Background/Purpose: Urticarial vasculitis (UV) is a rare disease characterized by dermal capillary inflammation responsible for long-lasting urticarial lesions. UV can be separated into 2 different entities according to complement fraction levels: hypocomplementemic urticarial vasculitis (HUV), associated with low C1q levels and inconstant anti-C1q antibodies, and normocomplementemic urticarial vasculitis (NUV).UV is characterized by frequent relapses or glucocorticoid (GC)-dependence. We report the efficacy of IL-1β inhibitors against relapsing and GC-dependent UVs.

Methods: This retrospective multicenter study included patients with relapsing and/or GC-dependent UVs treated with IL-1β inhibitors. Patients had to have biopsy-proven UV with long-lasting urticarial lesions and leukocytoclastic vasculitis in a skin biopsy, associated or not with systemic involvement. Relapsing and GC-dependent disease was defined as cutaneous lesions despite GCs at a dose >10 mg/day. For each patient, retrospectively collected data included clinical and biological characteristics, previous and concomitant treatments and theirevolutions, details on IL-1β inhibitors, and disease under IL-1β–inhibitor therapy at discontinuation. Clinical complete response (CR) was defined as total disappearance of cutaneous lesions and other systemic manifestations. Clinical partial response (PR) was defined as attenuation of disease activity without achieving CR. The primary endpoint was the CR rate.

Results: Six patients were included [3 men and 3 women, median age 41 (range 23–62) years], 4 with HUV and 2 with NUV. Median time from diagnosis to starting IL-1β inhibitors was 5.5 (range 1–16) years. All patients had cutaneous and joint involvement, and 2 had gastrointestinal manifestations. All patients received anakinra (100 mg/day); 5 had concomitant treatments, mostly GCs. Under IL-1β inhibitors, 5 (83%) patients achieved CR, within 7 days after starting treatment for 4 (80%) of them, and 1 had a PR. GC use was discontinued for 4 (80%).

In the HUV subgroup, no normalization of complement-fraction levels was obtained. Progressive discontinuation of IL-1β inhibitors was attempted for 4 patients but was constantly associated with relapses within a few days. Safety was good, but 2 patients experienced reactions at the injection site, leading to an anakinra-to-canakinumab switch for 1, with CR persistence. No death, infection or neutropenia occurred.

Conclusion: IL-1β inhibitors had a dramatic—but only suspensive—effect on persistent UV and could be considered alternative agents to treat relapsing and GC-dependent UV.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/dramatic-but-suspensive-effect-of-interleukin-1-inhibitors-on-persistent-urticarial-vasculitis/