Kidney Disease Center, the First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China.
Key Laboratory of Kidney Disease Prevention and Control Technology, Hangzhou, Zhejiang Province, China.
Clin Rheumatol. 2024 Jan;43(1):315-324. doi: 10.1007/s10067-023-06748-9. Epub 2023 Aug 29.
IgG4-related disease (IgG4-RD) is a multiorgan autoimmune disorder that causes irreversible injury. Deteriorated kidney functions are common but easily ignored complications associated with IgG4-RD. Yet the clinical manifestations and prognosis of this specific entity have not been fully illustrated.
Three hundred fifty patients with IgG4-RD were retrospectively enrolled and divided into 119 IgG4-RD with chronic kidney disease (IgG4-RD CKD+) and 231 IgG4-RD without CKD (IgG4-RD CKD-). Demographic clinical and laboratory characteristics and survival of two cohorts were compared using restricted cubic splines, logistic and Cox regression, and Kaplan-Meier analysis. A nomogram was generated for calculating the probability of CKD in IgG4-RD.
The spectrum of organ involvement was different between IgG4-RD CKD+ and CKD- cohorts (p<0.001). Lung (26.89%) and retroperitoneum (18.49%) involvement were more common in the IgG4-RD CKD+ cohort. Increased serum potassium and phosphorus, reduced calcium levels, and hypocomplementemia (all p<0.05) were observed in IgG4-RD CKD+. Restricted cubic splines revealed a U-shaped plot regarding associations between serum potassium and CKD. Kaplan-Meier analysis demonstrated significantly lower long-term survival rates in IgG4-RD patients with kidney function at CKD stages 4-5. Cox regression revealed declined kidney functions (G4 HR 6.537 (95% CI: 1.134-37.675)) associated with increased all-cause mortality in IgG4-RD patients. A nomogram was constructed to predict CKD in IgG4-RD promptly with a discrimination (C-index) of 0.846.
CKD in IgG4-RD was associated with poor outcomes and electrolyte disturbances. Patients with IgG4-RD should be aware of possible deterioration in kidney function. The nomogram proposed would help to identify the subtle possibility of CKD in IgG4-RD. Key points • IgG4-related diseases with deteriorated kidney function have specific clinical and laboratory characteristics. • It is crucial to recognize and address the negative impact of deteriorating kidney function in IgG4-related diseases to prevent further harm. • The nomogram proposed would help to identify subtle kidney involvement by evaluating the possibility of CKD in IgG4-related diseases.
IgG4 相关疾病（IgG4-RD）是一种多器官自身免疫性疾病，可导致不可逆转的损伤。肾功能恶化是 IgG4-RD 常见但容易被忽视的并发症。然而，该疾病的临床表现和预后尚未完全阐明。
回顾性纳入 350 例 IgG4-RD 患者，分为 IgG4-RD 合并慢性肾脏病（IgG4-RD CKD+）119 例和 IgG4-RD 无 CKD（IgG4-RD CKD-）231 例。采用限制性立方样条、Logistic 和 Cox 回归及 Kaplan-Meier 分析比较两组的人口学、临床和实验室特征及生存情况。建立列线图以计算 IgG4-RD 发生 CKD 的概率。
IgG4-RD CKD+和 CKD-两组的器官受累谱不同（p<0.001）。IgG4-RD CKD+组中肺部（26.89%）和腹膜后（18.49%）受累更为常见。IgG4-RD CKD+组中血清钾和磷升高、血钙降低、补体减少（均 p<0.05）。限制性立方样条显示血清钾与 CKD 之间呈 U 型关系。Kaplan-Meier 分析显示 IgG4-RD 患者肾功能处于 CKD 4-5 期时，长期生存率显著降低。Cox 回归显示，IgG4-RD 患者肾功能下降（G4 HR 6.537（95%CI：1.134-37.675））与全因死亡率增加相关。构建了一个列线图，用于快速预测 IgG4-RD 患者的 CKD，其鉴别（C-指数）为 0.846。
IgG4-RD 合并肾功能恶化与不良预后和电解质紊乱有关。IgG4-RD 患者应注意可能出现的肾功能恶化。所提出的列线图有助于识别 IgG4-RD 中 CKD 的潜在可能性。关键点：• 伴有肾功能恶化的 IgG4 相关疾病具有特定的临床和实验室特征。• 认识和处理 IgG4 相关疾病中肾功能恶化的负面影响至关重要，以防止进一步损害。• 所提出的列线图有助于通过评估 IgG4 相关疾病中 CKD 的可能性来识别细微的肾脏受累。
