Intravenous immunoglobulin therapy in immunoglobulin A vasculitis with gastrointestinal tract involvement

Objective The aim of this study is to evaluate the outcomes of patients who received intravenous immunoglobulin (IVIG) for immunoglobulin A vasculitis (IgAV) with gastrointestinal (GI) tract involvement, and to determine the differences between the groups that responded to IVIG and those that did not. Methods This retrospective study comprised 152 patients with IgAV between 2018 and 2022. Sixty-five patients (43%) had GI tract involvement. Patients with IgAV-GI involvement who had been treated with IVIG were evaluated. Patients were classified with IgAV according to the 2008 Ankara-EULAR/PRINTO/PRES. Their demographics, presentation, and management are reported. Results Twelve (7 boys/5 girls) of these patients were treated with IVIG. The median age was 90.1 (31-177) months. The mean follow-up period was 30.6 +/- 9.9 months. All patients had skin involvement, joint involvement (arthralgia or arthritis), and abdominal pain. All 12 patients were given steroids (30 mg/kg/day pulse methylprednisolone for 3-7 days, followed by 2 mg/kg/day steroids) before IVIG. Nine patients received cyclophosphamide treatment (four before IVIG and five after IVIG). Complete remission was achieved in 5 of the patients with IVIG. Four patients were diagnosed with IgAV concomitant familial Mediterranean fever, and colchicine treatment was initiated. Conclusions IVIG may be used in steroids and/or immunosuppressive drug resistant IgAV. It can be considered as a treatment option, especially in patients with multi-organ/system involvement, comorbid inflammatory diseases such as familial Mediterranean fever, and in patients with IgAV-GI tract involvement resistant to standard treatment in the advanced pediatric age group.

Automated summary

This study evaluated the outcomes of patients with immunoglobulin A vasculitis (IgAV) involving the gastrointestinal (GI) tract who received intravenous immunoglobulin (IVIG) treatment, and compared the differences between those who responded to IVIG and those who did not. The results showed that IVIG may be considered as a treatment option for IgAV patients who are resistant to steroids and/or immunosuppressive drugs, especially in cases with multi-organ/system involvement, comorbid inflammatory diseases, or resistance to standard treatment in pediatric patients.