Nephritic syndrome

Summary

Nephritic syndrome is characterized by glomerular damage leading to hematuria, pyuria, water

retention, and subsequent hypertension and edema. It can be caused by a variety of conditions
including autoimmune, hereditary, and infectious diseases. This learning card provides an
overview of nephritic syndrome; underlying conditions are discussed in more detail in other
learning cards. Nephritic diseases can present in varying degrees of severity, ranging from
asymptomatic hematuria to systemic involvement as in rapidly progressive glomerulonephritis.
The urine sediment is typically characterized by red blood cell (RBC) casts, mild to
moderate proteinuria (< 3.5 g/day), and sterile pyuria. Diagnosis of the underlying disease is
often based on presentation and laboratory values, although renal biopsy may be indicated for
confirmation.
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Definition

Nephritic syndrome consists of a combination of some or all of the following signs:

• Hematuria with acanthocytes

• RBC casts in urine
• Proteinuria (< 3.5 g/24h)
• Hypertension
• Mild to moderate edema
• Sterile pyuria
• Oliguria
• Azotemia

References:[ref][ref]
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 Associations Findings

• Occurs weeks after group A β- • Positive • Us

Poststreptococcal glomerulonephritis
hemolytic streptococci infections •
antistreptococcal antibodies (ASO, ADB) Su
o •
Pharyngitis/tonsillitis (most common): 1–2 weeks ↓ Serum C3 complement levels (se
o Skin infections: 3–4 weeks • Tea- or cola-colored urine be
• •
Usually affects children between the ages of 3–12 LM: glomeruli appear enlarged and
years hypercellular
• Can lead to RPGN in some cases • IM: granular subepithelial immune
complex depositions (IgG, IgM, C3)
→ “lumpy-bumpy” appearance
• EM: dome-shaped, subepithelial
deposits (“humps”)

• Most • Asymptomatic microhematuria with • Su

IgA nephropathy
common idiopathic glomerulonephritis worldwide intermittent gross hematuria (se
(Berger's disease) • Episodic gross hematuria during or directly after• ↑ Serum IgA be
•
upper respiratory tract (URT), gastrointestinal (GI) Normal C3 complement levels • Gl
infections, or strenuous exercise • LM: mesangial proliferation se
• Occurrence: ♂ > ♀ • IM: mesangial IgA deposits
• Peak incidence: 2 to 3 decade of life
nd rd • EM: mesangial immune
• ∼ 25-30% of the patients progress to end- complex deposits
stage renal disease (ESRD) within 20–25 years

Small • Pulmonary and nasopharyngeal involvement is • c-ANCA • Su

Granulomatosis with
vessel common → hemoptysis, nasal ulcers • Renal biopsy: segmental (se
polyangiitis (Wegener's)
vasculitis necrotizing glomerulonephritis be
• EM: immune complex depositions • Im
th
• Usually only mild respiratory symptoms • p-ANCA
Microscopic
polyangiitis

• Patients present with: • p-ANCA (∼ 50% of cases)

Churg-Strauss
o Asthma • Peripheral eosinophilia
syndrome
o Allergic rhinitis • Focal segmental
o Purpura necrotizing glomerulonephritis
o Peripheral neuropathy

• Two peaks of occurrence: 3rd decade of • Pulmonary infiltrates on chest x-ray • Im

Goodpasture syndrome
life (♂ > ♀) and ≥ 60 years of age (♀ > ♂) • IM: linear deposition th
(Anti–GBM antibody disease)• Caused by antibodies against type IV collagen of immunoglobulin (IgG) along • Pla
• Antibodies can cross-react with basement the glomerular basement membrane
membrane of pulmonary capillaries and lead
to pulmonary hemorrhage and hemoptysis
• Can lead to RPGN
 • Hereditary disorder • Persistent microhematuria • Re
Thin basement membrane
• Abnormalities of type IV collagen • Possible episodic gross hematuria, mo
disease
• Good prognosis typically occurring during or directly • Su
after an upper respiratory tract (se
infection or exercise be
• EM: diffuse thinning of glomerular
basement membrane

