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Clinical and imaging features of IgG4-related kidney disease

  • Kidneys, Ureters, Bladder, Retroperitoneum
  • Published:
Abdominal Radiology Aims and scope Submit manuscript

Abstract

Purpose

To assess the clinical and imaging features of IgG4-RKD for understanding and diagnosis of this disease.

Methods

CT and MR images of 34 patients with IgG4-RKD were retrospectively analyzed by two radiologists in consensus.

Results

The serum IgG4 level was found being increased in all patients. Renal involvement was bilateral (24/34, 70.6%) or unilateral (10/34,29.4%), multiple (29/34, 85.3%) or solitary (5/34, 14.7%). The lesions were wedge-shaped (21) or mass-like (4) in the renal parenchyma, whereas diffusely decreased renal density was noted in 2 patients. All lesions showed progressive contrast enhancement. The 4 mass-like lesions were misdiagnosed as renal malignancy. In 15 patients with follow-up imaging examinations, the number and size of renal lesions decreased after oral hormone treatment. The serum IgG4 levels were significantly decreased after therapy in all patients.

Conclusion

IgG4-RKD has various imaging appearances. Although the mass-like appearance mimics renal malignancy in some patients, progressive contrast enhancement in the lesion with elevated serum IgG4 suggests IgG4-RKD.

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Abbreviations

IgG4:

Immunoglobulin G4

IgG4-RD:

IgG4-related disease

IgG4-RKD:

IgG4-related kidney disease

C3:

Complement 3

C4:

Complement 4

CT:

Computed tomography

MR:

Magnetic resonance

MSCT:

Multi-slice Spiral CT

Hu:

Hounsfield unit

ERCP:

Endoscopic retrograde cholangiopancreatography

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Funding

This work was supported by the National Nature Science Foundation of China under Grant No. 81701747, Natural Science Foundation of Guangdong Province under Grant No. 2017A030313902.

Author information

Author notes

  1. Jian Ling and Huanjun Wang have contributed equally to this work, and they are co-first authors.

Authors and Affiliations

  1. Department of Radiology, The Eastern Hospital of the First Affiliated Hospital, Sun Yat-Sen University, No.183 Huangpu Eastern Road, Guangzhou, 510700, Guangdong, People’s Republic of China

    Jian Ling

  2. Department of Radiology, The First Affiliated Hospital of Sun Yat-Sen University, No.58 Zhongshan Road 2, Guangzhou, 510080, Guangdong, People’s Republic of China

    Huanjun Wang, Weibin Pan, Shurong Li, Jian Guan, Xiaoling Zhang & Yan Guo

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  1. Jian Ling
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  2. Huanjun Wang
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Correspondence to Yan Guo.

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Ling, J., Wang, H., Pan, W. et al. Clinical and imaging features of IgG4-related kidney disease. Abdom Radiol 45, 1915–1921 (2020). https://doi.org/10.1007/s00261-020-02477-8

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  • DOI: https://doi.org/10.1007/s00261-020-02477-8

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