Background/Purpose: Immunoglobulin A vasculitis (IgAV) is a small vessel vasculitis marked by IgA, deposition, palpable purpura with lower body predilection and variable joints, kidney and gastrointestinal tract involvement. In 2018, the addendum to the 2012 Chapel Hill Consensus Conference established the recognition of an exclusively or predominantly cutaneous form of the disease, more common in adults. Despite its frequency, standardized management of cutaneous IgA vasculitis remains elusive. The objective of this study was to compare colchicine, dapsone and glucocorticoids (GCs) to induce remission in newly diagnosed or relapsing skin-limited/dominant IgA vasculitis.

Methods: Patients with cutaneous IgAV treated with colchicine, dapsone, or GCs were included between January 1, 2014, and May 1, 2024. Data were collected retrospectively from 38 tertiary centers across eight countries in Europe. Inclusion criteria were age >8 years, biopsy-proven diagnosis of IgAV with evidence of IgA deposition in the skin and/or other organs, newly diagnosed or relapsing IgAV with active skin manifestations; absence of severe organ involvement; and a minimum follow-up of 3 months. The primary endpoint was to evaluate the proportion of patients achieving sustained remission of cutaneous involvement at day 90 (SRD90). Treatments were compared pairwise using inverse probability treatment weighting with the propensity score to adjust for confounders. Variables differing between responders and non-responders and susceptible to be associated with the endpoint were included in the propensity score. Patients receiving multiple therapies were counted per treatment line. Missing data were addressed with multiple imputation by chained equations.

Results: Of the 407 cases identified, 208 patients (114 males, 89 females; median age 44.9 years) were included in the study. Most were enrolled at initial diagnosis (174). At day 90, 109 patients (52.4%) had achieved sustained remission of cutaneous involvement. A total of 164 patients contributing to 216 therapeutic lines were analysed: 113 corresponded to colchicine, 83 to GCs, 20 to dapsone. Variables included in the propensity score were age at diagnosis, gender, necrotic lesions, myalgia and number of therapeutic lines. In the univariate analysis, no significant difference but trends were observed in the proportion of patients with SRD90 when comparing GCs vs colchicine (odds ratio [OR] 1.77 95%CI [0.97 – 3.20], p=0.059) and GCs vs dapsone (OR 1.82 95%CI [0.67 – 4.93], p=0.235). No difference was observed between colchicine and dapsone (OR 0.55 95% CI [1.68 – 5.15], p=0.360]). After weighting, GCs vs. colchicine showed balanced covariates (standardized mean differences < 0.10) and the proportion of SRD90 in the GCs group was higher (OR 1.88, 95%CI [1.04 - 3.41], p=0.034). No balance was achieved in comparisons involving dapsone due to low sample size.

Conclusion: GCs were significantly associated with a more favorable outcome than colchicine in achieving sustained remission of cutaneous involvement. The efficacy of dapsone versus colchicine or GCs could not be assessed using propensity scores, but suggests a similar response rate to colchicine and a lower efficacy compared to GCs.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/cutaneous-iga-vasculitis-emulation-of-a-target-trial-from-a-european-multicentric-retrospective-study/