Pharmacokinetics Of Emulsified Avacopan Applied By NG Tube

Inclusion Criteria

* Fulfillment of the definitions of the Second Chapel Hill Consensus Conference for ANCA-associated vasculitis (either granulomatosis with polyangiitis (GPA) or microscopic polyangiitis MPA).
* Positivity for ANCA, directed against PR3 or MPO.
* Diffuse alveolar hemorrhage.
* Respiratory failure requiring mechanical ventilation.
* Severe newly diagnosed disease or severe relapsing disease. Severe new or relapsing disease is defined as at least one major BVAS/WG item or a score ≥ 3 and the investigator deems standard treatment for severe disease is necessary.
* Minimum BVAS-WG of 3.
* Requirement of standard-of-care remission induction therapy for active severe ANCA-associated vasculitis (GPA or MPA).

Exclusion Criteria

* Diagnosis with eosinophilic granulomatosis with polyangiitis (EGPA, formally Churg-Strauss syndrome) as defined by the Chapel Hill consensus conference.
* Allergies: History of severe allergic reaction to avacopan
* History of documented anti-glomerular basement membrane disease (anti-GBM disease)
* Previous administration of avacopan within the last 5 days.
* Concomitant use of a strong CYP3A4 inhibitor.
* Aspartate aminotransferase \[AST\], alanine amino transferase \[ALT\], alkaline phosphatase, or total bilirubin elevation \>2.5 times the upper limit of normal (unless attributed to vasculitis) on routine liver function testing obtained within 3 days prior to anticipated treatment with avacopan.
* Evidence of prior active or current Hepatitis B virus (HBV), hepatitis C virus (HCV), or human immunodeficiency (HIV) infection.
* Active serious infection, including localized infection.
* Pregnancy and breastfeeding