Updated: 12/15/2019

Nephritic Syndrome

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Snapshot
  • A 26-year-old man presents with hematuria, periorbital edema, and jaundice. He has a medical history of opioid use disorder with prior hospitalizations for heroin overdose. He is on methadone but is non-adherent. His blood pressure is 155/102 mmHg. Physical examination is significant for scleral icterus, hepatomegaly, and palpable purpura. Serology shows decreased C3 and C4 levels and elevated anti-hepatitis C antibodies. Urinalysis demostrates dysmorphic red blood cells and red blood cell casts.  (Membranoproliferative glomerulonephritis)
Introduction
  • Clinical definition
    • renal disease secondary to an inflammatory process injuring the glomerulus
      • this results in damage involving the
        • basement membrane
        • capillary endothelium
        • mesangium
  • Presentation
    • symptoms
      • hypertension
      • hematuria
      • oliguria
      • headache
    • physical exam
      • edema
        • can be peripheral and/or periorbital
  • Diagnosis
    • studies
      • complete blood cell count
        • anemia may be noted
      • azotemia
      • complement levels
        • C3, C4, and CH50 should be obtained
      • urinalysis 
        • dysmorphic red blood cells (RBCs)
          • suggests hematuria is of glomerular origin
        • RBC casts
        • subnephrotic range proteinuria (< 3.5 g/day)
          • if the nephritic syndrome is severe enough it can lead to nephrotic range proteinuria (> 3.5 g/day)
    • renal biopsy
      • may be necessary to arrive to a definitive diagnosis and to determine prognosis
 
Nephritic Syndrome
Type
Pathophysiology
Renal Biopsy
Diagnostic Studies and Treatment
Acute poststreptococcal glomerulonephritis
  • Glomerulonephritis secondary to nephritogenic strains of streptococcus
    • type III hypersensitivity reaction 
  • Light microscopy
    • glomerular hypercellularity 
      • e.g., polymorphonuclear leukocytes 
  • Immunofluoresence
    • diffuse granular pattern on glomerular capillary and mesangium
      • deposition of IgG, IgM, and C3 
  • Electron microscopy
    • electron-dense, glomerular subepithelial immune-complex deposits ("humps")
  • Streptococcus titers and serologies are positive
    • e.g., ASO levels 
  • ↓ serum C3 levels
  • Typically self-resolves
Rapidly progressive glomerulonephritis
  •  Goodpasture syndrome  
    • type II hypersensitivity
    • anti-GBM antibodies against α3-chain of collagen type IV 
      • antibodies to the alveolar basement membrane result in hemoptysis and lung disease
  • Pauci-immune processes
    • granulomatosis with polyangiitis
    • microscopic polyangiitis
  • Light microscopy and immunofluoresence
    • crescent-shaped deposition of fibrin, C3b, glomerular parietal cells, monocytes, and macrophages
  • Granulomatosis with polyangiitis
    • PR3-ANCA/c-ANCA positive 
  • Microscopic polyangiitis
    • MPO-ANCA/p-ANCA
  • Corticosteroids and cyclophosphamide
IgA nephropathy (Berger disease)
  • IgA immune-complex deposition in glomerular mesangial cells that results in its proliferation 
    • patients present with hematuria and upper respiratory tract or gastrointenstinal infection
  • Light microscopy
    • mesangial proliferation
  • Immunofluoresence
    • IgA immune-complex deposition in the mesangium
  • ACE inhibitor or ARB for proteinuria and hypertension
Alport syndrome
  • Collagen type IV mutation that results in an abnormal basement membrane
    • more commonly an X-linked genetic disorder
    • characterized by
      • renal involvement
      • ocular involvement
      • sensorineural hearing loss
  • Electron microscopy
    • glomerular basement membrane lamellation
  • No curative treatment
Membranoproliferative glomerulonephritis (MPGN)  
  • Immune-complex and/or complement protein deposition in the mesangium and subendothelium of the capillaries
    • this results in proliferation of the mesangium and remodeling of the capillary wall
    • may be secondary to
      • hepatitis C virus
      • hepatitis B virus
      • C3 nephritic factor
        • MPGN type II
  • Can also present as a nephrotic syndrome
  • Light microscopy
    • mesangial proliferation and thickening of the capillary wall
  • Immunofluoresence
    • "tram-track" appearance
  • ↓ serum C3 and C4 levels
  • Treat underlying disease in secondary causes
    • e.g., control of hepatitis C virus infection
 
 

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(M2.RL.14.54) A 38-year-old female presents to emergency department with "swollen eyes and feet." The patient states that she was in her normal state of health until one week ago when she noticed that her shoes and rings were not fitting. In the ED the patient is afebrile with vitals HR 75, BP168/83. The patient cannot comment on her baseline blood pressure. Physical exam is otherwise notable for 2+ pitting edema at the ankles and periorbital edema. In the ED, urinalysis is positive for protein. The patient is admitted, and her kidney disease is further work-up, ultimately revealing low serum C3 and "tram-tracking" on kidney biopsy (Figure A). Which of the following, if part of the patient's medical history, would be a potential etiology for the her kidney disease? Tested Concept

QID: 104845
FIGURES:
1

8 years of HAART for HIV

10%

(9/90)

2

Chronic hepatitis C without interferon therapy

54%

(49/90)

3

Diagnosis of rheumaoid arthritis (RA) at the age of 28, managed with herbal medicine

20%

(18/90)

4

Untreated syphilis

1%

(1/90)

5

Diagnosis of type 1 diabetes at the age of 16 with intermittant periods of poor control

13%

(12/90)

M 6 E

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