To the Editor:
We sincerely thank Coppi at al for their interest in our study, “Mortality in patients with Sjögren disease: a prospective cohort study identifying key predictors,”1 and for their thoughtful remarks on the prognostic significance of cardiopulmonary complications in Sjögren disease (SjD).2
As Coppi et al accurately summarized in their letter,2 our multivariate analysis showed that a history of heart failure and pulmonary involvement were associated with a 4.24-fold and 3.31-fold increased risk of mortality, respectively.1 These results highlight the need for rigorous evaluation and close monitoring of affected patients. We wish to clarify, however, that our study did not specify the precise type of pulmonary involvement underlying this increased mortality risk. The variable “pulmonary involvement” did not refer exclusively to pulmonary hypertension, as suggested in the letter by Coppi et al2; it could also include other severe manifestations, such as interstitial lung disease—a frequent and serious extraglandular complication of Sjögren disease (SjD), with an estimated prevalence of 9-24% and subclinical abnormalities detectable in up to 75% of patients.3,4 According to the metaanalysis by Huang et al,5 patients with ILD have a 2.5-fold higher mortality risk than those without pulmonary involvement, in line with observations in other autoimmune diseases, such as systemic lupus erythematosus, inflammatory myopathies, and systemic sclerosis.6-8
Regarding the point on sex-related differences, our study was not specifically powered to detect such disparities, given the predominance of women in our cohort (94.6%). Nevertheless, this remains an important and insufficiently explored area. Future longitudinal research addressing sex-related differences in systemic involvement, cardiovascular risk, and survival outcomes may yield clinically meaningful insights.
We also appreciate the emphasis by Coppi et al on early diagnosis, structured monitoring, and individualized follow-up.2 We believe that incorporating validated systemic activity indexes, serological markers (such as complement C4 and erythrocyte sedimentation rate), and comprehensive cardiopulmonary assessments into a standardized, multidisciplinary framework could help translate prognostic knowledge into improved survival outcomes.
Once again, we thank Coppi et al for their constructive feedback,2 which complements our findings and underscores the importance of multidisciplinary, personalized care strategies for patients with SjD.
Footnotes
FUNDING
This work was funded by the Spanish Society of Rheumatology.
COMPETING INTERESTS
The authors declare no conflicts of interest relevant to this article.
- Copyright © 2026 by the Journal of Rheumatology
