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Kidney Week

Abstract: TH-PO682

Infection-Related ANCA-Negative Pauci-Immune Glomerulonephritis

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Sohail, Mohammad Ahsan, Cleveland Clinic Glickman Urological and Kidney Institute, Cleveland, Ohio, United States
  • Taliercio, Jonathan J., Cleveland Clinic Glickman Urological and Kidney Institute, Cleveland, Ohio, United States
  • Tomaszewski, Kristen, Cleveland Clinic Glickman Urological and Kidney Institute, Cleveland, Ohio, United States
  • Mehdi, Ali, Cleveland Clinic Glickman Urological and Kidney Institute, Cleveland, Ohio, United States
Introduction

Infection-related glomerulonephritis (IRGN) is typically an immune-complex mediated disease characterized by a diffuse proliferative process with mesangial, subendothelial and subepithelial deposits comprised of various combinations of IgM, IgG, IgA and C3. However, other forms of IRGN have also been reported, including those with a pauci-immune crescentic pattern of injury. The overwhelming majority of pauci-immune GN cases are associated with the presence of anti-neutrophil cytoplasmic antibodies (ANCA). However, approximately 2-10% of these patients may be ANCA negative, and here, we present two cases of IRGN, which were ANCA-negative, and demonstrated a pauci-immune crescentic pattern of injury.

Case Description

Table 1 describes the clinical characteristics, histologic features and outcomes for two patients who initially presented with acute kidney injury, were subsequently diagnosed with ANCA-negative pauci-immune crescentic GN on kidney biopsy and after further evaluation for secondary causes of pauci-immune GN, were found to have bacterial aortic/mitral valve endocarditis.

Discussion

This case series depicts two patients who developed ANCA-negative pauci-immune GN in association with bacterial endocarditis. The largest case series of 74 patients with ANCA-negative pauci-immune GN included 9 cases that were infection-related. 54% of these patients had extra-renal involvement and 23% of them required dialysis at diagnosis. The diverse glomerular presentations in association with infections have significant clinical implications since the prompt recognition of an underlying systemic infection is crucial to avoid inadvertent immunosuppressive therapy. It is imperative for clinicians to screen for occult infections not only when an immune-complex GN is seen, but also when a pauci-immune process is identified on a kidney biopsy.

Table 1
Clinical CharacteristicsCase 1Case 2
Age (Years) / Sex69 / Female59 / Female
Clinical ManifestationsExertional Dyspnea and Lower Extremity EdemaProgressive Weight Gain, Exertional Dyspnea and Lower Extremity Non-Blanching Petechial Rash
Serum Creatinine on Initial Presentation
(Baseline Creatinine) (mg/dL)
4.1 (1.0)3.0 (0.6)
Urinalysis

Urine Protein/Creatinine Ratio (mg/mg)
Microscopic Hematuria (>25 RBCs/HPF) (Dysmorphic RBCs)

0.45
Microscopic Hematuria: (>25 RBCs/HPF) (Dysmorphic RBCs)

0.95
Available Serologic TestingANA Negative
ANCA Negative
Low Serum C3 (71 mg/dL)
Low Serum C4 (7 mg/dL)
Polyclonal IgG Type 3 Cryoglobulinemia
IgM Lambda M-Protein
ANA Negative
ANCA Negative
Low C3 (79 mg/dL)
Normal C4 (24 mg/dL)
Blood Cultures

Echocardiogram Findings
Streptococcus Mutans

Mitral/Aortic Valve Vegetations
Streptococcus Mitis

Mitral Valve Regurgitation
Mitral/Aortic Valve Vegetations
Histologic Features on
Kidney Biopsy
Pauci-Immune GN
Endocapillary Hypercellularity
Cellular/Fibrous Crescents with Necrosis
Moderate IFTA
Pauci-Immune GN with Trace IgA, C3 and C1q
Endocapillary Hypercellularity
Cellular Crescents
Severe IFTA
Clinical OutcomesS/P Mitral and Aortic Valve Replacement
Initiated KRT at the time of kidney biopsy
Repeat kidney biopsy 1 month later showed focal global glomerulosclerosis without ongoing proliferative activity
Last Follow-Up: remains KRT-dependent 4 months following valvular surgery
S/P Mitral Valve Replacement and Aortic Valve Repair
Initiated KRT immediately after valvular surgery
Subsequent kidney recovery with cessation of KRT one week after initiation
Last Follow-Up: remains liberated from KRT with serum creatinine 1.23 mg/dL 3 months following valvular surgery

Interstitial Fibrosis and Tubular Atrophy (IFTA); Kidney Replacement Therapy (KRT)