Iwamura Narumichi, Matsukuma Yuta, Tsutsumi Kanako, Higashi Narumi, Shomura Seiya, Uesugi Noriko, Hamashoji Takafumi, Arita Yui, Deguchi Takashi, Nakano Toshiaki
Department of Nephrology, Japanese Red Cross, Yamaguchi Hospital, Yamaguchi, Japan.
Department of Medicine and Clinical Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
Mod Rheumatol Case Rep. 2025 Jul 25;9(2). doi: 10.1093/mrcr/rxaf009.
Systemic lupus erythematosus (SLE) predominantly involves the kidneys, causing lupus nephritis (LN). Patients with diffuse proliferative LN frequently experience poor outcomes despite advances in immunosuppressive therapies. Low-density lipoprotein apheresis (LDL-A) has been a potential therapeutic option for steroid-resistant nephrotic syndromes (NSs), but its efficacy in LN remains unknown. Here, we report the case of a 26-year-old female patient with SLE and LN classified as IV + V (G) A/C according to the renal pathology society, who developed refractory NS, severe haematuria, and declining renal function. The initial induction therapy, which included hydroxychloroquine, glucocorticoids, mycophenolate mofetil, and belimumab, proved to be ineffective. Consequently, LDL-A significantly improved proteinuria, haematuria, and kidney function. The urinary protein-to-creatinine ratio decreased from 7.15 to 0.61 g/gCr, and haematuria dropped from >100 to 10-19 erythrocytes per high-power field. Additionally, complement levels were improved and anti-double-stranded DNA antibody titres were reduced. Ascribing these improvements solely to LDL-A remains challenging, but the rapid proteinuria and haematuria reduction within 48 h indicates a substantial contribution of LDL-A to the clinical response. The effluent from LDL-A contained not only LDL cholesterol but also measurable amounts of immunoglobulin G and M, which may have contributed to the reduction in LN activity. This case represents the first report of a marked haematuria reduction following LDL-A in LN. LDL-A is a valuable adjunctive treatment in patients with refractory NS or highly active LN unresponsive to standard induction therapies.
系统性红斑狼疮(SLE)主要累及肾脏,导致狼疮性肾炎(LN)。尽管免疫抑制治疗取得了进展,但弥漫性增殖性LN患者的预后往往较差。低密度脂蛋白单采术(LDL-A)一直是治疗类固醇抵抗性肾病综合征(NSs)的一种潜在治疗选择,但其在LN中的疗效尚不清楚。在此,我们报告一例26岁女性SLE和LN患者,根据肾脏病理学会分类为IV + V(G)A/C,该患者出现难治性NS、严重血尿和肾功能下降。包括羟氯喹、糖皮质激素、霉酚酸酯和贝利尤单抗在内的初始诱导治疗被证明无效。因此,LDL-A显著改善了蛋白尿、血尿和肾功能。尿蛋白与肌酐比值从7.15降至0.61 g/gCr,血尿从每高倍视野>100个红细胞降至10 - 19个红细胞。此外,补体水平得到改善,抗双链DNA抗体滴度降低。仅将这些改善归因于LDL-A仍具有挑战性,但48小时内蛋白尿和血尿迅速减少表明LDL-A对临床反应有重大贡献。LDL-A流出液不仅含有LDL胆固醇,还含有可测量量的免疫球蛋白G和M,这可能有助于LN活性的降低。该病例是LN患者接受LDL-A治疗后血尿显著减少的首例报告。LDL-A是难治性NS或对标准诱导治疗无反应的高度活动性LN患者的一种有价值的辅助治疗方法。