Vasculitis

Pathophysiology/Presentation

• granulomatous inflammation, necrotizing vasculitis, ANCA+

• Always involves resp tract + kidneys

• Pulmonary-kidney syndrome; pulmonary hemorrhage and severe GN

• Upper airway symptoms (i.e. sinusitis, septum perforation)

• skin rash (non-blanching palpable purpura), hematuria, proteinuria, HTN, AKI, edema

Workup

• KFP: normal to severe AKI, may have rapidly rising sCr; sAlb low to normal

• CBC: low to normal Hb, no hemolysis, normal platelets

• UA: gross or microscopic hematuria, proteinuria mild to severe

• Complements: normal

• ANCA+

  • proteinase 3 (PR3); more common in Wegener’s

  • myeloperoxidase (MPO)

• Imaging:

  • Kidney US: mainly normal

  • Lung imaging/CT: alveolar hemorrhage, infiltrates, nodules

• Other ANCA+ vasculitides to keep in differential diagnosis:

  • Microscopic polyangiitis: mainly MPO-ANCA+, only renal involvement

  • Churg-Strauss: mainly MPO-ANCA+, eosinophil-rich necrotizing vasculitis

Treatment

• steroids, Cytoxan, Plasma Exchange

• may need dialysis

Prognosis/Follow up

• High mortality, often results in CKD/ESRD and need for kidney transplant, requires ongoing immunosuppression