• Clinical science

Nephritic syndrome

Abstract

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.

Definition

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

References:[1]

Etiology

Associations Findings Treatment
Poststreptococcal glomerulonephritis
  • Positive antistreptococcal antibodies (ASO, ADB)
  • ↓ Serum C3 complement levels
  • Tea- or cola-colored urine
  • LM: glomeruli appear enlarged and hypercellular
  • IM: granular subepithelial immune complex depositions (IgG, IgM, C3) → “lumpy-bumpy” appearance
  • EM: dome-shaped, subepithelial deposits (“humps”)
  • Usually self-limiting
  • Supportive treatment (see “Therapy” below)

IgA nephropathy

(Berger's disease)

  • Most common idiopathic glomerulonephritis worldwide
  • Episodic gross hematuria during or directly after upper respiratory tract (URT), gastrointestinal (GI) infections, or strenuous exercise
  • Occurrence: >
  • Peak incidence: 2nd to 3rd decade of life
  • ∼ 25-30% of the patients progress to end-stage renal disease (ESRD) within 20–25 years
  • Asymptomatic microhematuria with intermittent gross hematuria
  • ↑ Serum IgA
  • Normal C3 complement levels
  • LM: mesangial proliferation
  • IM: mesangial IgA deposits
  • EM: mesangial immune complex deposits
  • Supportive treatment (see “Therapy” below)
  • Glucocorticoids in severe cases
Small vessel vasculitis

Granulomatosis with polyangiitis (Wegener's)

  • Pulmonary and nasopharyngeal involvement is common → hemoptysis, nasal ulcers

Microscopic polyangiitis

  • Usually only mild respiratory symptoms

Churg-Strauss syndrome

Goodpasture syndrome

(Anti–GBM antibody disease)

Thin basement membrane disease

  • Persistent microhematuria
  • Possible episodic gross hematuria, typically occurring during or directly after an upper respiratory tract infection or exercise
  • EM: diffuse thinning of glomerular basement membrane
  • Renal function monitoring
  • Supportive treatment (see “Therapy” below)

Alport syndrome

  • Persistent microhematuria with intermittent gross hematuria
  • Associated with sensorineural hearing loss and abnormalities of the eye
  • EM: splitting and alternating thickening and thinning of the glomerular basement membrane
Lupus nephritis

Rapidly progressive glomerulonephritis

(RPGN)

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

References:[2][3][1][4][5][6][7][8][9][10]

Pathophysiology

References:[7][11]

Clinical features

References:[1]

Diagnostics

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:[12][13]

Treatment

References:[13]

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  • Yuste C, Gutierrez E, Sevillano AM, et al. Pathogenesis of glomerular haematuria. World J Nephrol. 2015; 4(2): p. 185. doi: 10.5527/wjn.v4.i2.185.
last updated 08/19/2018
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