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Introduction <p>C3 glomerulopathy (C3G) is an ultra-rare, complex, under-recognized kidney disease with a challenging diagnosis and no approved treatment. Nephrologists were surveyed to understand the treatment and management of C3G. This study presents the diagnostic challenges and treatment patterns of Japanese patients with C3G.</p> Methods <p>Data from the Adelphi C3G Disease Specific Programme™, a multinational survey of nephrologists treating patients with C3G in 8 countries including Japan, were retrospectively analyzed. Nephrologists completed patient-record forms on patient demographics, diagnosis, clinical characteristics, and treatment approaches.</p> Results <p>Sixteen nephrologists from Japan responded to the survey for 36 patients with C3G. Mean age at diagnosis and at the time of the survey was 45.4 and 48.6&#xa0;years, respectively. Common symptoms at diagnosis were proteinuria (100%) and hematuria (83%); 79% of patients had proteinuria of &#xa0;≥1&#xa0;g/day, and 3% had an estimated glomerular filtration rate of &lt;30&#xa0;mL/min/1.73&#xa0;m<sup>2</sup>. Median time from initial examination by general practitioner to definitive diagnosis was 8.4&#xa0;weeks; ~20% and 10% of patients had to wait for &gt;4 and &gt;8&#xa0;months, respectively, to get a confirmed C3G diagnosis; and 69% of patients had an additional biopsy. Angiotensin receptor blockers (68%), corticosteroids (64%), and sodium-glucose cotransporter-2 inhibitors (25%) were the main treatments utilized. Physicians perceived 19% of patients to have a gradually deteriorating disease condition.</p> Conclusion <p>This survey analysis explored the current status in diagnosis and management of patients with C3G in Japan. The lack of specific treatments emphasizes the need for novel targeted therapies addressing the root cause of C3G.</p>

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Diagnosis and treatment in adult patients with C3 glomerulopathy in Japan: a real-world survey

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年11月3日

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  • Naoki Nakagawa,
  • Yutaro Kotobuki,
  • Michel Kroes,
  • Shunsuke Eguchi,
  • Toshinaga Tsuji,
  • Alice Simons,
  • Susanna Libby,
  • Raisa Sidhu,
  • Serge Smeets,
  • Kazuma Iekushi

摘要

Introduction

C3 glomerulopathy (C3G) is an ultra-rare, complex, under-recognized kidney disease with a challenging diagnosis and no approved treatment. Nephrologists were surveyed to understand the treatment and management of C3G. This study presents the diagnostic challenges and treatment patterns of Japanese patients with C3G.

Methods

Data from the Adelphi C3G Disease Specific Programme™, a multinational survey of nephrologists treating patients with C3G in 8 countries including Japan, were retrospectively analyzed. Nephrologists completed patient-record forms on patient demographics, diagnosis, clinical characteristics, and treatment approaches.

Results

Sixteen nephrologists from Japan responded to the survey for 36 patients with C3G. Mean age at diagnosis and at the time of the survey was 45.4 and 48.6 years, respectively. Common symptoms at diagnosis were proteinuria (100%) and hematuria (83%); 79% of patients had proteinuria of  ≥1 g/day, and 3% had an estimated glomerular filtration rate of <30 mL/min/1.73 m2. Median time from initial examination by general practitioner to definitive diagnosis was 8.4 weeks; ~20% and 10% of patients had to wait for >4 and >8 months, respectively, to get a confirmed C3G diagnosis; and 69% of patients had an additional biopsy. Angiotensin receptor blockers (68%), corticosteroids (64%), and sodium-glucose cotransporter-2 inhibitors (25%) were the main treatments utilized. Physicians perceived 19% of patients to have a gradually deteriorating disease condition.

Conclusion

This survey analysis explored the current status in diagnosis and management of patients with C3G in Japan. The lack of specific treatments emphasizes the need for novel targeted therapies addressing the root cause of C3G.