Cutaneous vasculitis subtype ups cancer, infection risk in Sjögren’s disease

Key takeaways:

• Adults with Sjögren’s disease and cutaneous vasculitis are more likely to develop non-Hodgkin’s lymphoma.
• The higher risk was observed only among patients with the subtype type II cryoglobulinemic vasculitis.

Adults with Sjögren’s disease complicated by a subtype of cutaneous vasculitis are more likely to develop non-Hodgkin’s lymphoma and infection, data from a retrospective study show.

Cutaneous vasculitis (CV) is a severe complication of primary Sjögren’s disease, often indicating systemic involvement and a poor prognosis, Paul Breillat, MD, a clinical fellow in the department of internal medicine at the National Reference Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, Paris, and colleagues wrote in JAMA Dermatology.

Understanding the characteristics and outcomes of CV is essential for patient management; however, few studies have investigated the differences between the various CV subtypes, the researchers wrote.

Paul Breillat

“Although the development of CV during the course of Sjögren’s disease is associated with a poor prognosis — particularly with an increased risk for developing lymphoma — this risk is almost exclusively associated with the subgroup of vasculitis secondary to mixed type II cryoglobulinemia, with a monoclonal component,” Breillat told Healio. “We also show that although rituximab has demonstrated clinical efficacy in treating type II cryoglobulinemia, it still remains to be proven that this treatment prevents the occurrence of non-Hodgkin’s lymphoma in these patients.”

For the retrospective study, the researchers analyzed data from 54 adults with Sjögren’s

disease and CV (91% women), primarily cryoglobulinemic vasculitis and hypergammaglobulinemic vasculitis subtypes, compared with 108 controls with Sjögren’s

disease but without CV, matched by follow-up duration.

Most of the CV cases associated with Sjögren’s disease were classified as cryoglobulinemic vasculitis (57%), predominantly type II (83%) and hypergammaglobulinemic vasculitis (28%). The main 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 patients, mostly in the peripheral nervous system (28%). During follow-up, 13% of patients developed lymphoma and 13% died.

Compared with controls with Sjögren’s disease but without CV, those with Sjögren’s disease and CV had a higher lymphoma risk (13% vs. 4%; P = .04). Type II cryoglobulinemic vasculitis was associated with increased mortality/lymphoma risk, kidney involvement and peripheral nerve involvement.

Researchers observed that patients with type II cryoglobulinemic vasculitis were more likely to develop lymphoma (21% vs. 0; P = .02) compared with those who had other subtypes of small vessel vasculitis and were more likely to die during follow-up (29% vs. 0; P = .004).

In further analyses, rituximab (Rituxan, Genentech) showed no survival benefit for patients with type II cryoglobulinemic vasculitis compared with patients who received other treatment, according to the researchers.

“We believe that this work should encourage clinicians to be particularly vigilant in the follow-up of patients with Sjögren’s disease and type II cryoglobulinemic vasculitis, as the risk for lymphoma or death — particularly from infection — was found to be higher in this group,” Breillat told Healio. “Prospective studies are needed to determine whether treatment with rituximab or other anti-CD20 monoclonal antibodies reduce the risk for developing lymphoma in patients with Sjögren’s syndrome complicated by type II cryoglobulinemic vasculitis.”

For more information:

Paul Breillat, MD, can be reached at paul.breillat@inserm.fr; X: @PBreillat.