- Indian Rheumatology association
In the realm of rheumatology, Sjögren syndrome sometimes leads to lymphoma, a complication that remains difficult to predict. A recent Greek study sheds light on specific predictors for lymphoma risk, paving way for a preventive, individualized approach to patient care.
Patients diagnosed with Sjögren’s disease-associated MALT lymphoma, with a minimum of 3 years between Sjögren’s disease diagnosis and MALT lymphoma diagnosis, were included and matched 1:1 with Sjögren’s patients without lymphoma. They constructed three datasets: V1 at Sjögren’s diagnosis, V2 3–4 years before lymphoma diagnosis, and V3 0·5–1·5 years before lymphoma diagnosis. Their primary outcome was to identify lymphoma predictors in Sjögren’s disease, present at the time point of Sjögren’s diagnosis and 3–4 years before lymphoma diagnosis. Findings of 80 patients with lymphoma were included in V1 and V3 dataset, and 68 in V2 dataset. Mean age at Sjögren’s diagnosis was 48·6 years in lymphoma group and 48·7 years in control group. At V1 time point, rheumatoid factor was the only independent lymphoma predictor. At V2 time point, rheumatoid factor and ESSDAI ≥5 were independent lymphoma risk factors. The high disease activity during the transition from the V1 to V2 time point was attributed to specific B-cell-derived manifestations, including cryoglobulinemia and glandular, cutaneous, and haematological manifestations. Specific B-cell manifestations in combination with rheumatoid factor indicate a more advanced stage of the lymphomagenesis process.
The advent of new drugs has revolutionized the treatment of RA. Some medications have been identified as risk factors for malignancy. Immunosuppressants promote the development of various malignancies at different frequencies.
In this Japanese study, effect of anti rheumatic drugs on clinicopathologic features of lymphoproliferative diseases (LPD) were studied. 752 RA-LPD and 770 sporadic LPD patients were included. Histopathological analysis revealed a high frequency of Polymorphic LPD, Hodgkin’s lymphoma and EBV-positive mucocutaneous ulcer like LPD in RA-LPD. RA-LPD was associated with a better prognosis than sporadic LPD. Patients with RA-LPD were evaluated based on the anti-rheumatic drugs administered. The MTX plus tacrolimus and MTX plus TNFi groups had different affected site frequencies and histologic subtypes than MTX only group. Moreover, MTX and TNFi may synergistically affect susceptibility to EBV infection. In case of anti-rheumatic drugs administered after LPD onset, tocilizumab (TCZ)-only therapy was associated with lower frequency of regrowth after spontaneous regression than other regimens. Thus, anti-rheumatic drugs administered before LPD onset may influence the clinicopathologic features of RA-LPD, with patterns changing over time. Furthermore, TCZ-only regimens are recommended after LPD onset.
Goules AV, Chatzis L, Pezoulas VC, et al. Identification and evolution of predictors of Sjögren’s disease-associated mucosa-associated lymphoid tissue lymphoma development over time: a case-control study. Lancet Rheumatol. 2024;6(10):e693-e702.
Hoshida Y, Tsujii A, Ohshima S, et al. Effect of Recent Antirheumatic Drug on Features of Rheumatoid Arthritis-Associated Lymphoproliferative Disorders. Arthritis Rheumatol. 2024;76(6):869-881.
