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Abstract: FR-PO217

Renal Limited IgG4-Related Disease: A Unique Clinical Entity

Session Information

Category: Acute Kidney Injury

  • 103 AKI: Mechanisms


  • Zghayer, Aseel, Loyola University Health System, Maywood, Illinois, United States
  • Schneider, Julia, Loyola University Health System, Maywood, Illinois, United States

IgG4 related disease (IgG4-RD) is an immune mediated fibroinflammatory conditions that can affect many organs including kidneys, where involved organs share core pathologic, clinical and serological similarities. IgG4-related kidney disease (IgG4-RKD) is a part of spectrum of IgG4-RD and more than 90% of patients with IgG4-RKD have manifestations of extrarenal involvement. Here, we describe a rare case where kidney is the sole organ involved in IgG4-RD.

Case Description

A 74-year-old man with history of long standing hypertension was admitted after a routine blood test showed acute kidney injury, serum creatinine 5.4 mg/dl, was 1.3 mg/dl two years prior. Work up is shown in Table 1. BP was uncontrolled on admission and patient was asymptomatic. CT abdomen/pelvis did not show any abnormalities. Kidney biopsy revealed diffuse interstitial fibrosis and tubular atrophy involving 90% of the cortical parenchyma, and immuno-labeling showed diffuse IgG4-positive plasma cells in the interstitium with 17-31 cells/HPF in most concentrated areas (Image 1).


Tubulointerstitial nephritis (IgG4-TIN) is the most common renal manifestation of IgG4-RKD, it may present as acute or chronic renal insufficiency. Diagnosis of IgG-4 TIN is challenging without the presence of other extra-renal manifestations of IgG4-RD. IgG4 staining should be considered in TIN with presence of plasma cell infiltrates. To the best of our knowledge, our case is first to be reported in literature for isolated IgG4-TIN. Greater clinical awareness of this entity will lead to early diagnosis and early intervention.

Table 1.

Image 1.