Treatment of Sjögren Syndrome with Pulmonary Bronchitis or Bronchiolitis in Active Systemic Disease
Clinical Scenario
This protocol addresses patients with Sjögren syndrome who present with pulmonary bronchitis or bronchiolitis as part of active systemic disease. Managing this overlap requires an approach tailored to the organ-specific severity of systemic involvement, guided by standardised disease activity assessment.
Active Systemic Disease
In this population, systemic disease activity is evaluated using the ESSDAI (EULAR Sjögren's Syndrome Disease Activity Index), which captures the extent of organ involvement including the pulmonary domain. Treatment is calibrated to the severity level identified across these domains.
Treatment Approach (Partial)
The protocol for this scenario involves an inhaled bronchodilator-based intervention targeting the pulmonary component of active disease.
Full regimen details, sequencing, and clinical decision points are available in the complete structured protocol below.
Treatment Goal
Therapeutic response in this setting is defined as a reduction of ≥3 points in the global ESSDAI score, reflecting meaningful improvement in systemic disease activity.
References
- Treatment of systemic disease should be tailored to organ-specific severity using the ESSDAI definitions.
- With respect to the definition of the therapeutic response in systemic SjS, the TF recommends using a reduction of ≥3 points in the global ESSDAI score.
- DOI: 10.1136/annrheumdis-2019-216114
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