Renal/Urology · PANCE / PANRE

Post-Streptococcal Glomerulonephritis (PSGN)

Immune complex GN 1-3 weeks after group A strep pharyngitis or impetigo.

Also known as: PSGN, post-streptococcal GN, acute post-infectious GN

Overview

An immune complex-mediated glomerulonephritis occurring 1-3 weeks after infection with nephritogenic strains of group A beta-hemolytic streptococcus (GAS), typically pharyngitis or skin infection (impetigo). Presents with classic nephritic syndrome.

Epidemiology

Most common cause of acute glomerulonephritis in children worldwide. Peak age 4-12 years. Incidence has declined in developed countries with early antibiotic treatment of strep infections. Adults can be affected, with worse prognosis. Outbreaks may follow skin infection epidemics.

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Risk factors

  • Recent group A streptococcal pharyngitis (latency 1-2 weeks)
  • Recent streptococcal skin infection — impetigo, pyoderma (latency 3-6 weeks)
  • Crowded living conditions
  • Pediatric age (school-aged children)
  • Tropical/subtropical climates (impetigo predominant)

Pathophysiology

Nephritogenic strains of GAS (M types 1, 4, 12 in pharyngitis; M types 47, 49, 55 in impetigo) produce antigens (notably streptococcal pyrogenic exotoxin B and nephritis-associated plasmin receptor) that form circulating immune complexes or deposit in situ in subepithelial glomerular sites. Complement activation (alternative pathway predominantly) drives inflammation, mesangial and endothelial proliferation.

Clinical presentation

Symptoms

  • Abrupt onset of tea-colored or cola-colored urine (gross hematuria) 1-3 weeks after pharyngitis or 3-6 weeks after impetigo
  • Periorbital edema, especially in the morning
  • Decreased urine output
  • Headache and malaise from hypertension
  • Children may have nonspecific symptoms initially

Signs / physical exam

  • Hypertension (50-90% of children)
  • Periorbital and dependent edema
  • Tea-colored or smoky urine
  • Mild tenderness over flanks (uncommon)
  • Healed strep infection — pharyngeal exam often normal at presentation

Classic findings

Cola-colored urine + periorbital edema + hypertension in a child 1-3 weeks after sore throat or impetigo = classic PSGN.

Differential diagnosis

  • IgA nephropathy — Synpharyngitic hematuria (within 1-3 days, not 1-3 weeks); normal complement; recurrent episodes
  • Lupus nephritis — Systemic SLE features; ANA, anti-dsDNA; low C3 AND C4
  • Membranoproliferative GN — Persistent low complement; hepatitis C, cryoglobulinemia
  • ANCA vasculitis — Constitutional symptoms, sinopulmonary involvement; positive ANCA; normal complement
  • Henoch-Schönlein purpura (IgA vasculitis) — Palpable purpura, arthralgia, abdominal pain; normal complement
  • Other post-infectious GN — After staph, gram-negative, or viral infections; similar pathology

Diagnostic workup

Diagnostic criteria

Clinical diagnosis: nephritic syndrome + recent strep infection + low C3 + positive streptococcal serology. Biopsy reserved for atypical presentations (persistent low C3 >8 weeks, severe AKI, RPGN features) and shows endocapillary proliferative GN with subepithelial 'humps' on EM and granular IgG/C3 on IF.

Labs

  • Urinalysis: hematuria with dysmorphic RBCs and RBC casts, mild-moderate proteinuria (typically subnephrotic), pyuria
  • BMP: elevated creatinine, sometimes hyperkalemia
  • Low C3 (90%), normal C4 — pathognomonic complement pattern
  • Streptococcal serologies: ASO (positive in 60-80% after pharyngitis but less so after skin infection), anti-DNase B (more sensitive after impetigo), anti-hyaluronidase, anti-streptokinase
  • Throat culture or skin culture (often negative by time of GN presentation)
  • Streptozyme test combines multiple antibodies

Imaging

  • Renal ultrasound usually unnecessary; normal or mildly enlarged kidneys

Diagnostic algorithm

FeaturePSGNIgA Nephropathy
Latency after infection1-3 wk (pharyngitis); 3-6 wk (skin)1-3 days (synpharyngitic)
AgeChildren 4-12 yr (mainly)Young adults
Complement C3Low (resolves in 6-8 wk)Normal
SerologyASO, anti-DNase B positiveElevated IgA (~50%)
CourseSelf-limited (children); residual in adultsChronic, relapsing
Biopsy IFGranular IgG/C3, 'humps' on EMMesangial IgA deposits
Prognosis>95% recovery in children20-40% ESRD over 20 yr
Distinguishing post-streptococcal GN from IgA nephropathy — the two most common pediatric/young adult nephritic presentations.

Treatment

First-line

  • Supportive care — most cases resolve spontaneously
  • Sodium and fluid restriction for volume overload
  • Loop diuretic — furosemide — for edema and hypertension
  • Antihypertensives: CCB (amlodipine) or ACEi (if renal function stable); hydralazine or nicardipine for hypertensive emergency
  • Antibiotics (penicillin V, amoxicillin, or erythromycin if allergic) if active strep infection still present — does NOT alter renal course but limits transmission

Second-line / adjunct

  • Dialysis temporarily if severe AKI with hyperkalemia, uremia, or refractory volume overload (rare)
  • Glucocorticoids only if biopsy shows crescents (atypical RPGN course)
  • Monitor C3 — should normalize within 6-8 weeks; persistent low C3 should prompt alternative diagnosis (MPGN, lupus)
  • Family screening of household contacts for active strep infection
  • Long-term BP and proteinuria monitoring

Complications

  • Hypertensive emergency, posterior reversible encephalopathy syndrome (PRES)
  • Volume overload, pulmonary edema, congestive heart failure
  • Acute kidney injury requiring temporary dialysis (rare)
  • Rapidly progressive crescentic GN (atypical)
  • Adults: 25-50% may have persistent abnormalities (proteinuria, hypertension, CKD)
  • Children: excellent prognosis with >95% complete recovery

PANCE pearls

  • Low C3 + normal C4 is the classic complement signature; should normalize within 6-8 weeks. Persistent low C3 → consider MPGN or lupus.
  • ASO titer rises after pharyngitis but not reliably after skin infection — order anti-DNase B for impetigo-associated cases.
  • Antibiotics for the strep infection do NOT prevent or alter PSGN (unlike rheumatic fever, which IS prevented by antibiotics).
  • Children have excellent prognosis; adults have worse outcomes with 25-50% having residual abnormalities.
  • Hematuria can persist for months; proteinuria may persist longer. Hypertension and edema typically resolve within 1-2 weeks.

References

  • KDIGO 2021 — KDIGO 2021 Clinical Practice Guideline for the Management of Glomerular Diseases
  • Eison 2011 — Post-streptococcal acute glomerulonephritis in children (Eison et al., Pediatr Nephrol 2011)
  • Rodríguez-Iturbe — Pathogenesis of poststreptococcal glomerulonephritis (Rodríguez-Iturbe and Musser, JASN 2008)

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