Scleroderma cardiac crisis: A-life-threatening but reversible complication of systemic sclerosis

Autoimmun Rev. 2022 Oct;21(10):103162. doi: 10.1016/j.autrev.2022.103162. Epub 2022 Jul 31.

Abstract

Objective: To describe systemic sclerosis (SSc) heart involvement in the ICU.

Methods: We retrospectively studied patients with previous diagnosis of SSc admitted to the ICU for acute cardiac dysfunction between 2012 and mid-2021.

Results: 9 female patients were included, mainly with diffuse SSc (n = 7, 78%). Six (67%) had digital ulcers. All but one patient complained about physical cardiac symptoms (n = 8, 89%), 5 (56%) had electrocardiogram modifications. Biological exams revealed elevated troponin (705 μg/l [421-1582]) and Nt-pro-BNP (16,062 ng/l [10419-40,738]). Patients exhibited severe left ventricular ejection fraction (LVEF) impairment (20% [10-20] vs 58% [53-60] before ICU admission (p = 0.0002)) requiring vasopressors and/or inotropes for 7 patients (78%) and mechanical ventilation or renal replacement therapy for 4 patients (44%). LVEF spontaneously improved during ICU stay (LVEF 40% [30-40] vs 20% [10-20], p = 0.0007) and returned to baseline within 6 months following ICU discharge (LVEF 53% [31-61] vs 58% [53-60]). Seven (78%) patients survived the ICU-stay and 4 (44%) were alive at 6 months.

Conclusion: We report an uncommon and specific severe acute life-threatening cardiac dysfunction in SSc patients, which can be reversible but remains associated with a poor long-term prognosis, which can be reversible but remains associated with a poor long-term prognosis.

Keywords: Critical care; Heart failure; Systemic sclerosis.

Publication types

  • Review

MeSH terms

  • Female
  • Heart Diseases* / complications
  • Humans
  • Prognosis
  • Retrospective Studies
  • Scleroderma, Localized* / complications
  • Scleroderma, Systemic* / complications
  • Scleroderma, Systemic* / therapy
  • Stroke Volume
  • Troponin
  • Ventricular Function, Left

Substances

  • Troponin