Streptococcal Infection-related Nephritis (SIRN) Manifesting Membranoproliferative Glomerulonephritis Type I

Abstract

We herein report the case of an 18-year-old boy who developed nephrotic syndrome and hypertension after upper airway inflammation. Post-streptococcal acute glomerulonephritis was diagnosed on the basis of a high antistreptolysin O titer, hypocomplementemia, proteinuria, and microscopic hematuria. A renal biopsy was performed due to persistent proteinuria, and the pathological diagnosis was membranoproliferative glomerulonephritis (MPGN) type I. Glomeruli showed positive staining for nephritis-associated plasmin receptor (NAPlr), a nephritogenic group A streptococcal antigen, and plasmin activity was found in a similar distribution as NAPlr deposition. This rare case of streptococcal infection-related nephritis (SIRN) manifesting MPGN type I supports the histological diversity of SIRN.

Glomerular Deposition of Nephritis-Associated Plasmin Receptor (NAPlr) and Related Plasmin Activity: Key Diagnostic Biomarkers of Bacterial Infection-related Glomerulonephritis

“…The unique glomerular staining patterns of NAPlr and plasmin activity were found not only in patients with PSAGN but also in some patients with other glomerular diseases, such as C3 glomerulopathy [18,19], membranoproliferative glomerulonephritis (MPGN) type I [20,21], antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (both ANCA positive [22] and negative [23]), and IgA vasculitis [24], in which a preceding streptococcal infection is suggested by serological markers, and these cases are referred to as SIRN [2,25]. Although prominent endocapillary proliferation is a common histological feature, the differences in immune responses of the affected hosts may affect the specific histology.…”

Membranoproliferative glomerulonephritis related to a streptococcal infection in a girl with IgA deficiency: a case report

“…We do note, however, one reported case of MPGN with IgAD in an adult, which rapidly progressed to end stage renal failure within 4 months, with the patient ultimately dying [13]. In contrast, a young adult man with SIRN (manifesting as MPGN), but not IgAD, presented with nephrotic syndrome that gradually improved, with complete remission achieved without treatment [16]. In our patient, complete remission was achieved at the early stage after treatment initiation, despite MPGN.…”

Factors Affecting the Progression of Infection-Related Glomerulonephritis to Chronic Kidney Disease

“…Recently, glomerular NAPlr deposition and associated plasmin activity were reported to be observed not only in patients with PSAGN but also in those with other glomerular diseases, in whom preceding streptococcal infection had been suggested [26][27][28][29][30][31]. A-C) Double immunofluorescence (IF) staining for NAPlr (fluorescein isothiocyanate, green) and complement C3 (Alexa Fluor 594, red).…”

“…Recently, glomerular NAPlr deposition and associated plasmin activity were reported to be observed not only in patients with PSAGN but also in those with other glomerular diseases, in whom preceding streptococcal infection had been suggested [26][27][28][29][30][31]. In fact, the preceding infection might be an infection other than GAS, because the GAPDH of various bacteria show cross-immunoreactivity to the anti-NAPlr antibody, and simultaneously show plasmin-binding function [32][33][34].…”