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

Clara Vigneron1, Frédéric Pène2, Julien Charpentier3

  • 1Service de médecine intensive-réanimation, Hôpital Cochin, AP-HP. Centre. 27 rue du Faubourg Saint Jacques, 75014 Paris, France; AP-HP. Centre - Université de Paris, 27 rue du Faubourg Saint Jacques, Paris 75014, France.

Autoimmunity Reviews
|August 3, 2022
PubMed

Insights

Systemic sclerosis (SSc) patients admitted to the ICU can experience severe, life-threatening cardiac dysfunction. While this cardiac dysfunction may be reversible, it is linked to a poor long-term prognosis for SSc patients.

Area of Science:

  • Cardiology
  • Rheumatology
  • Intensive Care Medicine

Background:

  • Systemic sclerosis (SSc) is a multisystem autoimmune disease characterized by fibrosis, vascular dysfunction, and immune dysregulation.
  • Cardiac involvement is a significant cause of morbidity and mortality in SSc patients.
  • Acute cardiac dysfunction in SSc patients admitted to the ICU is not well-characterized.

Purpose of the Study:

  • To describe the clinical presentation, management, and outcomes of SSc patients with acute cardiac dysfunction in the ICU.
  • To highlight the specific cardiac manifestations and their reversibility in this patient population.

Main Methods:

  • Retrospective study of SSc patients admitted to the ICU for acute cardiac dysfunction between 2012 and mid-2021.
  • Analysis of clinical data, cardiac biomarkers, electrocardiogram (ECG) findings, echocardiographic parameters, and treatment strategies.
  • Evaluation of patient survival during ICU stay and at 6 months post-discharge.

Main Results:

  • Nine female SSc patients were included, predominantly with diffuse SSc and digital ulcers.
  • Most patients presented with cardiac symptoms and ECG modifications, along with elevated troponin and Nt-pro-BNP levels.
  • Severe left ventricular ejection fraction (LVEF) impairment was observed, requiring intensive cardiovascular support; however, LVEF showed spontaneous improvement during ICU stay and returned to baseline within 6 months.
  • Seven patients (78%) survived the ICU stay, but only 4 (44%) were alive at 6 months.

Conclusions:

  • SSc patients can experience uncommon, severe, and life-threatening acute cardiac dysfunction in the ICU.
  • This cardiac dysfunction, though potentially reversible, is associated with a poor long-term prognosis.
  • Early recognition and management of cardiac dysfunction are crucial in SSc patients admitted to the ICU.
Abstract

Keywords:
Critical careHeart failureSystemic sclerosis

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