Bonelli Grazia Dea, Sciascia Savino, Calatroni Marta, L'imperio Vincenzo, Fenoglio Roberta, Argolini Lorenza Maria, Carrara Camillo, Lepori Nicola, Reggiani Francesco, Bortoluzzi Alessandra, Catapano Fausta, Gatto Mariele, Tani Chiara, Longhitano Elisa, Garozzo Maurizio, Trezzi Barbara, Conte Emanuele, Santoro Domenico, Gerosa Maria, Mosca Marta, Roccatello Dario, Sinico Renato Alberto, Moroni Gabriella
Department of Medicine and Surgery, University of Milano-Bicocca, Monza, Italy.
Unit of Nephrology and Dialysis, ICS Maugeri, 27100, Pavia, Italy.
J Nephrol. 2025 Mar;38(2):643-653. doi: 10.1007/s40620-024-02178-1. Epub 2025 Jan 30.
In an Italian cohort of lupus podocytopathy patients, we aimed to characterize the presenting features, therapy, and outcomes, and explore differences between relapsing and non-relapsing patients.
We identified 29 patients with lupus podocytopathy from 1994 to 2023 in 11 Italian Nephrology/Rheumatology Units, and divided them into two groups: relapsing and non-relapsing. Given the limited sample size, a p-value ≤ 0.2 was considered as significant.
The median age of the patients was 43 (25-52) years, 89.7% were females, 89.6% presented with nephrotic syndrome, 34.4% with acute kidney dysfunction, and 44% with arterial hypertension. After corticosteroids and/or immunosuppressive therapy, complete (25 patients) or partial remission (4 patients) occurred within a median of 4 (1-9) months. Nine patients (31%) relapsed. After a further course of therapy, remission was achieved within 5 (2-11) months. Relapsing patients had higher serum creatinine (0.94 [0.73-2.65] vs 0.8 [0.6-1.1] mg/dl; p = 0.12), lower estimated glomerular filtration rate (76 [32.5-107.5] vs 93 [59.3-109.7] ml/min/1.73m; p = 0.23) and higher proteinuria (7.7 [5.9-11.7] vs 6.5 g/day [3.2-10.1]; p = 0.14) at lupus podocytopathy diagnosis than non-relapsing subjects. Activity indexes at biopsy were higher [(1 (0-2) vs 0 (0-1); p = 0.08] and cutaneous systemic lupus erythematosus manifestations were more prevalent (44.4% vs 10.5%; p = 0.06) in relapsing patients. After an observation of 49 (18-23) months, 86.2% of patients were in complete remission while 13.8% remained in partial remission. One patient developed mild chronic kidney function impairment.
Lupus podocytopathy typically presents with nephrotic syndrome and kidney dysfunction, it responds favourably to treatment, and generally results in a favourable renal outcome. We observed that more active renal and extrarenal lupus manifestations at the onset of lupus podocytopathy were indicative of higher susceptiblity to disease recurrence.
在一组意大利狼疮性足细胞病患者中，我们旨在描述其临床表现、治疗方法和预后，并探讨复发患者和未复发患者之间的差异。
我们在意大利的11个肾脏病/风湿病科单位中，确定了1994年至2023年期间的29例狼疮性足细胞病患者，并将他们分为两组：复发组和未复发组。鉴于样本量有限，p值≤0.2被视为具有统计学意义。
患者的中位年龄为43（25-52）岁，89.7%为女性，89.6%表现为肾病综合征，34.4%伴有急性肾功能不全，44%患有动脉高血压。在接受糖皮质激素和/或免疫抑制治疗后，25例患者完全缓解，4例患者部分缓解，中位缓解时间为4（1-9）个月。9例患者（31%）复发。在接受进一步治疗后，5（2-11）个月内实现缓解。复发患者在狼疮性足细胞病诊断时的血清肌酐水平较高（0.94[0.73-2.65] vs 0.8[0.6-1.1]mg/dl；p = 0.12），估计肾小球滤过率较低（76[32.5-107.5] vs 93[59.3-109.7]ml/min/1.73m；p = 0.23），蛋白尿水平较高（7.7[5.9-11.7] vs 6.5g/天[3.2-10.1]；p = 0.14）。复发患者活检时的活动指数较高[（1（0-2）vs 0（0-1）；p = 0.08]，皮肤系统性红斑狼疮表现更为常见（44.4% vs 10.5%；p = 0.06）。经过49（18-23）个月的观察，86.2%的患者完全缓解，13.8%的患者仍部分缓解。1例患者出现轻度慢性肾功能损害。
狼疮性足细胞病通常表现为肾病综合征和肾功能不全，对治疗反应良好，总体肾脏预后良好。我们观察到，狼疮性足细胞病发病时更活跃的肾脏和肾外狼疮表现提示疾病复发的易感性更高。
