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Updated: Jun 1 2022

Nephritic Syndrome

  • Snapshot
    • A 14-year-old boy presents with hematuria, problems with vision, and deafness. Approximately 2 weeks prior to symptom development, he recovered from a viral upper respiratory tract infection. Family history is significant for deafness and early initiation of lisinopril in his father. His temperature is 99°F (37.2°C), blood pressure is 140/90 mmHg, pulse is 75/min, and respirations are 18/min. Physical examination is signifcant for anterior lenticonus and sensorineural hearing loss. Urinalysis demonstrates dysmorphic red blood cells and red blood cell casts. (Alport syndrome)
  • 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 
      • Immunofluorescence
        • diffuse granular pattern on glomerular capillary walls and mesangium
          • deposition of IgG, IgM, and C3
      • Electron microscopy
        • electron-dense, glomerular subepithelial immune-complex deposits ("humps")
      • Streptococcus titers and serologies are positive
      • ↓ 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 immunofluorescence
        • 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's disease)
      • IgA immune-complex deposition in glomerular mesangial cells that results in its proliferation 
        • patients present with hematuria and upper respiratory tract or gastrointestinal infection
      • Light microscopy
        • mesangial proliferation
      • Immunofluorescence
        • 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 dominant disorder 
        • characterized by
          • renal involvement
          • ocular involvement
          • sensorineural hearing loss
      • Electron microscopy
        • glomerular basement membrane lamellation
        • "basket weave" appearance 
      • 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
      • Light microscopy
        • mesangial proliferation and thickening of the capillary wall
      • Immunofluorescence
        • "tram-track" appearance 
      • ↓ serum C3 and C4 levels
      • Treat underlying disease in secondary causes
        • e.g., control of hepatitis C virus infection
      • Diffuse proliferative glomerulonephritis (DPGN)
      • > 50% of glomeruli (thus diffuse) demonstrates an inflammatory process on histology
      • Common in patients with systemic inflammatory diseases (e.g., systemic lupus erythematosus)
        • can be secondary to membranoproliferative glomerulonephritis
      • Light microscopy
        • capillary "wire-looping"
      • Electron microscopy
        • immune complex deposition affecting the
          • subendothelial region
          • maybe intramembranous
      • Immunofluorescence
        • granular appearance
      • Can lead to death in patients with systemic lupus erythematosus
      • Aggressively treat with steroids
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