethal of rheumatologic conditions, is the cause of death in >50% of SSc cases, led by pulmonary fibrosis followed by pulmonary hypertension and then scleroderma renal crisis (SRC). Multiple other preventable and treatable SSc-related vascular, cardiac, gastrointestinal, nutritional and musculoskeletal complications can lead to disability and death. Vascular injury with subsequent inflammation transforming to irreversible fibrosis and permanent damage characterizes SSc. Organ involvement is often present early in the disease course of SSc, but requires careful history-taking and vigilance in screening to detect. Inflammation is potentially reversible provided that treatment intensity quells inflammation and other immune mechanisms. In any SSc phenotype, opportunities for early treatment are prone to be under-utilized, especially in slowly progressive phenotypes that, in contrast to severe progressive ILD, indolently accrue irreversible organ damage resulting in later-stage life-limiting complications such as pulmonary hypertension, cardiac involvement, and malnutrition. A single SSc patient visit often requires much more physician and staff time, organization, vigilance, and direct management for multiple organ systems compared to other rheumatic or pulmonary diseases. Efficiency and efficacy of comprehensive SSc care enlists trending of symptoms and bio-data. Financial sustainability of SSc care benefits from understanding insurance reimbursement and health system allocation policies for complex patients. Sharing care between recognised SSc centers and local cardiology/pulmonary/rheumatology/gastroenterology colleagues may prevent complications and poor outcomes, while providing support to local specialists. As scleroderma specialists, we offer a practical framework with tools to facilitate an optimal, comprehensive and sustainable approach to SSc care. Improved health outcomes in SSc relies upon recogntion, management and, to the extent possible, prevention of SSc and treatment-related complications.
Identifiants
pubmed: 34538573
pii: S1521-6942(21)00049-8
doi: 10.1016/j.berh.2021.101707
pmc: PMC8670736
mid: NIHMS1742238
pii:
doi:
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
101707Subventions
Organisme : NHLBI NIH HHS
ID : K23 HL150237
Pays : United States
Organisme : NIAMS NIH HHS
ID : R01 AR073270
Pays : United States
Organisme : NIAMS NIH HHS
ID : R01 AR050840
Pays : United States
Organisme : NHLBI NIH HHS
ID : T32 HL144470
Pays : United States
Organisme : NHLBI NIH HHS
ID : L30 HL129466
Pays : United States
Informations de copyright
Copyright © 2021 Elsevier Ltd. All rights reserved.
Déclaration de conflit d'intérêts
Declaration of competing interest None of the authors have conflicts of interest to report that are related to the reported content of this paper.
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