Glomerular DiseasesMay 4, 20265 min read

Everything You Need to Know About Nephritic syndrome (IgA, post-strep GN) for Step 1

Deep dive: definition, pathophysiology, clinical presentation, diagnosis, treatment, HY associations for Nephritic syndrome (IgA, post-strep GN). Include First Aid cross-references.

Nephritic syndrome is one of those Step 1 “pattern-recognition” topics that pays off fast: a patient with dark (cola-colored) urine, hypertension, and RBC casts should immediately push you toward an inflammatory glomerular process. The two highest-yield etiologies you’ll see early (and test often) are IgA nephropathy and post-streptococcal glomerulonephritis (PSGN)—and the key is learning how to separate them by timing, serologies, and histology.


Nephritic Syndrome: The Core Definition (Know This Cold)

Nephritic syndrome = inflammation of the glomerulus leading to:

  • Hematuria (often gross, “tea/cola-colored”)
  • RBC casts (bleeding that occurred within the nephron)
  • Mild-to-moderate proteinuria (typically < 3.5 g/day)
  • Azotemia (↓ GFR → ↑ BUN/Cr)
  • Oliguria
  • Hypertension (salt/water retention + RAAS)
  • Periorbital edema (can occur, but classically more dramatic in nephrotic syndromes)

Why RBC casts matter (Step 1 logic)

RBCs + Tamm-Horsfall protein in tubules → RBC casts → strongly suggests glomerular bleeding, not lower-tract bleeding.

First Aid cross-reference: Renal → Glomerular diseases; Nephritic vs nephrotic syndromes tables; Poststreptococcal GN and IgA nephropathy.


Pathophysiology: What “Nephritic” Really Means

Nephritic syndromes are driven by immune-mediated injuryinflammation → damage to the glomerular filtration barrier.

Key mechanisms

  • Immune complex deposition (classic for IgA nephropathy, PSGN)
  • Complement activation (often low C3 in PSGN)
  • Leukocyte recruitment + proliferation of glomerular cells
  • Capillary wall damage → RBC leak → hematuria + casts
  • Reduced filtration surface area → ↓ GFR → azotemia, HTN, oliguria

Big Picture: IgA Nephropathy vs PSGN (Quick Compare Table)

FeatureIgA Nephropathy (Berger)Post-strep GN (PSGN)
Typical triggerURI/GI infectionStrep throat or impetigo
Timing after infectionDays (often synpharyngitic)Weeks (classically 1–3 weeks after pharyngitis; 3–6 weeks after skin infection)
ComplementUsually normalLow C3 (hypocomplementemia)
SerologiesNo classic single marker (IgA may be ↑)↑ ASO, ↑ anti-DNase B
LMMesangial proliferationHypercellular glomeruli (diffuse)
IFMesangial IgA depositionLumpy-bumpy” granular deposits (IgG, IgM, C3)
EMMesangial electron-dense depositsSubepithelial humps
Clinical pearlOften recurrent episodes with URIsFirst episode after strep; kids common
PrognosisVariable; can progress to CKDOften resolves in children; worse in adults

IgA Nephropathy (Berger Disease): High-Yield Deep Dive

What it is

An immune complex–mediated nephropathy characterized by IgA deposition in the mesangium, often after mucosal infections.

Pathophysiology (board-friendly version)

  • Mucosal immune activation → production of abnormally glycosylated IgA
  • Formation of IgA-containing immune complexes
  • Mesangial deposition → complement activation (often via alternative/lectin pathways) → inflammation

Clinical presentation

  • Episodic hematuria (gross or microscopic)
  • Occurs within days of an URI (“synpharyngitic”)
  • May have:
    • Mild proteinuria
    • Hypertension
    • Elevated creatinine if more severe

HY association: HSP (IgA vasculitis)

IgA nephropathy is closely related to Henoch–Schönlein purpura (IgA vasculitis):

  • Palpable purpura (buttocks/legs)
  • Arthralgias
  • Abdominal pain
  • Renal involvement identical pattern (IgA deposition)

First Aid cross-reference: Immunology/Rheumatology → Henoch-Schönlein purpura; Renal → IgA nephropathy.

