Introduction:

IgG4-related Kidney desease (IgG4-RKD) has a prevalence of 8 to 24%, more frequent in males (73-87%), with a mean age of 65 years.

Objectives:

to determinate the kidney involvement finding and their frequency in patients with IgG4-related disease (IgG4-RD). To describe and compare demographic, clinical characteristics, laboratory data and treatment among patients with IgG4-RD who have kidney involvement versus patients without kidney involvement.

Materials and methods:

we conducted a retrospective observational study of patients with IgG4-related kidney (IgG4-RKD) in a IgG4-RD cohort. Data were collected through review of medical records. We describe clinical parameters at baseline, histological and radiological findings and treatment.

Results:

of 44 patients with IgG4-RD, 6 (13.6%) had IgG4-RKD. Patients were aged 50,8±17,1 years, and 4 were female. All of them had involvement the other organs systems. The most common radiological finding was low-density lesions on enhanced CT. Three patients had a renal biopsy, the main histopathological diagnosis being IgG4 tubulointerstitial nephritis. All patients received glucocorticoids and immunosupresor as initial management. Patients with kidney disease, as opposed to patients without kidney involvement had a higher number of involved organs, and IgG2, IgG3 serum levels and lower C3 level.

Conclusions:

in general, the characteristics of patients with ER-IgG4 and kidney involvement were similar to published works.

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