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Kidney Week

Abstract: FR-PO071

IgG4-Related Disease as a Cause of Urinary Tract Obstruction and AKI: A Case Report

Session Information

  • AKI Clinical: Predictors
    November 03, 2017 | Location: Hall H, Morial Convention Center
    Abstract Time: 10:00 AM - 10:00 AM

Category: Acute Kidney Injury

  • 003 AKI: Clinical and Translational


  • Andrabi, Suhaib A., Harlem hospital Center , New York , New York, United States
  • Noor, Beenish, Harlem hospital Center , New York , New York, United States
  • Fajardo, Melissa Carmen, Harlem hospital Center , New York , New York, United States
  • Rahaman, Olurntobi, Harlem Hospital Center, New York, New York, United States
  • Jain, Sudhanshu, Harlem hospital Center , New York , New York, United States
  • Wallach, Jeffrey D., Harlem hospital Center , New York , New York, United States

IgG4-related disease (IgG4-RD) is a fibroinflammatory condition with multisystem involvement.We report the case of a male admitted for with chronic abdominal pain who was found to have acute kidney injury , retroperitoneal fibrosis and hydroureteronephrosis.


A 61 year old male presented with a 4-month history of persistent lower abdominal pain , unintentional weight loss of ~15 kg over last year but no urinary complaints.
Medical history significant for Hypertension , cerebrovascular accident and chronic kidney disease. Examination findings included a BP 163/101, lower abdomen tenderness and hyper pigmented skin macules on both shins.
Initial investigations revealed BUN/Creatinine of 51 /7.9 (previous Creatinine 1.6 one year ago), leukocytosis, severe anemia, and thrombocytosis. Urinalysis was bland.
Initial non-contrast Abdominal CT showed bilateral hydroureteronephrosis,perinephric stranding and mid-ureter compression by a soft tissue mass . Also noted was a tissue mass , suspicious for an acute hemorrhage from a aneurysmal infrarenal aorta and retroperitoneal fibrosis. A contrast CT scan ruled out a ruptured aneurysm.

The patient underwent IR-guided nephrostomy tubes/stents with subsequent internalization. There was rapid improvement in renal function and Cr stabilized at ~2 .
The focus then shifted to evaluation of retroperitoneal fibrosis(RPF). Serum IgG4 levels were elevated [258, normal <121]. Flow cytometry showed no evidence oflymphoproliferative disorder or presence of blasts.
Steroid therapy was initated. Biopsy of the retroperitoneal mass revealed extensively hyalinized and fibrotic soft tissue without evidence of fibromatosis or malignancy. Immunohistochemical analyses revealed plasma cells including IgG4 positive plasma cells.
A renal biopsy was not performed due to hydronephrosis .Given patient’s prompt response to treatment, he was discharged to follow up as outpatient.


IgG4-RD is now recognized as a link between many clinical entities that were previously regarded as organ-specific disorders .
Obstructive uropathy occurs in about 45%-65% of reported patients with IgG4-related RPF. Management involves relieving the obstruction, halting progression of the fibrotic process and preventing recurrence.
Obstructive uropathy with RPF should prompt a work up for IgG4-RD.