[Guidelines for uniform management of breath-holding spells].

OBJECTIVES

To investigate the effect of treatment in a series of patients with Sjögren's disease (SjD) associated interstitial lung disease (ILD).

METHODS

Twenty-four primary SjD-ILD patients, followed-up from October 2022 to June 2025 were included in the study. Based on clinical judgement, 12 received treatment for ILD, while 12 did not, following a "watch-and-wait" policy. Participants were evaluated in 2 time points with an interval of 24±6 months. ILD was diagnosed by HRCT according to Fleischner Society definitions, performed at baseline due to respiratory symptoms and/or abnormal pulmonary function tests. Spirometry and diffusing capacity for carbon monoxide (DLCO) were performed at both visits. Progression of ILD was defined as absolute decline of predicted forced vital capacity (FVC) ≥5%.

RESULTS

The treatment regimens of 12 SjD-ILD patients who received treatment included rituximab, mycophenolate mofetil, azathioprine and tocilizumab. The treated group displayed higher extent of ILD on HRCT at baseline visit (median: 20% vs. 10%, p=0.006), more frequently findings of small airways disease on HRCT (58% vs. 8%, p=0.027) and tended to present lower FVC (mean: 81.3% vs. 96.7%, p=0.086) compared to the untreated. FVC and DLCO remained stable between baseline and follow-up visit in both groups. However, the change in DLCO between the two visits was worse in the treated than untreated patients (mean: -11.2% vs. 4.8%, p=0.003). The number of SjD-ILD patients presenting progression of ILD did not differ between the two groups (25% vs. 33%, p=0.999).

CONCLUSIONS

SjD-ILD clinical course is variable, with the most aggressive form to be controlled by immunosuppressive treatment.