Granulomatosis with polyangiitis diagnostic study of choice

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Ali Poyan Mehr, M.D. [2]Associate Editor(s)-in-Chief: Krzysztof Wierzbicki M.D. [3]Amandeep Singh M.D.[4]


Determination of ANCAs can aid in the diagnosis, but positivity is not conclusive and negative ANCAs are not sufficient to reject the diagnosis. Cytoplasmic staining ANCAs that react with the enzyme proteinase 3 (cANCA) in neutrophils (a type of white blood cell) are associated with Granulomatosis with polyangiitis. If the patient has renal failure or cutaneous vasculitis, these are the most logical organs to obtain a biopsy from. Rarely, thoracoscopic lung biopsy is required.

Diagnostic Criteria

A diagnosis of Granulomatosis with polyangiitis can be made when three out of the six criteria are established. They are:[1]

  1. a histopathology that shows granuloma
  2. the upper respiratory tract is involved
  3. there is a stenosis that is present in larynx, trachea, and the bronchioles
  4. the pulmonary system is involved
  5. the presence of anti-neutrophil cytoplasmic antibodies
  6. the presence of glomerulonephritis

In 1990, the American College of Rheumatology accepted classification criteria for Granulomatosis with polyangiitis. They were not intended for diagnosis, but for inclusion in randomized controlled trials. Two or more positive criteria have a sensitivity of 88.2% and a specificity of 92.0% of describing Granulomatosis with polyangiitis.[2]

  • Nasal or oral inflammation:
    • painful or painless oral ulcers or
    • purulent or bloody nasal discharge
  • Lungs: abnormal chest X-ray with:
    • nodules,
    • infiltrates or
    • cavities
  • Kidneys: urinary sediment with:
    • microhematuriaor
    • red cell casts
  • Biopsy: granulomatous inflammation
    • within the arterial wall or
    • in the perivascular area

According to the Chapel Hill Consensus Conference (CHCC) on the nomenclature of systemic vasculitis (1992), establishing the diagnosis of Granulomatosis with polyangiitis demands:[3]

  • a granulomatous inflammation involving the respiratory tract, and
  • a vasculitis of small- to medium-sized vessels.

Several investigators have compared the ACR and Chapel Hill criteria.[4]


  1. Noone D, Hebert D, Licht C (2016). "Pathogenesis and treatment of ANCA-associated vasculitis-a role for complement". Pediatr Nephrol.   ( ):  . doi:10.1007/s00467-016-3475-5. PMID 27596099.
  2. Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis. Arthritis Rheum 1990;33:1101-7. PMID 2202308.
  3. Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, Hagen EC, Hoffman GS, Hunder GG, Kallenberg CG,et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference.Arthritis Rheum 1994;37:187-92. PMID 8129773.
  4. Bruce IN, Bell AL. A comparison of two nomenclature systems for primary systemic vasculitis. Br J Rheumatol1997;36:453-8. PMID 9159539.