Diagnosis

Often suspected clinically, confirmed by biopsy if needed (atypical course, significant proteinuria, declining kidney function).

Biopsy findings you must know:

  • LM: Mesangial proliferation/expansion
  • IF: Granular mesangial IgA
  • EM: Mesangial electron-dense deposits

Treatment (Step 1 + Step 2 angle)

  • Supportive: BP control (ACEi/ARB especially if proteinuria)
  • In higher-risk disease: immunosuppression may be considered (typically specialist-driven)
  • Monitor for progression to CKD

Prognosis

Variable. Some have benign recurrent hematuria; others progress to CKD (risk rises with significant proteinuria, HTN, reduced GFR).


Post-streptococcal Glomerulonephritis (PSGN): High-Yield Deep Dive

What it is

A nephritic syndrome occurring after infection with nephritogenic strains of group A strep:

  • Strep pyogenes causing pharyngitis
  • or impetigo

Timing is everything

  • 1–3 weeks after pharyngitis
  • 3–6 weeks after impetigo

If hematuria happens at the same time as the sore throat, think IgA nephropathy, not PSGN.

Pathophysiology

  • Formation of immune complexes involving streptococcal antigens
  • Deposition in glomeruli → complement activation → inflammation
  • Classic lab: low C3 due to complement consumption

Clinical presentation (classic vignette)

Child with:

  • Recent strep infection
  • Dark urine + periorbital edema
  • Hypertension
  • Oliguria
  • Possibly mild proteinuria

Diagnosis

Key supportive labs:

  • Elevated anti-streptolysin O (ASO) (more useful after pharyngitis)
  • Elevated anti–DNase B (more useful after skin infections)
  • Low C3 (complement consumption)

Urinalysis:

  • Hematuria
  • RBC casts
  • Mild-to-moderate proteinuria

Biopsy findings (must memorize):

  • LM: Enlarged, hypercellular glomeruli (diffuse proliferative pattern)
  • IF:Lumpy-bumpy” granular deposits of IgG, IgM, C3 along GBM and mesangium
  • EM: Subepithelial humps (immune complex deposits)

First Aid cross-reference: Renal → Poststreptococcal GN; Microbiology → Strep pyogenes complications (rheumatic fever vs PSGN).

Treatment

  • Supportive care: control blood pressure, manage volume overload (diuretics), monitor renal function/electrolytes
  • Treat active infection if still present (antibiotics prevent transmission; they don’t reliably prevent PSGN once immune response is triggered)

Prognosis

  • Children: often recover completely
  • Adults: higher risk of persistent renal dysfunction

How to Nail the Differential on Test Day

Nephritic syndrome vs nephrotic syndrome (rapid distinction)

  • Nephritic: hematuria + RBC casts + HTN + azotemia; proteinuria is present but not massive
  • Nephrotic: heavy proteinuria > 3.5 g/day, edema, hyperlipidemia, lipiduria (fatty casts)

IgA nephropathy vs PSGN (rapid distinction)

Ask:

  1. When did hematuria start relative to infection?
    • Days = IgA nephropathy
    • Weeks = PSGN
  2. Complement low?
    • Low C3 = PSGN (classically)
  3. Serologies for strep?
    • ASO / anti-DNase B elevated = PSGN

High-Yield Associations & “Buzz Phrases” (Memorize)

IgA nephropathy

  • Synpharyngitic hematuria
  • Mesangial IgA deposition”
  • Associated with HSP (IgA vasculitis)

PSGN

  • 1–3 weeks after strep throat
  • 3–6 weeks after impetigo
  • Subepithelial humps” (EM)
  • Lumpy-bumpy” granular IF
  • Low C3, ↑ ASO, ↑ anti-DNase B

Mini Self-Quiz (Quick Vignette Practice)

  1. Teen develops gross hematuria 2 days after a sore throat. Complement is normal. Most likely diagnosis?
  • IgA nephropathy
  1. Child had impetigo 4 weeks ago, now has cola-colored urine and periorbital edema. Labs show low C3 and elevated anti–DNase B. Biopsy EM shows what?
  • Subepithelial humps (PSGN)
  1. UA shows RBC casts and mild proteinuria in both conditions. What feature most strongly points to PSGN?
  • Low C3 + weeks-later timing after strep infection