Sjögren Syndrome
ICD-10 M35.0 · ICD-11 4A43.2

Sjögren Syndrome with Pulmonary Bronchitis or Bronchiolitis: What to Do When Inhaled Bronchodilators Are Not Enough

This protocol is for patients with Sjögren syndrome who have pulmonary bronchitis or bronchiolitis as part of active systemic disease. It applies specifically when the initial treatment approach has not achieved the required level of disease control.

The preceding treatment step used inhaled bronchodilators to address pulmonary involvement. Progression to this protocol is indicated when that approach failed to reach the therapeutic target: a reduction of ≥3 points in the global ESSDAI score.

When inhaled therapy proves insufficient, the evidence-based next step involves systemic glucocorticoid therapy. The specific form of administration depends on disease severity. Full sequencing and all clinical decision points are available in the structured protocol.

Target: ESSDAI reduction ≥3 points
Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1136/annrheumdis-2019-216114

Treatment of systemic disease should be tailored to organ-specific severity using the ESSDAI definitions.

GCs should be used at the minimum dose and length of time necessary to control active systemic disease.

GC (recommended dose in mg/kg/day); short-term course whenever possible; consider methylprednisolone pulses in severe cases.

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.

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