5. Nephritic Syndrome - Sakrani

• Azotemia vs uremia

  • azotemia = elevated BUN + creatinine
• What is the main matrix of nephritic cast?

  tamm horsfall mucoprotein
• RBC cast is important indicator of?

  glomerulonephritis
• WBC casts - associated with?

  • tubulointerstitial disease
  • acute pyelonephritis
• waxy casts

  • renal stasis - associated with severe chronic kidney disease
• granular casts

  • stasis of urine
  • seen in acute tubular necrosis
• PSGN classic presentation

  • hematuria
  • edema
  • hypertension
• PSGN follow

  • strep pharyngitis in winter
  • strep skin infection (strep pyoderma) in summer
• PSGN follows skin infection (impetigo) and pharyngitis with Grp A B-hemolytic strep with M antigen
• PSGN Lab finding

  • hematuria + RBC casts
  • decrease serum total and C3 complement
  • increase titer for anti-streptolysin O (ASO) and anti-DNAse
• PSGN - do not use immunosuppressant like steroid for treatment
• PSGN - biopsy

  • subepithelial immune deposits - humps
• IgA Nephropathy (Berger‘s disease ) present as - episodic hematuria - with deposition of IgA in the mesangium
• IgA nephropathy - recurrent episodes of hematuria following?

  upper respiratory infection
• IgA nephropathy diagnosis - presence of

  • mesangial IgA deposits
  • mesangial hypercellularity
• Henoch Schonlein purpura (IgA vasculitis) - presence of IgA immune complex in the small blood vessels of

  • skin
  • intestine
  • joints
• IgA vasculitis - triad

  • purpuric rash
  • ab colicky pain
  • arthralgias
• IgA vasculitis - treatment = pulse steroid

  • if worsening proteinuria + GFR
• Rapidly progressive GN (Crescentic GN) - rapid loss of GRF with progression to ESRD

  • crescent formation is due to glomerular cell death
• RPGN types

  • type 1 - anti-GBM antibody = good pasture
  • type 2 - immune complex mediated
  • type 3 - pauci immune - NO Ig
• Good pasture syndrome - pt present with lung hemorrhage + GN

  • antibody against GM antigen
• Alport syndrome vs Goodpasture - both anti-glomerular BM disease

  • alport - eye + hearing loss
  • good pasture - hematuria + hemoptysis - renal
• Good pasture treatment - triple arm management

  • pulse steroid
  • cyclophosphamide
  • daily plasmapheresis to remove anti-GBM antibodies
• ANCA - affect small to medium blood vessels

  • treat early because it can lead to multiple organ failure
• ANCA - affect small vessels - glomerular capillaries causing gn

  • have serum ANCA+
• ANCA type + antibody

  • granulomatosis with polyangiitis = anti PR3 antibodies
  • microscopic polyangiitis + eosinophilic granulomatosis with polyangiitis = anti MPO antibodies
• Granulomatosis with Polyangiitis (formerly Wegener’s granulomatosis)

  • renal biopsy - granulomas with segmental necrotizing GN without immune deposits
• Common cause of Granulomatosis with Polyangiitis (formerly Wegener’s granulomatosis)

  • exposed to silica dust - people with alpha1 antitrypsin deficiency
• Eosinophilic Granulomatosis with Polyangiitis (Formerly Churg-Strauss Syndrome

  • may have exudative pleural effusion + eosinophils
• what is common with active lupus nephritis?

  hypocomplementemia
• What antibodies is present with systemic lupus?

  anti-dsDNA antibodies
• What is recommended as diagnostic for lupus?

  renal biopsy with evidence of nephritis
• SLE (lupus) - wire loop appearance

  
• Class 3 + 4 Lupus treatment

  • high dose steroid + a second agent
  • after response - maintain with mycopheno or azathio
• if ANCA or good pasture - tx

  • plasmapheresis