2 research outputs found
Clinical picture, outcome and predictive factors of lymphoma in primary Sjogren's syndrome: results from a harmonized dataset (1981-2021)
Objectives Primary Sjogren’s Syndrome (pSS) carries the highest risk for
non-Hodgkin’s lymphoma (NHL) development among systemic autoimmune
diseases. However, the paucity of data on the long-term survival of
those patients and the lack of established predictors for each lymphoma
histologic subtype prompted our present study. Methods We
retrospectively analysed 121 patients diagnosed with NHL according to
the WHO classification criteria. All patients fulfilled the 2016
ACR-EULAR classification criteria for pSS. Cumulative clinical,
laboratory, radiologic, treatment regimens and histologic data were
recorded, harmonized and analysed. Overall survival (OS) and event-free
survival (EFS) curves were calculated. A mucosa-associated lymphoid
tissue lymphoma (MALTL) prediction model was developed by applying
innovative data-driven analysis of clinical features present at the time
of pSS diagnosis. Results MALTLs constituted the majority of lymphomas
(92/121, 76.0%) followed by diffuse large B-cell lymphomas (DLBCL)
(11/121, 9.0%) and nodal marginal zone lymphomas (NMZL) (8/121, 7%).
MALTLs show salivary glands localization, limited disease and often bone
marrow and nodal involvement. The 10-year OS and EFS rates were 79% and
45.5% for MALTLs, 40.9% and 24.2% for DLBCL and 46% and 31% for
NMZL. Cryoglobulinemia, focus score and the total EULAR SS Disease
Activity Index (ESSDAI) composite index at pSS diagnosis were proven
independent MALTL predictors. Even though MALTLs have a comparatively
good survival outlook, they are accompanied by frequent events
throughout their clinical course. Conclusions Common features of pSS,
present at diagnosis, can predict future lymphomagenesis meriting a more
intensive follow-up plan
Combined seronegativity in Sjögren's syndrome
Objectives: To describe the clinical spectrum of Sjögren's syndrome (SS) patients with combined seronegativity. Methods: From a multicentre study population of consecutive SS patients fulfilling the 2016 ACR-EULAR classification criteria, patients with triple seronegativity [anti-Ro/SSA(-), anti-La/SSB(-), RF(-) and ANA(+)] and quadruple seronegativity [anti-Ro/SSA(-), anti-La/SSB(-), RF(-) and ANA(-)] were identified retrospectively. Both groups were matched in an 1:1 ratio with 2 distinct control SS groups: i) classic anti-Ro/SSA seropositive patients [SS(+)] and ii) classic anti-Ro/SSA seropositive patients with negative rheumatoid factor [SS(+)/RF(-)] to explore their effect on disease expression. Clinical, laboratory and, histologic features were compared. A comparison between triple and quadruple seronegative SS patients was also performed. Reesults: One hundred thirty-five SS patients (8.6%) were identified as triple seronegative patients and 72 (4.5%) as quadruple. Triple seronegative patients had lower frequency of peripheral nervous involvement (0% vs. 7.2% p=0.002) compared to SS(+) controls and lower frequency of interstitial renal disease and higher prevalence of dry mouth than SS(+)/RF(-) controls. Quadruple seronegative patients presented less frequently with persistent lymphadenopathy (1.5% vs. 16.9 p=0.004) and lymphoma (0% vs. 9.8% p=0.006) compared to SS(+) controls and with lower prevalence of persistent lymphadenopathy (1.5% vs. 15.3% p=0.008) and higher frequency of dry eyes (98.6% vs. 87.5% p=0.01) and autoimmune thyroiditis (44.1% vs. 17.1% p=0.02) compared to SS(+)/RF(-) SS controls. Study groups comparative analysis revealed that triple seronegative patients had higher frequency of persistent lymphadenopathy and lymphoma, higher focus score and later age of SS diagnosis compared to quadruple seronegative patients. Conclusions: Combined seronegativity accounts for almost 9% of total SS population and is associated with a milder clinical phenotype, partly attributed to the absence of rheumatoid factor