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Kidney Week

Abstract: TH-PO132

An Unusual Complication of IgG4-Related Interstitial Nephritis as Resistant Hypertension

Session Information

  • AKI: Mechanisms - I
    November 03, 2022 | Location: Exhibit Hall, Orange County Convention Center‚ West Building
    Abstract Time: 10:00 AM - 12:00 PM

Category: Acute Kidney Injury

  • 103 AKI: Mechanisms

Authors

  • Wang, Hubert, Stony Brook University Renaissance School of Medicine, Stony Brook, New York, United States
  • Revelo Penafiel, Monica Patricia, University of Utah Health, Salt Lake City, Utah, United States
  • Khan, Sobia N., Stony Brook University Renaissance School of Medicine, Stony Brook, New York, United States
Introduction

IgG4-related disease is immune-mediated, can affect multiple organ systems, and is characterized by tissue biopsy with dense lymphoplasmacytic infiltrate, high numbers of IgG4-positive plasma cells, and varying degrees of tissue fibrosis in a storiform pattern. We present a unique case of resistant hypertension (HTN) as a prominent manifestation in IgG4-related interstitial nephritis.

Case Description

A 64-year-old male with history of HTN and livedoid vasculopathy presented for acute kidney injury with no prior kidney disease. He finished 10 days of Bactrim DS 2 weeks before admission. Home medications included bisoprolol-HCTZ 5mg-6.25mg daily. BP 187/94. Labs: Na 129 mmol/L, K 3.2 mmol/L, BUN 41 mg/dL, SCr 3.2 mg/dL. Urinalysis showed + protein. ANA positive, C3/C4 normal, and SPEP/UPEP with Kappa chain elevation and M-spike. Immunofixation showed IgG Kappa monoclonal antibody. Bone marrow biopsy showed 10-15% CD138 positive plasma cells, diagnosed with smoldering multiple myeloma. Renal ultrasound unremarkable. Renal biopsy consistent with acute interstitial nephritis and mild interstitial fibrosis and tubular atrophy. He was treated with prednisone. HTN controlled on amlodipine, labetalol, and hydralazine. Electrolyte abnormalities prevented diuretic use.
He developed AKI on CKD 8 months later. Repeat renal biopsy showed mixed interstitial inflammatory infiltrate with IgG4-positive plasma cells and obliterative arteriopathy consistent with IgG4-mediated interstitial nephritis. He was treated with prednisone taper and rituximab. He presented for hypertensive emergency 2 months later, treated with nicardipine infusion, oral minoxidil added, but complicated by bilateral pleural effusions. Secondary HTN workup negative. Final HTN regimen: clonidine patch 0.6 mg weekly, oral clonidine 0.2 mg in AM and 0.3 mg at noon and PM, hydralazine 100 mg TID, imdur 30 mg BID, labetalol 800 mg TID, nifedipine 60 mg BID, and aldactone 12.5 mg daily.

Discussion

The prominent characteristic of our patient’s IgG4-related interstitial nephritis was resistant hypertension. He had negative renal artery duplex, unremarkable abdominal MRI, and plasma renin activity and serum aldosterone concentration inadequate to conclude on hyperaldosteronism. No prior case report describes resistant HTN as a prominent manifestation in IgG4-related interstitial nephritis.