Type II cryoglobulinemic vasculitis associated with poor prognosis in patients with Sjögren disease: JAMA

A new study published in the Journal of American Medical Association showed that Type II cryoglobulinemic vasculitis was the most common form of cutaneous vasculitis (CV) in individuals with primary Sjögren disease and was associated with poorer outcomes.

A severe and varied side effect of primary Sjögren disease, cutaneous vasculitis (CV) frequently signifies systemic involvement and a bad prognosis. The patient management requires an understanding of its traits and results. Thus, to characterize the clinical characteristics, treatment, and prognosis of various CV types in patients with Sjögren disease, this study was carried out.

The patients with Sjögren disease and CV from the pathology departments of 3 university hospitals in Paris, France (2011–2021), as well as a nationwide case call, were included in this research. The patients were matched 1:2 with controls who had Sjögren illness but no CV and who satisfied the American College of Rheumatology/European League Against Rheumatism criteria.

The existence of a CV categorized by the dermatological addition of the Chapel Hill classification. Immunological data, clinical features, demographics, and therapies received were documented. The incidence and mortality risk of lymphoma, as well as the responsiveness to treatment, were the main outcomes.

Of the 54 patients with Sjögren disease with CV (median [IQR] age at diagnosis: 42 [27.7-56.0] years; 49 [91%] female), the most common classifications for CV were hypergammaglobulinemic vasculitis (15 patients [28%]) and cryoglobulinemic vasculitis (29 individuals [57%]). The incidence of lymphoma was greater in patients with Sjögren disease and CV than in controls (12 of 54 [13%] vs. 4 of 108 [4%]; P =.04).

Type II cryoglobulinemic vasculitis was linked to higher ESSDAI (EULAR Sjögren Syndrome Disease Activity Index) scores (median [IQR], 15 [12-23]; P =.005), higher mortality or lymphoma risk (hazard ratio, 6.8; 95% CI, 1.8-25.5; P =.005), and more frequent kidney involvement (7 of 24 patients [29%] vs 1 of 25 patients [4%]; P =.02) and peripheral nervous system involvement (15 of 24 patients [63%] vs 3 of 25 patients [12%]; P <.001) than other forms of CV. When compared to other therapies, rituximab-based therapy did not improve survival for individuals with type II cryoglobulinemic vasculitis.

Overall, only type II cryoglobulinemic vasculitis was linked to a poor outcome among the CV subtypes in individuals with Sjögren disease in this cohort analysis. For these individuals, early detection and surveillance for systemic complications are crucial. To determine the best treatment approaches for enhancing results, more investigation is required.

Source:

Breillat, P., Le Guern, V., d’Humières, T., Battistella, M., Legendre, P., Lenormand, C., Kottler, D., Mathian, A., Jachiet, M., El Khalifa, J., Mahévas, T., Comarmond, C., Sene, D., Amoura, Z., Gottenberg, J.-E., Mouthon, L., Bouaziz, J.-D., Mariette, X., Chasset, F., … EMSED (Etude des maladies systémiques en dermatologie) study group and the French ASSESS cohort. (2025). Cutaneous vasculitis in primary Sjögren disease. JAMA Dermatology (Chicago, Ill.). https://doi.org/10.1001/jamadermatol.2025.2665