Treatment of Sjögren Syndrome with Interstitial Lung Disease (NSIP, Organising Pneumonitis, or Lymphoid Interstitial Pneumonitis) and Active Systemic Disease
This protocol covers Sjögren syndrome presenting with interstitial lung disease — specifically non-specific interstitial pneumonia (NSIP), organising pneumonitis, or lymphoid interstitial pneumonitis — in the setting of active systemic disease. The nature and severity of pulmonary involvement, assessed within the broader systemic picture, directly shapes the management approach.
Clinical Context
Interstitial lung disease is a significant organ manifestation within the spectrum of systemic Sjögren syndrome activity. Treatment should be tailored to organ-specific severity, as defined by established disease activity measures. The degree of respiratory impairment influences the intensity of the selected approach.
Treatment Goal
The primary therapeutic target is a clinically meaningful reduction in systemic disease activity — specifically, a decrease of 3 or more points in the global ESSDAI (EULAR Sjögren's Syndrome Disease Activity Index) score.
Treatment Approach
The protocol centres on glucocorticoid therapy. The specific strategy — including how it is administered and at what intensity — is calibrated according to the severity of disease presentation. The complete evidence-based regimen, including full decision criteria, is available in the structured protocol.
Full regimen, sequencing, and recommendations available via the link below.
References
DOI: 10.1136/annrheumdis-2019-216114
- Treatment of systemic disease should be tailored to organ-specific severity using the ESSDAI definitions.
- ILD with NHYA III/IV.
- GCs should be used at the minimum dose and length of time necessary to control active systemic disease, administering pulses of methylprednisolone followed by doses of 0.5 mg/kg/d or lower as induction therapy in severe presentations, and doses <0.5 mg/kg/d in moderate/less-severe presentations, with a final target of withdrawing GCs in inactive patients as soon as possible or at least trying to target a maintenance dose of 5 mg/daily or less with the aid of GC-sparing immunosuppressive agents.
- 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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