Systemic treatment reduces risk for new Sjögren’s disease activity in presence of lymphoma
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Key takeaways:
- In marginal zone lymphoma, systemic treatment lowered new Sjögren’s disease activity risk, while combination therapy decreased lymphoma relapse risk.
- Pulmonary involvement negatively correlated with survival.
Systemic treatment strategies lower the risk for new Sjögren’s disease activity when there are complications with marginal zone lymphoma, according to a study published in The Lancet Rheumatology.
The researchers also found that a combination of anti-CD20 therapy and chemotherapy resulted in a lower risk for lymphoma relapse.
“Sjögren’s disease is the autoimmune disease with the highest risk of lymphoma, surpassing both systemic lupus erythematosus and rheumatoid arthritis,” Juliette Rocca, MD, of Bicêtre Hospital, at Paris-Saclay University, and Sorbonne University, in France, and colleagues wrote.
“There is a gap in the management of lymphomas complicating Sjögren’s disease,” they added. “Although autoimmune disease activity is a major driver of lymphomagenesis in this disease entity, no specific guideline takes autoimmune disease activity into account. Additionally, there has been no evaluation of the effect of lymphoma treatment on autoimmune disease activity. Thus, the close link between lymphoma and autoimmune disease activity might lead to the hypothesis that choice of treatment could modify the prognosis of autoimmune disease.”
To learn more about non-Hodgkin lymphomas in Sjögren’s disease, including treatment strategies and prognoses, Rocca and colleagues conducted a retrospective, observational study of 106 patients with Sjögren’s disease diagnosed with lymphoma before January 2020. The researchers used patient data from two Paris centers — a French multicenter, prospective cohort, and practitioners registered with a French working group.
The study involved three main outcomes: lymphoma-progression-free survival; overall survival; and new Sjögren’s disease systemic activity, defined as new or worsening systemic involvement in any domain of the EULAR Sjögren’s Syndrome Disease Activity Index.
Overall, mucosa-associated lymphoid tissue lymphoma — a type of marginal zone lymphoma — was the most frequently observed histological subtype (64%), followed by other marginal zone subtypes (13%), according to the researchers. The median follow-up time for patients with any marginal zone lymphoma was 6.5 years (IQR, 4-13), and in these patients, pulmonary localization was associated with mortality (HR = 7.92; 95% CI, 1.7-37), based on multivariable analysis.
Treatments to address marginal zone lymphoma included a “watch-and-wait” approach (23%); first-line localized treatment, either surgery or radiotherapy (16%); and first-line systemic treatment (61%), the researchers wrote. Among these patients, 32% had lymphoma relapse, 11% died during follow-up, and 33% experienced new Sjögren’s disease systemic activity.
According to the researchers, patients with marginal zone lymphoma who received systemic treatment at diagnosis demonstrated a lower risk for new Sjögren’s disease activity (HR = 0.43; 95% CI, 0.21-0.9), based on inverse probability of treatment weighing analysis. Compared with first-line monotherapy, those who received chemotherapy and anti-CD20 therapy saw improved lymphoma-progression-free survival (HR = 0.36; 95% CI, 0.14-0.94).
“Our results show that we need to harmonize the management of lymphoma complicating Sjögren’s disease,” Rocca and colleagues wrote. “Establishing therapeutic guidelines specific to Sjögren’s disease-associated lymphomas would be a useful step, as is the case for Helicobacter pylori-associated gastric MALT lymphoma.
“Our study suggests that the decision to treat lymphoma might affect the prognosis of Sjögren’s disease, emphasizing the continuum between autoimmunity and lymphoma in this condition,” they added. “With regard to active treatment versus watch and wait, the activity of Sjögren’s disease beyond lymphoma is a strong argument in favor of systemic treatment. Finally, in patients for whom first-line systemic treatment is considered, combination therapy with anti-CD20 and chemotherapy is likely to reduce the risk of lymphoma relapse compared with monotherapy.”
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