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首页> 外文期刊>asian journal of surgery >Tailored Use of Avacopan in a Case With Refractory Antineutrophil Cytoplasmic Antibody-Associated Renal Vasculitis and Concominant Complement System Activation
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Tailored Use of Avacopan in a Case With Refractory Antineutrophil Cytoplasmic Antibody-Associated Renal Vasculitis and Concominant Complement System Activation

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摘要

To the Editor: We read with great interest the recent study by van Leeuwen et al.1 regarding the compassionate use of avacopan in difficult-to-treat antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Avacopan is a novel promising treatment for antibody-associated vasculitis targeting the complement system by blocking C5a receptors.2,3 Here, we report the tailored use of avacopan in a case with refractory antibody-associated vasculitis and concominant complement system activation. An 84-year-old man with pre-existing hypertension and diabetes presented to our tertiary hospital with lower extremity purpuric papules, acute kidney injury (serum creatinine of 1.69 mg/dl, reference range: 0.5–1 mg/dl; eGFR 40 ml/min per 1.73 m2 , reference: 60 ml/min per 1.73 m2 ), albuminuria (5044 mg/g creatinine, reference: 30 mg/g), and hematuria (Figure 1a–c). A skin biopsy showed dermal vasculitis, and laboratory testing confirmed presence of myeloperoxidase-ANCAs (8.4 IU/ml, reference: 3.5 IU/ml). A kidney biopsy confirmed focal class necrotizing and crescentic ANCAassociated renal vasculitis (Figure 1d). On the basis of these findings, steroid pulse (500 mg i.v. methylprednisone) and rituximab (375 mg/m2 , 750 mg) treatment was initiated for remission induction therapy. After a total number of 3 infusions of rituximab, laboratory testing showed depleted peripheral CD19þ B cells (2/ml, reference range: 100 500/ml) and regression of myeloperoxidase-ANCAs (2.2 IU/ml, reference: 3.5 IU/ml). However, kidney function and albuminuria worsened with requirement kidney replacement therapy (Figure 1a–c). A second kidney biopsy confirmed persistence of active ANCAassociated renal vasculitis (Figure 1e). On the basis of complement system activation with serum C3c lowering (0.62 g/l, reference range: 0.82 1.93 g/l) and progressive intrarenal complement C3 deposits that were not present at first presentation (Figure 1f and g), a tailored treatment with avacopan (30 mg twice daily) was initiated. Thereafter, kidney function and albuminuria improved and kidney replacement therapy was no longer required after 10 days of avacopan treatment (Figure 1a–c). Here, we report real-life practice data on the tailored use of avacopan in a case of refractory antibodyassociated vasculitis. In this case, avacopan had additional effects with respect to improved disease control and in line with previous reports.1 Moreover, we provide the first evidence, to the best of our knowledge, that assessment of complement system activation might enable identification of patients that benefit from a complement-targeted therapy.

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    《asian journal of surgery》 |2023年第2期|376-378|共3页
  • 作者

    Samy Hakroush; Björn Tampe;

  • 作者单位

    Institute of Pathology,University Medical Center Göttingen;

    SYNLAB Pathology Hannover,SYNLAB Holding Germany;

    Department of Nephrology and Rheumatology,University Medical Center Göttingen;

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  • 正文语种 英语
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