Imaging findings of IgG4-related kidney disease without extrarenal organ involvement: A case report

IgG4-related disease (IgG4-RD) is a systemic chronic inflammatory disorder that can affect almost every organ. IgG4-RD includes IgG4-related kidney disease (IgG4-RKD), but lesions affecting the kidney alone or first are very rare, and a complete understanding is lacking. Computed tomography (CT) and magnetic resonance imaging (MRI) findings can show the typical characteristics of IgG4-RKD and provide information for accurate and rapid diagnosis.

We report a case of a 60-year-old woman who was admitted to our hospital for dizziness and instability while walking, her bilateral eyelids were also slightly swollen. She had no medical history.

CT and MRI images of the patient revealed multiple local and diffuse patchy lesions in the bilateral renal parenchyma and mass-like tissue in the bilateral renal pelvis, accompanied by right hydronephrosis. A pathological examination of renal samples showed numerous lymphocyte and plasma cell infiltration. Immunohistochemistry demonstrated approximately 50% of the IgG-positive plasma cells to be IgG4+. The serum IgG level was obviously elevated, with both C3and C4 levels were reduced. The patient was diagnosed with IgG4-RKD.

The patient received corticosteroid therapy at another hospital.

The bilateral kidney lesions were smaller on follow-up CT images.

IgG4-RKD exhibits some characteristic imaging features. Despite the relatively low incidence of IgG4-RKD, it should be included in differential diagnoses when images show multiple lesions in kidneys with mild and delayed enhancement and hypointensity on T2WI in middle-aged to elderly patients.