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

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.