Article
ANCA-positivity and efficacy of Rituximab in IgG4-related disease
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Authors
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Simon Ronicke
- Department of Nephrology and Hypertension, Hannover Medical School, Hannover
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Jan Hinrich Bräsen
- Institute of Pathology, Hannover Medical School, Hannover
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Marcus Hiß
- Department of Nephrology and Hypertension, Hannover Medical School, Hannover
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Herrmann Haller
- Department of Nephrology and Hypertension, Hannover Medical School, Hannover
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Annette D. Wagner
- Department of Nephrology and Hypertension, Hannover Medical School, Hannover
| Published: | September 9, 2020 |
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Outline
Text
Introduction: IgG4-related disease (IgG4-RD) is an inflammatory multiorgan condition with diverse phenotype [1]. IgG4-RD is characterized by elevated serum IgG4 levels and tissue infiltration by IgG4-positive plasma cells [2]. ANCA-positivity in IgG4-RD patients has been reported [3]. Potential overlap of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) and IgG4-RD has been suspected [4]. Rituximab (RTX) is effective for both induction therapy and maintenance therapy in IgG4-RD [5] and AAV [6].
Methods: Objective of this study was to observe efficacy of RTX in a group of patients with histologically classified IgG4-RD. Patients were tested for ANCAs at diagnosis. We observed parameters of clinical and serological remission (serum IgG4, steroid use) under RTX therapy.
Results: 10 patients with IgG4-RD were included, displaying a heterogenous spectrum of disease phenotypes. ANCAs were found 5/10 patients. Results are summarized in Table 1 [Tab. 1]. We observed good efficacy of RTX leading to steroid reduction to ≤5mg/d in 8/10 patients. The median follow-up period was 3.2 years (0.6 to 6.0 years). Relapses of IgG4-RD requiring increase doses of steroids were observed in two cases after previous steroid reduction or steroid discontinuation respectively.
Conclusion: We observed very good efficacy of RTX in induction and maintenance treatment in a heterogenous group of patients with IgG4-RD regardless of phenotype and ANCA-positivity. Relapses were observed in few cases after steroid reduction and discontinuation.
Disclosures: Potential conflict of interest: SR: employee Ada Health GmbH, JHB, MH, HH, ADW: none declared.
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