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Intravenous immunoglobulin therapy in immunoglobulin A vasculitis with gastrointestinal tract involvement

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Abstract

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.

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Data availability

Data can be provided upon request.

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Funding

An honorarium, grant, or other form of payment was not given to any author to produce the manuscript. The authors have no relevant financial or non-financial interests to disclose.

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Authors and Affiliations

  1. Division of Pediatric Rheumatology, Department of Pediatrics, Ankara City Hospital, University of Health Sciences, 06800, Bilkent, Cankaya/Ankara, Turkey

    Nimet Öner, Elif Çelikel, Zahide Ekici Tekin, Vildan Güngörer, Tuba Kurt, Pakize Nilüfer Tekgöz, Müge Sezer, Cüneyt Karagöl, Serkan Coşkun, Melike Mehveş Kaplan, Merve Cansu Polat & Banu Çelikel Acar

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  1. Nimet Öner
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  2. Elif Çelikel
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  3. Zahide Ekici Tekin
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Contributions

All authors made substantial contributions to the conception or design of the work, have approved the final manuscript, and takes full responsibility for the manuscript. NO, BCA reviewed and revised the manuscript, ZET, EC, VG and BCA contributed to the writing of the manuscript, TK, NT, MS, CK, SC, MMK and MCP were responsible for data collection and analysis.

Corresponding author

Correspondence to Nimet Öner.

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The authors have no conflict of interest.

Ethical approval

The study was approved by the Ankara City Hospital, ethics committee before the study. (Issue No: 22.12.2021, E2-21-1173). Due to the retrospective design of the study, no patient consent was obtained.

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Öner, N., Çelikel, E., Tekin, Z.E. et al. Intravenous immunoglobulin therapy in immunoglobulin A vasculitis with gastrointestinal tract involvement. Clin Exp Med 23, 1773–1782 (2023). https://doi.org/10.1007/s10238-022-00950-w

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  • DOI: https://doi.org/10.1007/s10238-022-00950-w

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