Clin Nephrol. 2023 Jan;99(1):41-48. doi: 10.5414/CN110892.
Overlap syndrome is the combination of autoimmune liver diseases, and this term usually describes the coexistence of autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC) in the same patient. Membranous nephropathy (MN) is the most common pattern of idiopathic nephrotic syndrome in patients without diabetes. The coexistence of PBC-AIH overlap syndrome and MN is very rare. Herein, the patient we describe exhibited large amounts of proteinuria and hepatic dysfunction nearly at the same time. We administered azathioprine to our patient. Fortunately, the patient demonstrated a good response to azathioprine, including a partial reduction in proteinuria from ~ 12.5 g/D to 2.62 g/D after 21 months of observation and the improvement of liver function. Our findings suggest that azathioprine may be a suitable treatment option for patients presenting with coexisting PBC-AIH overlap syndrome and MN.
重叠综合征是自身免疫性肝病的组合，这一术语通常描述的是同一患者中自身免疫性肝炎（AIH）和原发性胆汁性肝硬化（PBC）的共存。膜性肾病（MN）是无糖尿病患者特发性肾病综合征中最常见的类型。PBC-AIH 重叠综合征和 MN 的共存非常罕见。在此，我们描述的患者表现出大量蛋白尿和肝功能障碍几乎同时发生。我们给患者使用了硫唑嘌呤。幸运的是，患者对硫唑嘌呤有良好的反应，包括蛋白尿从约 12.5 g/D 减少到 2.62 g/D，观察 21 个月后肝功能改善。我们的发现表明，硫唑嘌呤可能是同时患有 PBC-AIH 重叠综合征和 MN 的患者的一种合适的治疗选择。
