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

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.