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Kidney Week

Abstract: PO0123

IgG4-Related Disease with Renal Involvement in a Patient with HIV Infection

Session Information

Category: Trainee Case Report

  • 102 AKI: Clinical, Outcomes, and Trials

Authors

  • Flores, Karen, The Ohio State University, Columbus, Ohio, United States
  • Parikh, Samir V., The Ohio State University, Columbus, Ohio, United States
  • Shidham, Ganesh B., The Ohio State University, Columbus, Ohio, United States
Introduction

Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory disease that can affect multiple organs. It is characterized by lymphadenopathy and diffuse enlargement of one or multiple organs. Pathologic features include tissue infiltration by IgG4-positive plasma cells, storiform fibrosis, and increased tissue eosinophils. Tubulointerstitial nephritis is the most common renal manifestation. We describe a case of IgG4-RD in a patient with co-existing HIV infection.

Case Description

A 65-year old male with CKD 3, hypertension, hepatitis B, and HIV was hospitalized due to malaise and acute kidney injury with serum creatinine of 4.2 mg/dL and 1.5 g/d proteinuria.. HIV was controlled with undetectable viral load on treatment. Infectious work up was negative. CT scan revealed diffuse retroperitoneal lymphadenopathy. PET scan showed diffuse lymphadenopathy and increased uptake in bilateral kidneys and parotid glands. Right inguinal lymph node biopsy showed atypical interfollicular T-cell infiltrate and dense polytypic plasmacytic infiltrate with a kappa/lambda ratio of 3:1 by ISH. TCR gene rearrangement and IGH studies were negative for a monoclonal population. Testing for HHV8 and EBV was negative. SPEP showed a small IgG lambda clone (0.3 g/dL). UPEP showed a small amount of lambda free light chains. Bone marrow biopsy showed no evidence of T cell lymphoma but revealed hypocellular marrow. Total IgG (5048 mg/dL) and IgG4 (572 mg/dL) were elevated and both C3 and C4 were low. Kidney biopsy revealed dense interstitial infiltration of plasma cells strongly positive for IgG4 (>50/hpf) and no electron dense deposits. The patient was diagnosed with IgG4-RD with renal involvement. He was treated with high dose prednisone and mycophenolate mofetil. Renal function improved from 4.6 mg/dL to a 1.9mg/dL after 3 months of treatment. IgG4 levels improved 70mg/dL and C3/C4 normalized suggesting disease control had been achieved.

Discussion

We describe a case of IgG4-RD with renal involvement in a patient with HIV infection. There are only a few reports in the literature. Differential diagnosis of kidney disease during HIV infection is broad. IgG4-RD is a severe systemic disorder that can be mistaken for infection or malignancy. IgG4-RD should be considered in the differential for acute kidney injury in the setting of HIV.