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Abstract: TH-PO557

Tubulointerstitial Nephritis as the Initial Presentation of IgG4-Related Disease

Session Information

  • Trainee Case Reports - I
    October 25, 2018 | Location: Exhibit Hall, San Diego Convention Center
    Abstract Time: 10:00 AM - 12:00 PM

Category: Trainee Case Reports

  • 1202 Glomerular Diseases: Immunology and Inflammation

Authors

  • Togonu-Bickersteth, Babajide, Lehigh Valley Health Network, Easton, Pennsylvania, United States
  • Kopyt, Nelson P., Lehigh Valley Hospital, Bethlehem, Pennsylvania, United States
Introduction

IgG4 related disease (IgG4-RD) is a systemic, autoimune fibrosing condition that was recently recognized, initially as autoimmune pancreatitis, less than 15 years ago. Since then, it has been shown to involve other organs, manifesting as cholangitis, retroperitoneal fibrosis, aortitis, pneumonitis and nephritis. While membraonus glomerulonephritis has been described, tubulointerstitial nephritis (TIN) is the most common for m of IgG-4 related kidney disease (IgG4-RKD). It is marked by IgG4 positive lymphoplasmocytic infiltrate.

Case Description

A 40 y/o caucasian male with a history of Asthma and Graves disease presented for evaluation of elevated creatinine, which was 1 mg/dl at baseline and on evaluation was 1.99 mg/dL. His creatinine increased over the next 6 months to a peak of 3.5. Urinalysis showed mild proteinuria and a bland sediment. He was referred for renal biopsy which showed significant plasma cell infiltrate with more than 50 IgG4 positive cells per HPF, as well as lymphocytic tubulitis. Intrestingly, eosinophils were seen on the biopsy. The clinical significance of this is as yet unclear. He was shown to have leveated total IgG as well as elevated IgG4. He subsequently developed respiratory symtoms and CT imging of his chest showed a nodule, with surrounding ground glass changes. Steroid therapy was initiated and he has had resolutionof the chest findings and improvement in creatinine.

Discussion

This case adds to our body of knowledge and supports proposed diagnostic criteria which combines histology, imaging and serology. It also supports the notion that renal involvement may precede systemic findings. Diagnosis of this condition warrants awareness and vigilance as specific immune staining is required and may need to be requested to make the diagnosis.