The heightened risk of non-Hodgkin lymphoma (NHL) development in primary Sjogren 
syndrome (SS) is well established. Several adverse clinical and laboratory 
predictors have been described. In the current work, we aimed to formulate a 
predictive score for NHL development, based on clinical, serological, and 
histopathological findings at the time of SS diagnosis. In the present 
case-control study of 381 primary SS patients and 92 primary SS patients with 
concomitant NHL, clinical, serological, and histopathological variables at the 
time of SS diagnosis were retrospectively recorded. For the identification of 
predictors for NHL development univariate and multivariate models were 
constructed. Salivary gland enlargement (SGE), lymphadenopathy, Raynaud 
phenomenon, anti-Ro/SSA or/and anti-La/SSB autoantibodies, rheumatoid factor 
(RF) positivity, monoclonal gammopathy, and C4 hypocomplementemia were shown to 
be independent predictors for NHL development. On the basis of the number of 
independent risk factors identified, a predictive risk score for NHL development 
was formulated. Thus, patients presenting with ≤2 risk factors had a 3.8% 
probability of NHL development, those with 3 to 6 risk factors 39.9% (OR 
(95%CI): 16.6 [6.5-42.5], P < 0.05), while in the presence of all 7 risk factors 
the corresponding probability reached 100% (OR [95%CI]: 210.0 [10.0-4412.9], 
P < 0.0001). In conclusion, an easy to use diagnostic scoring tool for NHL 
development in the context of SS is presented. This model is highly significant 
for the design of early therapeutic interventions in high risk SS patients for 
NHL development.