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29.08.2023 | Original Article

Clinical phenotypes and prognosis of IgG4-related diseases accompanied by deteriorated kidney function: a retrospective study

verfasst von: Luying Guo, Zhenzhen Yang, Yamei Cheng, Xingxia Wang, Xue Ren, Meifang Wang, Pengpeng Yan, Jia Shen, Ke Sun, Huiping Wang, Jianyong Wu, Jianghua Chen, Rending Wang

Erschienen in: Clinical Rheumatology | Ausgabe 1/2024

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Abstract

Introduction

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.

Method

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.

Results

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.

Conclusions

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.

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Titel: Clinical phenotypes and prognosis of IgG4-related diseases accompanied by deteriorated kidney function: a retrospective study
verfasst von: Luying Guo
Zhenzhen Yang
Yamei Cheng
Xingxia Wang
Xue Ren
Meifang Wang
Pengpeng Yan
Jia Shen
Ke Sun
Huiping Wang
Jianyong Wu
Jianghua Chen
Rending Wang
Publikationsdatum: 29.08.2023
Verlag: Springer International Publishing
Erschienen in: Clinical Rheumatology / Ausgabe 1/2024
Print ISSN: 0770-3198
Elektronische ISSN: 1434-9949
DOI: https://doi.org/10.1007/s10067-023-06748-9

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Abstract

Introduction

Method

Results

Conclusions

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