• X-linked (usually affects males) • Persistent microhematuria with • Su

Alport syndrome
• Mutation in gene for type IV collagen intermittent gross hematuria (se
• Often leads to ESRD • Associated with sensorineural hearing be
loss and abnormalities of the eye • Re
• EM: splitting and alternating thickening tra
and thinning of the glomerular to
basement membrane of
syn

Lupus nephritis • Complication of systemic lupus erythematosus • ANA, anti-dsDNA antibodies • Su

• Can be nephritic or nephrotic • Histologic changes are very variable (se
• Can lead to RPGN in some cases be
• Im
th

• •
Glomerular diseases that progress to ESRD within Can be caused by a variety of diseases:• Im
Rapidly progressive
weeks to months o Goodpasture syndrome (anti-GBM th
glomerulonephritis
disease) • Pla
(RPGN) o Poststreptococcal glomerulonephritis
o Systemic lupus erythematosus (SLE)
o Microscopic polyangiitis
o Granulomatosis with
polyangiitis (Wegener's)
• LM, IM, EM: crescent
formation, monocytes, macrophages

LM = light microscopy, IM = immunofluorescent microscopy, EM = electron microscopy

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Etiology
 References:[ref][ref][ref][ref][ref][ref][ref][ref][ref][ref]
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Pathophysiology

• Inflammation → cytokine release → glomerular capillary damage

o Porous glomerular basement membrane → leakage of proteins and RBCs → nephritic sediment (all
blood components are detectable on urinalysis)
▪ Proteinuria (< 3.5 g/24h): leakage of proteins
▪ Hematuria: leakage of RBCs, which stick together and form red blood cell casts in the renal
tubules
o Oliguria: inflammatory infiltrates reduce fluid movement across the membrane (↓ GFR)
o Azotemia: inflammation prevents sufficient filtering and excretion of urea
o Salt retention → intravascular volume expansion → hypertension and edema

References:[ref][ref]
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Clinical features

• Intermittent gross hematuria (red or brown urine"cola-colored urine"),

• Hypertension
• Pitting edema
• If ↓GFR: oliguria and uremic symptoms (see uremia)
• For a comparison of nephrotic and nephritic syndrome see nephrotic vs. nephritic syndrome

References:[ref]
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Diagnostics

• Urinalysis:
o Nephritic sediment
▪ Hematuria (either micro- or intermittent macrohematuria)
▪ Dysmorphic red blood cells (acanthocytes)
▪ Red blood cell casts
▪ Mild to moderate proteinuria of > 150 mg/24h but < 3.5 g/24h (non-selective glomerular proteinuria)
▪ Sterile pyuria and sometimes WBC casts
• Renal biopsy: sometimes indicated in patients with a nonspecific disease pattern to confirm
diagnosis
• Blood tests
o ↑ Creatinine, ↓ GFR
o Azotemia with ↑ BUN

Glomerular hematuria is a typical finding in nephritic syndrome. It is characterized

by acanthocytes, RBC casts, and mild to moderate proteinuria! (Nonglomerular hematuria is
characterized by bright red or pink urine, the occurrence of blood clots,
normal RBC morphology, and the absence of RBC casts.)

References:[ref][ref]
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Differential diagnoses

• See nephrotic vs. nephritic syndrome.

The differential diagnoses listed here are not exhaustive.

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 Treatment

• Supportive therapy
o Low-sodium diet
o Water restriction
• Medical therapy
o If proteinuria and/or hypertension: angiotensin-converting enzyme inhibitors or angiotensin-
receptor blockers
o If severe hypertension and/or edema: diuretics
o Sometimes immunosuppressive therapy is indicated.
• If severe renal insufficiency or kidney failure: renal replacement therapy (e.g., hemodialysis,
possibly transplantation)
• For management of specific diseases: See the “Therapy” sections of the respective learning
cards.

References:[ref]
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Sources

last updated 02/12/2020