Adverse outcome in SS-associated NHL—predictors revealed

Non-Hodgkin lymphoma (NHL) is a potentially fatal complication affecting 5–10% of patients with Sjögren syndrome. New findings suggest that high international prognostic index (IPI) and EULAR Sjögren syndrome disease activity index (ESSDAI) scores, measured at the time of NHL diagnosis, are predictors of adverse outcome and could potentially be used to guide therapeutic decision-making. This retrospective study compared 77 patients who had Sjögren syndrome and histologically confirmed NHL with 167 controls who had Sjögren syndrome but not NHL. High ESSDAI (total >10) scores predicted poor survival, whereas high IPI scores (presence of 3–4 factors) predicted a substantially increased risk of death, relapse, treatment failure, NHL progression and histological transformation.