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Occurrence of the ANCA

  • Disease Prevalence ANCA positive Antigen specificity
  • GPA 24/mio 40% - 98% PR3
  • MPA 25/mo 60% - 75% MPO
  • eGPA 10/mio 35% - 40% MPO

ANCA-associated small vessel vasculitis without immune complex formation

  • Granulomatosis with polyangiitis
  • Microscopic polyangiitis
  • Eosinophilic granulomatosis with polyangiitis
  • Renal limited ANCA vasculitis

ANCA-associated glomerulopathy

Membranous nephropathy Lupus nephritis IgA nephropathy

Small vessel vasculitis with immune complex formation

Goodpasture's disease

Medicines/ Substances

Propylthiouracil Levamisol (an agent for "stretching" cocaine)

inflammatory bowel disease

Colitis ulcerosa Primary biliary cirrhosis


In the context of bacteremia Subacute endocarditis Tbc Aspergillosis

Non-vasculitic rheumatic diseases, which can feign a non-specific ANCA titer

Systemic lupus erythematosus Primary Sjögren's syndrome Dermato-/polymyositis Systemic sclerosis Rheumatoid arthritis Reactive arthritis Antiphospholipid syndrome

adaptiert nach Praxis 2020; 109 (5): 325–331