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. 2025 Jul 4:e70094.
doi: 10.1111/eci.70094. Online ahead of print.

First-line diagnostic tests to intercept primary heart involvement in systemic sclerosis: Clinical associations from the SPRING-SIR registry

Antonio Tonutti  1   2 , Francesca Motta  1   2 , Rossella De Angelis  3   4 , Edoardo Cipolletta  3 , Clodoveo Ferri  5   6 , Gianluigi Bajocchi  7 , Silvia Bellando-Randone  8 , Cosimo Bruni  8 , Martina Orlandi  6 , Giovanni Zanframundo  9 , Rosario Foti  10 , Giovanna Cuomo  11 , Alarico Ariani  12 , Edoardo Rosato  13 , Gemma Lepri  8 , Francesco Girelli  14 , Elisabetta Zanatta  15   16 , Silvia Laura Bosello  17 , Ilaria Cavazzana  18 , Francesca Ingegnoli  19 , Fabio Cacciapaglia  20 , Giuseppe Murdaca  21 , Giuseppina Abignano  22 , Giorgio Pettiti  23 , Alessandra Della Rossa  24 , Maurizio Caminiti  25 , Anna Maria Iuliano  26 , Giovanni Ciano  27 , Lorenzo Beretta  28 , Gianluca Bagnato  29 , Ennio Lubrano  30 , Ilenia De Andres  31 , Luca Idolazzi  32 , Marta Saracco  33 , Cecilia Agnes  34 , Corrado Campochiaro  35 , Marco Fornaro  36 , Federica Lumetti  6 , Amelia Spinella  6 , Luca Magnani  7 , Giacomo De Luca  35   36 , Veronica Codullo  9 , Elisa Visalli  10 , Carlo Iandoli  11 , Antonietta Gigante  13 , Greta Pellegrino  37 , Erika Pigatto  38 , Maria Grazia Lazzaroni  18 , Enrico De Lorenzis  17 , Gianna Mennillo  22 , Marco Di Battista  24 , Giuseppa Pagano-Mariano  25 , Federica Furini  39 , Licia Vultaggio  39 , Simone Parisi  40 , Clara Lisa Peroni  40 , Gerolamo Bianchi  41 , Enrico Fusaro  40 , Gian Domenico Sebastiani  26 , Marcello Govoni  39 , Salvatore D'Angelo  22   42 , Franco Cozzi  38 , Franco Franceschini  18 , Serena Guiducci  8 , Lorenzo Dagna  35   36 , Andrea Doria  15 , Dilia Giuggioli  6 , Valeria Riccieri  37 , Carlo Salvarani  43 , Florenzo Iannone  20 , Marco Matucci-Cerinic  35   36 , Carlo Selmi  1   2 , Maria De Santis  1   2 ; SPRING‐SIR (Systemic Sclerosis PRogression INvestiGation group of the Italian Society for Rheumatology)
Collaborators, Affiliations
Free article

First-line diagnostic tests to intercept primary heart involvement in systemic sclerosis: Clinical associations from the SPRING-SIR registry

Antonio Tonutti et al. Eur J Clin Invest. .
Free article

Abstract

Introduction: Primary heart involvement (pHI) is an overlooked and poorly characterised complication of systemic sclerosis (SSc), associated with the risk of heart failure, arrhythmia and death. Despite consensus definition by the World Scleroderma Foundation/Heart Failure Association (WSF/HFA), diagnostic criteria and risk factors remain poorly elucidated.

Methods: Out of 1922 patients in the Italian national SPRING registry, we excluded those with potentially confounding conditions according to WSF/HFA, and those with incomplete ECG or echocardiographic assessment, resulting in 600 subjects with clearly defined parameters to intercept SSc-pHI. Cross-sectional and longitudinal analyses were performed to identify factors associated with pHI.

Results: ECG and/or echocardiographic signs of SSc-pHI were identified in 25% of patients at enrollment and were associated with older age (OR 1.04; 95% CI 1.02-1.06), diffuse cutaneous SSc (OR 1.85; 95% CI 1.05-3.26) and intestinal symptoms (OR 1.79; 95% CI 1.03-3.08). Diastolic dysfunction (62%) and conduction disturbances (34%) were the most frequent phenotypes, while diffuse hypokinesia with reduced ejection fraction was the least common (3%). During follow-up, new-onset signs of pHI were observed in an additional 25% of patients, particularly in those with skeletal muscle involvement (HR 2.83; 95% CI 1.01-7.73).

Conclusions: pHI is a severe complication potentially affecting one-quarter of patients with SSc. Early detection is crucial, particularly in those with diffuse skin fibrosis, muscular involvement and intestinal manifestations.

Keywords: autoimmunity; connective tissue disease; echocardiography; immunology; risk factors; systemic sclerosis.

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References

REFERENCES

    1. De Luca G, Matucci‐Cerinic M, Mavrogeni SI. Diagnosis and management of primary heart involvement in systemic sclerosis. Curr Opin Rheumatol. 2024;36(1):76‐93.
    1. Allanore Y, Meune C, Kahan A. Outcome measures for heart involvement in systemic sclerosis. Rheumatology (Oxford). 2008;47(Suppl 5):v51‐v53.
    1. Meune C, Vignaux O, Kahan A, Allanore Y. Heart involvement in systemic sclerosis: evolving concept and diagnostic methodologies. Arch Cardiovasc Dis. 2010;103(1):46‐52.
    1. Bruni C, Ross L. Cardiac involvement in systemic sclerosis: getting to the heart of the matter. Best Pract Res Clin Rheumatol. 2021;35(3):101668.
    1. Bruni C, Buch MH, Furst DE, et al. Primary systemic sclerosis heart involvement: a systematic literature review and preliminary data‐driven, consensus‐based WSF/HFA definition. J Scleroderma Relat Disord. 2022;7(1):24‐32.