Paraproteinemias in Sjögren disease: cryoglobulinemia and serum monoclonal gammopathy identify distinct clinical phenotypes and differential lymphoma susceptibility

Semin Arthritis RheumPublished Cross-sectional study

Main finding · LymphomaResult — go to the resultOdds ratio 6.30 95% CI 2.66–14.92

Authors' conclusion

Cryoglobulinemia in SjD associates with vasculitic disease activity, whereas the coexistence of serum MC and cryoglobulins identifies a distinct, high-risk subset characterized by advanced B-cell expansion and increased lymphoma susceptibility.

Study context

Population
Patients with sjögren disease (SjD) in the Italian GRISS registry
Participants
1,202 patients
Exposure
Monoclonal component (MC)-alone, CRYO-alone and MC+CRYO vs SjD without paraproteinemia
  • MC-alonen = 58
  • CRYO-alonen = 32
  • MC+CRYOn = 35
  • Controlsn = 1,077reference
Outcome
Lymphoma diagnosis
Methodological detailsretrospective, multicentre, Italian GRISS registry, logistic regression adjusted for confounders
  • Retrospective
  • Multicentre
  • Italian GRISS registry
  • Logistic regression adjusted for confounders

Main finding

Lymphoma

Odds ratio

6.30

95% CI 2.66–14.92

P < 0.001

MC+CRYOvs Controls
OR 6.30 (95% CI 2.66–14.92); P < 0.001

As reported in the abstract

Observational data: associations, not effects.

Secondary outcomes

Lab lymphoproliferative risk markers and ClinESSDAI vasculitic domains with cryoglobulinemia (CRYO-alone or MC+CRYO)

RF
P < 0.001
Low C4
P < 0.001
Cutaneous
P < 0.001
Renal
P < 0.05
PNS
P < 0.001

ClinESSDAI lymphoproliferative domains in MC+CRYO

Constitutional
P < 0.05
Glandular
P < 0.05
Hematological
P < 0.001
Lymphadenopathy
P < 0.001

Original publication

Paraproteinemias in Sjögren disease: cryoglobulinemia and serum monoclonal gammopathy identify distinct clinical phenotypes and differential lymphoma susceptibility. Semin Arthritis Rheum. Published 19 April 2026. doi:10.1016/j.semarthrit.2026.152986

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