Infection-Related Cryoglobulinemic Glomerulonephritis with Serum Anti-Factor B Antibodies Identified and Staining for NAPlr/Plasmin Activity Due to Infective Endocarditis

Abstract

In this rare case of infection-related cryoglobulinemic glomerulonephritis with infective endocarditis, a 78-year-old male presented with an acute onset of fever and rapidly progressive glomerulonephritis. His blood culture results were positive for Cutibacterium modestum, and transesophageal echocardiography showed vegetation. He was diagnosed with endocarditis. His serum immunoglobulin M, IgM-cryoglobulin, and proteinase-3-anti-neutrophil cytoplasmic antibody levels were elevated, and his serum complement 3 (C3) and C4 levels were decreased. Renal biopsy results showed endocapillary proliferation, mesangial cell proliferation, and no necrotizing lesions on light microscopy, with strong positive staining for IgM, C3, and C1q in the capillary wall. Electron microscopy showed deposits in the mesangial area in the form of fibrous structures without any humps. Histological examination confirmed a diagnosis of cryoglobulinemic glomerulonephritis. Further examination showed the presence of serum anti-factor B antibodies and positive staining for nephritis-associated plasmin receptor and plasmin activity in the glomeruli, suggesting infective endocarditis-induced cryoglobulinemic glomerulonephritis.

Keywords: cryoglobulinemic glomerulonephritis; factor B; infection-related glomerulonephritis; infective endocarditis; nephritis-associated plasmin receptor (NAPlr).

Figure 1

Kidney biopsy specimen. Light microscopy shows slight endocapillary proliferation with neutrophils and lymphocytes (A). Immunofluorescence staining showed that IgM (B), C3 (E), and C1q (F) were strongly positive in the ciliary loop. Immunoglobulin G (IgG), (C), and IgA (D) tests were negative. Electron microscopy showed high electron density deposits in the mesangial area and subendothelial (G). In a high-power field, the deposits exhibited a fibrous structure (H).

Figure 2

Additional tests of a kidney biopsy specimen. Both NAPlr (A) and plasmin activity (B) were segmentally and weakly positive in glomeruli of sequential sections of fresh frozen tissue. The distribution of the positive portion for those staining was similar (A,B).