This protocol applies to patients with active systemic Sjögren syndrome who have developed glomerulonephritis — specifically membranous glomerulonephritis, mesangial glomerulonephritis, or focal segmental glomerulosclerosis — without cryoglobulinaemia. Treatment of systemic disease is tailored to organ-specific severity using the ESSDAI definitions.
The preceding treatment step used glucocorticoids (oral regimen for moderate disease, or intravenous methylprednisolone pulses followed by an oral regimen for severe disease). Escalation to the current protocol is indicated when that approach fails to achieve a reduction of ≥3 points in the global ESSDAI score.
When glucocorticoids have not produced an adequate systemic response, an immunosuppressive agent becomes the next consideration. Multiple agents have been used in primary Sjögren syndrome — the full protocol details the options and the factors that inform selection between them.
The primary therapeutic target is a reduction of ≥3 points in the global ESSDAI score — the task-force definition of a meaningful systemic response in Sjögren syndrome.
DOI: 10.1136/annrheumdis-2019-216114
Treatment of systemic disease should be tailored to organ-specific severity using the ESSDAI definitions.
Renal failure; rapidly-progressive glomerulonephritis; hypokalaemic paralysis.
The lack of head-to-head studies comparing the efficacy and safety profile of immunosuppressive agents in primary SjS-2002 (leflunomide, methotrexate, azathioprine, mycophenolate, cyclophosphamide) does not permit a recommendation on the use of one agent over another.
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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