Sjögren's disease & overlap diseases

Treatment modalities of marginal zone lymphoma and overall survival, haematological response, and underlying Sjögren's disease activity: a multicentre, retrospective, observational study

Lancet RheumatolPublished Retrospective cohort study

Main finding · New SjD systemic activity (marginal zone lymphoma)Result — go to the resultHazard ratio 0.43 95% CI 0.21–0.90

Authors' conclusion

A systemic treatment strategy for Sjögren's disease-associated lymphoma, rather than localised treatment or a watch and wait strategy, reduces the risk of new Sjögren's disease systemic activity and combination therapy is associated with decreased risk of lymphoma relapse.

Study context

Population
Patients with SjD (2016 ACR-EULAR criteria) who developed lymphoma diagnosed before Jan 1, 2020; two expert centres in Paris, the French ASSESS cohort and Club Rhumatismes et Inflammation practitioners
Participants
50 / 13 / 19 / 32 / 18
Exposure
Systemic treatment vs localised treatment or watch and wait at lymphoma diagnosis; chemotherapy plus anti-CD20 combination vs monotherapy as first-line therapy
  • Systemic treatmentn = 50
  • Localised treatmentn = 13reference
  • Watch and waitn = 19reference
  • Combination therapyn = 32
  • Monotherapyn = 18reference
Outcome
Effect of lymphoma treatment on three endpoints: lymphoma progression-free survival, new SjD systemic activity, and overall survival
Methodological detailsmulticentre, observational, inverse probability of treatment weighting
  • Multicentre
  • Observational
  • Inverse probability of treatment weighting

Main finding

As reported in the abstract

New SjD systemic activity (marginal zone lymphoma) · median follow-up 7 years

Hazard ratio

0.43

95% CI 0.21–0.90

Systemic treatmentvs Localised treatment or watch and wait
HR 0.43 (95% CI 0.21–0.90)

Observational data: associations, not effects.

Further findings

OutcomeHR for lymphoma progression (95% CI)
Lymphoma progression-free survival, first-line combination therapyvs monotherapyInverse probability of treatment weighting0.36 (0.14–0.94)

Mortality by pulmonary localisation (MZL, exploratory multivariable analysis)

HR for death (95% CI)
Pulmonary localisationMultivariable analysis7.92 (1.70–37.0)

New SjD systemic activity and overall survival, combination therapy vs monotherapy

No differences in new SjD systemic activity or overall survival according to combination therapy or monotherapy

Parts of this Brief are withheld: the automatic checks could not confirm them against the abstract.

Original publication

Treatment modalities of marginal zone lymphoma and overall survival, haematological response, and underlying Sjögren's disease activity: a multicentre, retrospective, observational study. Lancet Rheumatol. Published 22 August 2024. doi:10.1016/s2665-9913(24)00198-x

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