A Girl with Immunoglobulin G4-Related Kidney Disease; A case report
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Introduction : Immunoglobulin G4-Related Disease (IgG4-RD) is a rare condition, particularly in the pediatric population, and it is an uncommon cause of Acute Tubulointerstitial Nephritis (ATIN). Case Presentation: A renal biopsy of a girl with sudden onset renal failure revealed ATIN in the absence of any identifiable infections, medications, or obvious autoimmune disorders. Immunohistochemical staining of the renal biopsy showed IgG-positive plasma cells within the interstitium, along with elevated plasma IgG4 levels. These findings led to a diagnosis of immunoglobulin IgG4-RD of the kidney, a rare finding in this age group. The girl responded well to a combination therapy of steroids and Mycophenolate Mofetil (MMF). Conclusion IgG4-RD should be considered in the differential diagnosis of ATIN, especially with interstitial plasma cell infiltration and fibrotic changes in renal biopsy.
