Perioperative Outcomes in Patients with Systemic Sclerosis: Analysis of a Large Single- Center Case Series

Abstract

Background There are significant knowledge gaps regarding perioperative outcomes for patients with systemic sclerosis (SSc), a rare immune-mediated disease characterized by cutaneous and organ-based fibrosis. The primary aim of this study was to describe the incidence of various perioperative complications in a single-center population of SSc. Methods 278 SSc patients with 605 unique surgical encounters were identified over a ten-year period (Jan 1, 2010, through Jan 1, 2020). Data collection included airway management, intraoperative adverse events (pulmonary aspiration, cardiac arrest, difficult airway designation), and 30-day postoperative complications (pneumonia, myocardial infarction, atrial fibrillation, congestive heart failure). Results Hypertension (57.9%), pulmonary hypertension (PH) or pulmonary arterial hypertension (PAH) (52.5%), interstitial lung disease (ILD) (32.4%), and gastroesophageal reflux disease (82.4%) were common in our study population. Difficult direct laryngoscopy occurred in 10.9% of cases, but the first attempt success rate was high at 93.2%. Video laryngoscopy was used in 24.1% of cases. The most common intraoperative complications were hypotension and bradycardia within 15 minutes of induction (24.3% and 17.5%, respectively), and vasopressor use (34.0%). The in-hospital complication rate was 14.7% while the 30-day postoperative complication rate was 27.6%, with ileus > 3 days (5.6%) and postoperative pneumonia (POP) (5.5%) occurring most often. Microstomia was an independent predictor of difficult airway designation [Adjusted Odds Ratio (ORadj): 3.42, 95% Confidence Interval (CI) = 1.13, 10.31, p = 0.029]. ILD was not associated with 30-day POP (ORadj: 1.41, 95% CI = 0.54, 3.71, p = 0.482). A composite of PH/PAH was an independent predictor for 30-day POP (ORadj: 2.61, 95% CI = 1.08, 6.29, p = 0.033), 30-day CHF diagnosis (ORadj: 4.15, 95% CI = 1.33, 12.94, p = 0.014), and 30-day mortality (ORadj: 3.57, 95% CI = 1.24, 10.28, p = 0.018). Conclusions This study identified perioperative characteristics of patients with SSc. Current knowledge on perioperative outcomes in SSc in the surgical population is predominately based on reports of single case reports or small case series. Our findings suggest that intraoperative and airway complications are similar but postoperative complications are high when compared to known incidences in surgical populations. Secondly, this study demonstrates that composite PAH/PH but not ILD was a strong predictor of POP, CHF and 30-day mortality in this patient population.