Objectives To describe how systemic disease is treated in a large cohort of Spanish patients with primary Sjögren syndrome in daily practice, focusing on the adequacy of drug therapies for the level of systemic activity measured by the ESSDAI score.
Methods By December 2014, our database included 1120 consecutive patients who fulfilled the 2002 classification criteria for primary SS. Therapeutic schedules were classified into 4 categories: no systemic therapies, hydroxychloroquine and/or low dose of glucocorticoids (≤20 mg/d), high dose of glucocorticoids (>20 mg/d) and use of second-line therapies (immunosuppressive agents, intravenous immunoglobulins and/or rituximab).
Results The cohort consisted of 1120 patients, including 1048 (94%) females and 72 (6%) males, with a mean age at diagnosis of 54 years. The main drug-based therapeutic approaches for systemic Sjögren ever used during the follow-up were hydroxychloroquine (HCQ) in 282 (25%) patients, glucocorticoids in 475 (42%, used at doses >20 mg/d in 255 -23%>), immunosuppressive agents in 148 (13%), intravenous immunoglobulins (IVIG) in 25 (2%) and rituximab in 35 (3%) patients. The use of systemic therapies was more frequent in males (p=0.036) and was associated with the presence at diagnosis of anemia (p<0.001), thrombocytopenia (p=0.001), neutropenia (p=0.002), rheumatoid factor (p<0.001), anti-Ro/SS-A (p=0.033), monoclonal band (p=0.002), cryoglobulins (p=0.003), low C3 (p=0.003) and low C4 (p=0.014). The ESSDAI score at diagnosis was higher in patients who received systemic therapy than in those who did not (8.12 vs. 4.20, p<0.001). Multivariate analysis identified neutropenia, rheumatoid factor and the ESSDAI score as independent variables. Hydroxychloroquine was associated with a lower risk of death (adjusted HR of 0.57, 95% 0.34-0.95). We classified 16 (7%) of the 255 patients treated with >20 mg of corticoids and 21/148 (14%) of those treated with immunosuppressive agents patients as inadequately treated, mainly associated with articular involvement of low/moderate activity.
Conclusions The management of systemic Sjögren should be organ-specific, using low doses of corticoids in patients with moderate systemic activity, limiting the use of high doses of corticoids and second-line therapies to refractory or potentially-severe scenarios. The use of systemic therapies for dryness, chronic pain or fatigue is not warranted.
Disclosure of Interest None declared