August 08, 2025
2 min learn
Key takeaways:
- Adults with Sjögren’s illness and cutaneous vasculitis usually tend to develop non-Hodgkin’s lymphoma.
- The upper threat was noticed solely amongst sufferers with the subtype sort II cryoglobulinemic vasculitis.
Adults with Sjögren’s illness sophisticated by a subtype of cutaneous vasculitis usually tend to develop non-Hodgkin’s lymphoma and an infection, knowledge from a retrospective examine present.
Cutaneous vasculitis (CV) is a extreme complication of main Sjögren’s illness, typically indicating systemic involvement and a poor prognosis, Paul Breillat, MD, a medical fellow within the division of inside medication on the Nationwide Reference Heart for Uncommon Systemic Autoimmune Illnesses, Hôpital Cochin, Paris, and colleagues wrote in JAMA Dermatology.
Knowledge derived from Breillat P, et al. JAMA Dermatol. 2025;doi:10.1001/jamadermatol.2025.2665.
Understanding the traits and outcomes of CV is important for affected person administration; nevertheless, few research have investigated the variations between the assorted CV subtypes, the researchers wrote.
Paul Breillat
“Though the event of CV through the course of Sjögren’s illness is related to a poor prognosis — significantly with an elevated threat for creating lymphoma — this threat is nearly solely related to the subgroup of vasculitis secondary to blended sort II cryoglobulinemia, with a monoclonal part,” Breillat informed Healio. “We additionally present that though rituximab has demonstrated medical efficacy in treating sort II cryoglobulinemia, it nonetheless stays to be confirmed that this therapy prevents the incidence of non-Hodgkin’s lymphoma in these sufferers.”
For the retrospective examine, the researchers analyzed knowledge from 54 adults with Sjögren’s
illness and CV (91% girls), primarily cryoglobulinemic vasculitis and hypergammaglobulinemic vasculitis subtypes, in contrast with 108 controls with Sjögren’s
illness however with out CV, matched by follow-up length.
A lot of the CV instances related to Sjögren’s illness had been categorised as cryoglobulinemic vasculitis (57%), predominantly sort II (83%) and hypergammaglobulinemic vasculitis (28%). The primary cutaneous findings included palpable purpura (93%), ulcerated/necrotic lesions (27%) and long-lasting urticaria and livedo reticularis (7%). Extracutaneous involvement was reported for 33% of sufferers, principally within the peripheral nervous system (28%). Throughout follow-up, 13% of sufferers developed lymphoma and 13% died.
In contrast with controls with Sjögren’s illness however with out CV, these with Sjögren’s illness and CV had a better lymphoma threat (13% vs. 4%; P = .04). Sort II cryoglobulinemic vasculitis was related to elevated mortality/lymphoma threat, kidney involvement and peripheral nerve involvement.
Researchers noticed that sufferers with sort II cryoglobulinemic vasculitis had been extra more likely to develop lymphoma (21% vs. 0; P = .02) in contrast with those that had different subtypes of small vessel vasculitis and had been extra more likely to die throughout follow-up (29% vs. 0; P = .004).
In additional analyses, rituximab (Rituxan, Genentech) confirmed no survival profit for sufferers with sort II cryoglobulinemic vasculitis in contrast with sufferers who acquired different therapy, in keeping with the researchers.
“We consider that this work ought to encourage clinicians to be significantly vigilant within the follow-up of sufferers with Sjögren’s illness and kind II cryoglobulinemic vasculitis, as the chance for lymphoma or demise — significantly from an infection — was discovered to be greater on this group,” Breillat informed Healio. “Potential research are wanted to find out whether or not therapy with rituximab or different anti-CD20 monoclonal antibodies cut back the chance for creating lymphoma in sufferers with Sjögren’s syndrome sophisticated by sort II cryoglobulinemic vasculitis.”
For extra data:
Paul Breillat, MD, may be reached at paul.breillat@inserm.fr; X: @PBreillat.
