Background: Sjögren disease (SjD) is a systemic autoimmune disorder with a marked female predominance and an increasingly recognised, although incompletely characterised, cardiovascular (CV) burden. This review examines the biological, clinical, and gender-related determinants of CV involvement in women with SjD. Methods: We conducted a structured narrative review of PubMed/MEDLINE covering the period from January 2010 to July 2026, supplemented by backward citation searching to identify relevant earlier studies. Original SjD-specific studies were prioritised, while systematic reviews, meta-analyses, consensus statements, and evidence from related autoimmune or cardiovascular populations were used to provide clinical and mechanistic context. Evidence was interpreted according to study design, population specificity, and recognised methodological limitations. Results: SjD-specific observational evidence supports an association with systemic immune activation, endothelial dysfunction, subclinical vascular injury, and selected cardiopulmonary manifestations. Sex-hormone signalling, menopausal changes, and X-linked immune mechanisms may contribute to female susceptibility, although their relationship with CV outcomes remains largely hypothetical. Evidence concerning coronary microvascular dysfunction, heart failure with preserved ejection fraction, arrhythmias, autonomic dysfunction, and pulmonary hypertension is heterogeneous and frequently derived from small cohorts or indirect evidence. In women, fatigue, dyspnoea, pain, palpitations, and autonomic symptoms may overlap with systemic manifestations of SjD and complicate recognition of concomitant CV disease. Conclusions: Cardiovascular care in women with SjD may benefit from combining established prevention and symptom-driven diagnostic pathways with attention to interstitial lung disease, systemic organ involvement, treatment exposure, and reproductive and hormonal history. The proposed assessment framework is intended to provide practical clinical orientation and to support individualised decision-making, rather than to define a standardised SjD-specific screening pathway. Prospective, sex-disaggregated studies are needed to clarify the prognostic relevance of these factors and to inform future recommendations regarding cardiovascular assessment and follow-up.

Cardiovascular phenotypes in women with Sjögren disease: hormonal, immune, and clinical perspectives / Coppi, F., Angeli, F., Bucciarelli, V., Moscucci, F., Crisci, G., Renda, G., Nodari, S., Pagnoni, G., Bauce, B., Montisci, R., Giuggioli, D., Pizzi, C., Sciacqua, A., Pelà, G., Sciomer, S., Gallina, S., Paolillo, S., Perrone Filardi, P., Sinagra, G., Mattioli, A.V.. - In: CURRENT PROBLEMS IN CARDIOLOGY. - ISSN 0146-2806. - 52:1(2026), pp. "-"-"-". [10.1016/j.cpcardiol.2026.103481]

Cardiovascular phenotypes in women with Sjögren disease: hormonal, immune, and clinical perspectives

Perrone Filardi, Pasquale;Sinagra, Gianfranco;
2026-01-01

Abstract

Background: Sjögren disease (SjD) is a systemic autoimmune disorder with a marked female predominance and an increasingly recognised, although incompletely characterised, cardiovascular (CV) burden. This review examines the biological, clinical, and gender-related determinants of CV involvement in women with SjD. Methods: We conducted a structured narrative review of PubMed/MEDLINE covering the period from January 2010 to July 2026, supplemented by backward citation searching to identify relevant earlier studies. Original SjD-specific studies were prioritised, while systematic reviews, meta-analyses, consensus statements, and evidence from related autoimmune or cardiovascular populations were used to provide clinical and mechanistic context. Evidence was interpreted according to study design, population specificity, and recognised methodological limitations. Results: SjD-specific observational evidence supports an association with systemic immune activation, endothelial dysfunction, subclinical vascular injury, and selected cardiopulmonary manifestations. Sex-hormone signalling, menopausal changes, and X-linked immune mechanisms may contribute to female susceptibility, although their relationship with CV outcomes remains largely hypothetical. Evidence concerning coronary microvascular dysfunction, heart failure with preserved ejection fraction, arrhythmias, autonomic dysfunction, and pulmonary hypertension is heterogeneous and frequently derived from small cohorts or indirect evidence. In women, fatigue, dyspnoea, pain, palpitations, and autonomic symptoms may overlap with systemic manifestations of SjD and complicate recognition of concomitant CV disease. Conclusions: Cardiovascular care in women with SjD may benefit from combining established prevention and symptom-driven diagnostic pathways with attention to interstitial lung disease, systemic organ involvement, treatment exposure, and reproductive and hormonal history. The proposed assessment framework is intended to provide practical clinical orientation and to support individualised decision-making, rather than to define a standardised SjD-specific screening pathway. Prospective, sex-disaggregated studies are needed to clarify the prognostic relevance of these factors and to inform future recommendations regarding cardiovascular assessment and follow-up.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11368/3150879
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