Abstract: FR-PO951
Not All Proteinuria in Pregnancy Is Preeclampsia
Session Information
- Glomerular Diseases: Potpourri
October 25, 2024 | Location: Exhibit Hall, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Naeem, Iqra, Marshfield Clinic Health System, Marshfield, Wisconsin, United States
- Basharat, Ahmad, Marshfield Clinic Health System, Marshfield, Wisconsin, United States
- Blonsky, Rebecca, Marshfield Clinic Health System, Marshfield, Wisconsin, United States
- Zhong, Weixiong, University of Wisconsin-Madison, Madison, Wisconsin, United States
Group or Team Name
- Marshfield Clinic and Health System, Dept of Internal Medicine and Dept of Nephrology; University of Wisconsin Madison, Dept of Pathology.
Introduction
Atypical hemolytic uremic syndrome (aHUS) is a rare disease caused by dysregulation of the alternative complement cascade leading to uncontrolled terminal complement activation causing a systemic thrombotic microangiopathy (TMA). Nephrotic syndrome is a rare presentation of aHUS. We present a case of a pregnant patient presenting with nephrotic syndrome as the sole manifestation of renal limited aHUS.
Case Description
27-year-old female with history inclusive of prematurity presented at 19 weeks gestation with new onset severe hypertension and anasarca. Patient was found to have 4.268 g/24hr of proteinuria. She was started on steroids while serologic workup for nephrotic syndrome was completed. Her hypertension was treated with diuretics and calcium channel blocker. The patient did not respond to steroids and serologic work up was non-revealing including CBC and LFTs. Patient had pregnancy complication with severe fetal growth restriction and near reversal of placental flows. Kidney biopsy showed findings consistent with acute on chronic TMA as shown in figure. Serologic workup for aHUS demonstrated elevated levels of complement factor Bb. Other potential causes for TMA were negative as was genetic testing. Unfortunately, she lost her pregnancy at 21 weeks gestation, and we changed her antihypertensives to ACE-I. The proteinuria peaked at 9.87 g/g and following addition of ACE-I improved to 1.333 g/g. Eculizumab was the started following appropriate vaccination with remission allowing for decreasing doses of ACE-I.
Discussion
Pregnancy-triggered aHUS can occur during pregnancy and up to 60 days postpartum and accounts for 7% of all aHUS cases. Typically, aHUS present as a systemic TMA with extrarenal manifestations. Isolated nephrotic syndrome is rare, particularly in adults, making this case unique. Eculizumab remains the mainstay of treatment with better outcomes when initiated early in the disease course.