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Abstract: FR-PO624

Renal Limited PR3-ANCA Vasculitis due to Abiotrophia defectiva Infective Endocarditis

Session Information

Category: Glomerular Diseases

  • 1302 Glomerular Diseases: Immunology and Inflammation

Authors

  • Pozo Garcia, Leonardo, Baylor College of Medicine, Houston, Texas, United States
  • Varughese, Jeryl Philip, Baylor College of Medicine, Houston, Texas, United States
  • Ali, Sehrish, Baylor College of Medicine, Houston, Texas, United States
  • Khairallah, Pascale, Baylor College of Medicine, Houston, Texas, United States
Introduction

Abiotrophia defectiva (A. defectiva) is a rare cause of infective endocarditis (IE) and in some cases, it can lead to glomerulonephritis (GN). There is only one report in the literature associating this organism with ANCA-positive vasculitis with renal involvement. This entity is scarcely described.

Case Description

A 25-year-old gentleman with a history of type 1 diabetes mellitus and tetralogy of Fallot with multiple repairs including surgical pulmonary valve replacement, tricuspid annuloplasty, and two prosthetic implants on the right ventricle outflow tract was admitted to the ICU for decompensated right heart failure and night sweats with plans for surgical repair. Received further workup, inotropes, IV diuretics, and CVP monitoring. Initial imaging was consistent with diffuse lung and heart nodularity. Blood cultures grew gram-positive cocci, later identified as A. defectiva. Antibiotics adjusted. CTA consistent with pulmonary valve and artery vegetations and pulmonary septic emboli. The patient underwent re-replacement of the pulmonary valve, pulmonary artery patch, and tricuspid valve replacement. Valve cultures consistent with A. defectiva. Renal function deteriorated from baseline 0.8 mg/dL to 1.4 mg/dL. Urinalysis had 2+ protein, 3+ blood, and 50-100 RBC/HPF. UPC 3 gm/gm. PR3 ANCA elevated 249 with repeat 323 (NL <19 AU/mL). He was started on pulsed-dosed steroids empirically. Renal biopsy revealed pauci-immune GN with mesangial IgM2+ and C3 staining at the vascular pole. The patient was discharged on ceftriaxone and steroid taper.

Discussion

The patient developed renal limited PR3-ANCA GN due to A. defectiva IE. GN associated with IE is a well-documented entity commonly found in patients with IE caused by staphylococci and streptococci. Only one other case has been previously reported with A. defectiva as the pathogenic organism causing both IE and biopsy-proven PR3-ANCA vasculitis. It is unclear if heavy induction immunosuppression followed by maintenance immunosuppression is needed in these rare cases. Individualized treatment is recommended from our experience.