Yazarlar : Presented at EULAR, Jenny Powers

Yayın : DGNews

Yayın Yılı : 2014

Pubmed Linki : http://www.docguide.com/disease-activity-index-sj-gren-s-syndrome-predicts-lymphoma-development?tsid=5

Konu : Lenfoma

Literatür İçeriği :  PARIS -- June 16, 2014 -- The EULAR Sjögren’s Syndrome Disease Activity Index (ESSDAI) may be used to predict the development of lymphoma in patients with Sjögren’s syndrome, according to retrospective findings reported here at the 2014 Annual Congress of the European League Against Rheumatism (EULAR).

“We identified a specific haematological and immunological profile that included cytopenia, hypocomplementaemia, monoclonal band, and cryoglobulinaemia as laboratory predictors of haematological neoplasia,” explained Pilar Brito-Zeron, Hospital Clinic, Barcelona, Spain, on June 13. “If you have a Sjögren’s patient with these features, you have to be very careful, because this patient has a higher probability of developing lymphoma.”

Registry data from 904 patients (follow-up for a mean of 79 months) showed that 25 (3%) patients developed lymphoproliferative disease after Sjögren’s syndrome diagnosis.

When ESSDAI criteria were retrospectively applied to these data, baseline characteristics at diagnosis emerged that were associated with lymphoma development: being male (hazard ratio HR = 5.78), older age (HR = 1.04), C3 values <0.82 g/L (HR = 3.75), C4 values <0.07 g/L (HR = 3.22), monoclonal serum band (HR = 4.23), and cryoglobulins (HR = 4.44). The significant independent variables that were associated with the development of lymphoma by Cox regression were sex, low C3, monoclonal band, and cryoglobulins.

“In Spain, we have one of the largest cohorts of [patients with] Sjögren’s syndrome…in the world,” said Dr. Brito-Zeron.

Adding to this unique opportunity to test the utility of the ESSDAI was a multicentre registry that began in 2005 to collect data regarding the treatment and outcome of patients with Sjögren’s syndrome.

The investigators found that activity scores >1 in the ESSDAI constitutional (HR = 4.06) and haematological (HR = 2.59) domains were associated with the development of lymphoma; haematological activity independently was associated with lymphoma development.

Evaluation with the ESSDAI constitutional domain demonstrated an elevated risk for lymphoma development associated with patients with the highest activity degree for fever >38.5°C, night sweats, and/or involuntary weight loss of >10% (HR = 9.11).

No statistically significant associations were found between higher risk of lymphoma and the remaining domains or the total ESSDAI score.

[Presentation title: Association Between Systematic Activity and Lymphoma in Primary Sjogren Syndrome: Baseline ESSDAI Predictors in 921 Spanish Patients (GEAS-SS Registry). Abstract OP0211]

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