An Update on Systemic Sclerosis and its Perioperative Management
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PREOPERATIVE EVALUATION (BJ SWEITZER, SECTION EDITOR)
An Update on Systemic Sclerosis and its Perioperative Management Zyad J. Carr 1,2
&
John Klick 3,4 & Brittany J. McDowell 5,6 & Jean G. Charchaflieh 1,2 & Kunal Karamchandani 5,6
# Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Purpose of Review Systemic sclerosis or scleroderma (SSc) is a systemic, immune-mediated disease characterized by abnormal cutaneous and organ-based fibrosis that results in progressive end-organ dysfunction and decreased survival. SSc results in significant challenges for the practicing anesthesiologist due to its rarity, multi-system involvement, and limited evidence-based guidance for optimal perioperative care. In this update, we briefly discuss the recent evidence on the pathophysiology and current management of SSc, review the anesthesia-related literature, and extrapolate these observations into an optimal perioperative strategy for the care of SSc patients. Recent Findings Evidence shows that patients with SSc demonstrate an increased risk for perioperative myocardial infarction, high rates of interstitial lung disease, pulmonary arterial hypertension, neurological disease, gastric dysmotility disorders, and challenging airway management, all findings that may result in suboptimal perioperative outcomes. Summary Advances in SSc medical management have resulted in improved survival, likely increasing the number of patients who will be exposed to perioperative care. Optimal perioperative management and risk stratification should expand beyond the well-described airway challenges and consider numerous systemic manifestations of systemic sclerosis such as pulmonary arterial hypertension, interstitial lung disease, and cardiac sequelae. Keywords Systemic sclerosis . Scleroderma . Anesthesia . Perioperative
Introduction Systemic sclerosis is an autoimmune disorder characterized by small-vessel vasculopathy, abnormal collagen deposition with fibrosis, and autoantibody-mediated immune dysfunction characteristically observed in the integumentary system. This article is part of the Topical Collection on Preoperative Evaluation * Zyad J. Carr [email protected] 1
Department of Anesthesiology, Yale New Haven Hospital, New Haven, CT 06510, USA
2
Yale University School of Medicine, New Haven, CT 06510, USA
3
Department of Anesthesiology, University of Vermont Medical Center, Burlington, VT 05405, USA
4
Larner College of Medicine at The University of Vermont, Burlington, VT 05405, USA
5
Department of Anesthesiology, Penn State Health Milton S. Hershey Medical Center, Hershey, PA 17033, USA
6
Penn State School of Medicine, Hershey, PA 17033, USA
In addition, progressive fibrosis of visceral organs such as the heart, the lungs, the kidneys, and the gastrointestinal tract results in substantial morbidity and mortality. Proposed mechanisms highlight the importance of small-vessel vasculopathy, with broad interplay of genetic and environmental triggers, as a causative factor for both cutaneous and viscer
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