Cutaneous Vasculitis Subtype Associated With Poor Outcomes in Sjögren Disease

A new cohort study investigating cutaneous vasculitis (CV) in people with primary Sjögren disease has found an association between type II cryoglobulinemic vasculitis and poor prognosis.1

“Several types of CV have been described in the setting of Sjögren disease, including cryoglobulinemic vasculitis, urticarial vasculitis, and hypergammaglobulinemic vasculitis (HGV). Few studies have investigated the clinical and biological differences between these different entities.2 However, within this spectrum of CV in Sjögren disease, the prognosis and therapeutic management may be very different and need to be clarified in the era of new targeted therapies,” lead investigator Paul Breillat, MD, Service de Médecine Interne, Centre de Référence Maladies Systémiques Autoimmunes et Autoinflammatoires Rares d’Ile de France, de l’Est et de l’Ouest, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP)-CUP, Université de Paris Cité, Paris, France, and colleagues wrote.1

Breillat and colleagues conducted a multicenter cohort study including patients with Sjögren disease and CV from pathology departments of 3 university hospitals in Paris, France, between 2011-2021, and a national case call. Participants had to meet American College of Rheumatology/European League Against Rheumatism criteria and were matched 1:2 to control participants with Sjögren disease but without CV. Investigators collected and analyzed data between March 2023 and March 2025.

Altogether, the study included 54 patients with CV and Sjögren disease with a median age at diagnosis of CV of 42 years (IQR, 27.7-56.0) and 91% (n = 49) were female. The study also included 108 control participants. CV subtypes were more frequently classified as cryoglobulinemic vasculitis (n = 29; 57%) or hypergammaglobulinemic vasculitis (n = 15 patients; 28%).1

Breillat and colleagues found that compared to control participants, those with Sjögren disease and CV had a higher lymphoma incidence (n = 12; 13% vs n = 4; 4%; respectively, P = .04). Compared to other types of CV, type II cryoglobulinemic vasculitis was associated with increased mortality or lymphoma risk (hazard ratio, 6.8 [95% CI, 1.8-25.5]; P = .005), higher EULAR Sjögren Syndrome Disease Activity Index (ESSDAI) scores (median [IQR], 15 [12-23]; P = .005), and more frequent kidney involvement (n = 7 of 24; 29% vs n = 1 of 25; 4%; P = .02) and peripheral nervous system involvement (n = 15 of 24; 63% vs n = 3 of 25; 12%; P < .001). They also found that rituximab-based therapy did not show any survival benefit for patients with type II cryoglobulinemic vasculitis compared to other treatments.1

“These findings are important, as they emphasize that type II cryoglobulinemic vasculitis should be distinguished from other CV, even though vasculitis is considered a severity factor in the ESSDAI score regardless of subtype… Further studies in larger cohorts are needed to clarify this potential effect,” Breillat and colleagues concluded.1 “… among patients with CV-complicated Sjögren disease, only type II cryoglobulinemic vasculitis was associated with severe visceral involvement, higher risk of non-Hodgkin lymphoma, and mortality. This highlights the need for a specific monitoring for these patients.”

The investigators acknowledged limitations of the study, including its retrospective design and potential missing data. Furthermore, half of the patients were diagnosed with CV based on clinical criteria rather than pathological confirmation, although patients with uncertain or doubtful diagnosis of CV were excluded.

REFERENCES

1. Breillat P, Véronique Le Guern, d’Humières T, et al. Cutaneous Vasculitis in Primary Sjögren Disease. JAMA Dermatology. Published online August 6, 2025. doi: 10.1001/jamadermatol.2025.2665

2. Quartuccio L, Isola M, Baldini C, et al. Clinical and biological differences between cryoglobulinaemic and hypergammaglobulinaemic purpura in primary Sjögren’s syndrome: results of a large multicentre study. Scandinavian Journal of Rheumatology. 2014;44(1):36-41. doi: 10.3109/03009742.2014.923931