IgG4-Associated Tubulointerstitial Nephritis: Two Case Reports and a Literature Review

Abstract

IgG4-related systemic disease (IgG4-RSD) is an autoimmune disease that includes a wide variety of lesions. IgG4-RSD is characterized by high levels of serum IgG4, abundant levels of IgG4-positive plasma cells and T-lymphocyte infiltration in various organs. Tubulointerstitial nephritis (TIN) is a major finding when the kidneys are involved and is effectively treated with corticosteroid therapy. We herein describe two cases of IgG4-related TIN. Such cases have rarely been reported in China.

A unique case of IgG4-related skin disease and sclerosing cholangitis in a patient with previous hepatitis exposure

“…Of those, only 81 cases reported having obtained viral serologies for hepatitis, and 14 cases (17%) reported having obtained positive hepatitis serologies, indicating past or current infection ( Table I ). 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 Given that majority of the cases (87%) were not tested for hepatitis, the current literature remains limited and warrants future scientific investigation into the contribution of hepatitis to IgG4-RD.…”

“… 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 Given that majority of the cases (87%) were not tested for hepatitis, the current literature remains limited and warrants future scientific investigation into the contribution of hepatitis to IgG4-RD.…”

Hypergammaglobulinemia in 2259 children with Henoch–Schönlein Purpura

“…He believes that IgG4related tubulointerstitial nephritis and HSP together led to renal injury in that patient [6]. IgG4-related diseases are characterized by hyperglobulinemia and hypocomplementemia [7], IgG4 related diseases should be distinguished from HSP [8].…”

Neck abscess: An unusual clinical presentation of immunoglobulin G4 related disease

“…In some cases, imaging can help differentiate malignant from inflammatory lesions, but histology is needed for a definitive diagnosis. Imaging is more helpful in monitoring response to therapy …”

“…Imaging is more helpful in monitoring response to therapy. 8 Recently, comprehensive diagnostic criteria have been proposed for practical use. All of the following criteria have to be met for a definitive diagnosis of IgG4-RD: the presence of diffuse or localized mass or swelling involving one or more organs, elevated serum IgG4 concentrations more than 135 mg/dL, and tissue infiltration with dense lymphoplasmacytic infiltrate and fibrosis, with IgG4-positive plasma cells more than 10/high-power field (HPF) or with an IgG4/IgG ratio higher than 40% to 50%.…”