Multi-Organ System Screening, Care, and Patient Support in Systemic Sclerosis

Cecília Varjú; John D Pauling; Lesley Ann Saketkoo

doi:10.1016/j.rdc.2023.01.002

Multi-Organ System Screening, Care, and Patient Support in Systemic Sclerosis

Rheum Dis Clin North Am. 2023 May;49(2):211-248. doi: 10.1016/j.rdc.2023.01.002.

Authors

Cecília Varjú¹, John D Pauling², Lesley Ann Saketkoo³

Affiliations

¹ Department of Rheumatology and Immunology, Medical School, University of Pécs, Pécs, Hungary.
² Department of Rheumatology, North Bristol NHS Trust, Bristol, UK; Musculoskeletal Research Unit, Translational Health Sciences, Bristol Medical School, University of Bristol, Bristol, UK.
³ New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans, LA 70112, USA; University Medical Center - Comprehensive Pulmonary Hypertension Center and Interstitial Lung Disease Clinic Programs, New Orleans, LA, USA; Section of Pulmonary Medicine, Louisiana State University School of Medicine, New Orleans, LA, USA; Tulane University School of Medicine, New Orleans, LA, USA. Electronic address: Lsaketk@tulane.edu.

PMID: 37028832
DOI: 10.1016/j.rdc.2023.01.002

Abstract

Systemic sclerosis (SSc) is a heterogenous systemic autoimmune disease of complex multi-organ manifestations with a disease-specific mortality of >50%. The patient journey is fraught with severe, diverse, and diffuse physical impairment, psychological burden, and diminishing health-related quality of life. SSc remains unfamiliar to many clinicians. Delayed/misdiagnosis, inadequate screening, and attention for common complications with potentially preventable disability/death contribute to patients feeling isolated and unsupported. We present actionable standards including screening, anticipatory guidance, and counseling in patient-centered SSc-care emphasizing psycho-social health as the central goal, whereas robust vigilance and efforts to improve biophysical health and survival are imperatives that support this goal.

Keywords: Gastrointestinal disease; Health-related quality of life; Interstitial lung disease; Ischemic; Patient-centred; Pulmonary hypertension; Renal crisis; Shared decision-making.

Publication types

Review

MeSH terms

Humans
Hypertension, Pulmonary* / complications
Lung Diseases, Interstitial* / complications
Quality of Life
Scleroderma, Systemic* / complications
Scleroderma, Systemic* / diagnosis
Scleroderma, Systemic* / therapy