Monday 5/16/16 Pauci-Immune GN

  • Diffuse Alveolar Hemorrhage is caused by disruption of the alveolar-capillary basement membrane
    • Diagnosed via progressively more hemorrhage after saline lavage on BAL (see image below!)
    • Chest Xray in DAH is non-specific, there can be new patchy/diffuse opacities
    • 33% DON’T have hemoptysis
    • Classically, DAH presents as acute onset cough, dyspnea, and hemoptysis
    • Causes
      • 1)  Vasculitis (MPA, GPA, Churg-Strauss)
      • 2) Pulmonary Renal Syndromes: Goodpastures, IgA Nephropathy
      • 3) Autoimmune: SLE, APLS
      • 4) Meds:  Amiodarone, cocaine, nitrofurantoin, anticoagulants
  • Nephritic Syndromes: Rapidly progressive GN occurs among the nephritic syndromes (not nephrotic syndromes). The nephritic syndromes can be divided per the image below.
    • c-anca more associated with Wegener, mpa and churg strauss more associated with p-anca, but significant overlap
    • Wegeners a/w sinusitis and Churg-strauss associated with eosinophilia and asthma
    • Pauci-Immune GN is called that because there are no immune deposits seen on immunofluoroscopyDAH
    • GN

 

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