Background/Purpose: Studies from our and other laboratories have shown that clinical, laboratory and serological features at the first evaluation of a patient with primary Sjogren’s syndrome (pSS), can predict non-Hodgkin lymphoma (NHL) development in the future. In this study, we aimed to create a predictive model for NHL development, based on risk factors found at a routine clinical, serological and histopathological evaluation.

Methods: In the present retrospective case control study, clinical, serological and histopathological variables at the time of SS diagnosis (based on revised European/American classification criteria) were recorded in 381 pSS patients and 92 pSS patients complicated by NHL, after thorough chart review. For the identification of predictors for NHL development multivariate logistic regression models were constructed. The probability for NHL development was estimated, by adding the observed risk factors consecutively, one to the other.

Results: Clinical, serological and histopathological manifestations at the time of SS diagnosis, including salivary gland enlargement (SGE), palpable purpura, peripheral neuropathy, Raynaud’s phenomenon, lymphadenopathy, the presence of autoantibodies against Ro/SSA and/or La/SSB antigens, rheumatoid factor (RF) positivity, low C4 complement levels, cryoglobulinemia, monoclonal gammopathy and Tarpley score ≥ 3 in minor salivary gland biopsy, were shown as independent predictors for lymphoma development (Table 1). The probability for NHL development in pSS patients, depending on the presence of the above mentioned risk factors, was 25.5% [OR (95%CI): 4.44 (2.07-7.50), p<0.0001] for patients carrying anti-Ro/anti-La antibodies, 39.4% [OR (95%CI): 3.61 (2.10-6.25), p<0.0001] for those having in addition to autoantibodies a Tarpley score ≥ 3, 54.7% [OR (95%CI): 5.93 (3.15-11.16), p<0.0001],  for those having in addition to the above risk factors SGE, 72.7% [OR (95%CI): 9.16 (2.36-35.58), p=0.001]  for those having also a monoclonal gammopathy and 80% [OR (95%CI): 13.03 (1.43-118.61), p=0.01] for those having additionally lymphadenopathy. This probability reached 100%, if in the latter subset of risk factors any of the remaining found risk factors was added. When ROC curves for the predictive model were fitted, the area under the curve (AUC) was 0.86, 95%CI: 0.81-0.90, p<0.0001.

 Conclusion: This work showed that if more than five (anti-Ro/La antibodies positivity, Tarpley score ≥ 3, SGE, monoclonal gammopathy and lymphadenopathy) of the observed adverse predictors are present at the first evaluation of a pSS patient, the probability for lymphoma development is 100%.

Table 1. Independent adverse predictors for SS related NHL development, identified by multivariate analysis.

SS: Sjogren’s syndrome, SGE: salivary gland enlargement, NHL: non-Hodgkin’s lymphoma, PNS: peripheral nervous system, RF: rheumatoid factor, MSG: minor salivary gland

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/predicting-the-risk-for-lymphoma-development-in-sjogrens-syndrome-an-easy-to-use-clinical-tool/