Clinical and imaging features of IgG4-related kidney disease.

To assess the clinical and imaging features of IgG4-RKD for understanding and diagnosis of this disease. CT and MR images of 34 patients with IgG4-RKD were retrospectively analyzed by two radiologists in consensus. 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. 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.