Objective. To systematically review the prevalence, risk and associated factors of organ damage in Sjögren’s disease (SjD) and to assess its impact on quality of life and long-term outcomes. Methods. A systematic search of PubMed (2005-2025) identified studies assessing damage accrual in SjD. Longitudinal and cross-sectional studies enrolling patients fulfilling the 2002 AECG and/or 2016 ACR/EULAR classification criteria were included. Damage was defined using validated indices, the Sjögren’s Syndrome Damage Index (SSDI) or the Sjögren’s Syndrome Disease Damage Index (SSDDI), or through conceptual definitions of irreversible disease attributable injury. The lymphoma domain was excluded. Study selection followed PRISMA guidelines, and predefined PICO frameworks guided data extraction. Results. Twenty-three studies were included. Glandular damage was reported in 25–86% of patients, while systemic damage affected 9–73%. Older age, longer disease duration, higher baseline ESSDAI, hypergammaglobulinaemia, hypocomplementaemia, and absence of hydroxychloroquine therapy were the most consistent predictors of damage accrual. Pulmonary and renal involvement were associated with increased mortality and hospitalisation rates. Cumulative SSDDI scores correlated with reduced health-related quality of life (HRQoL). Conclusion. Organ damage in SjD is common nd progressive, reflecting sustained immunologic activity and aging-related vulnerability. Damage burden predicts poorer outcomes and diminished HRQoL. Standardisation of damage definitions and assessment tools is essential to improve comparability across studies and to guide preventive therapeutic strategies.

Damage accrual in Sjögren's disease. Prevalence, risk factors and impact on quality of life: a systematic review

Dal Pozzolo, Roberto;Bartoloni, Elena;
2025

Abstract

Objective. To systematically review the prevalence, risk and associated factors of organ damage in Sjögren’s disease (SjD) and to assess its impact on quality of life and long-term outcomes. Methods. A systematic search of PubMed (2005-2025) identified studies assessing damage accrual in SjD. Longitudinal and cross-sectional studies enrolling patients fulfilling the 2002 AECG and/or 2016 ACR/EULAR classification criteria were included. Damage was defined using validated indices, the Sjögren’s Syndrome Damage Index (SSDI) or the Sjögren’s Syndrome Disease Damage Index (SSDDI), or through conceptual definitions of irreversible disease attributable injury. The lymphoma domain was excluded. Study selection followed PRISMA guidelines, and predefined PICO frameworks guided data extraction. Results. Twenty-three studies were included. Glandular damage was reported in 25–86% of patients, while systemic damage affected 9–73%. Older age, longer disease duration, higher baseline ESSDAI, hypergammaglobulinaemia, hypocomplementaemia, and absence of hydroxychloroquine therapy were the most consistent predictors of damage accrual. Pulmonary and renal involvement were associated with increased mortality and hospitalisation rates. Cumulative SSDDI scores correlated with reduced health-related quality of life (HRQoL). Conclusion. Organ damage in SjD is common nd progressive, reflecting sustained immunologic activity and aging-related vulnerability. Damage burden predicts poorer outcomes and diminished HRQoL. Standardisation of damage definitions and assessment tools is essential to improve comparability across studies and to guide preventive therapeutic strategies.
2025
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11391/1612523
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