Wegeners granulomatosis diagnostic criteria
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Vasculitis such as Wegener's granulomatosis is usually only suspected when a patient has had unexplained symptoms for a longer period of time. 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 enzymeproteinase 3 (cANCA) in neutrophils (a type of white blood cell) are associated with Wegener's.
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. On histopathological examination, a biopsy will showleukocytoclastic vasculitis with necrotic changes and granulomatousinflammation (clumps of typically arranged white blood cells) on microscopy. The latter is the main reason for the appellation of "Wegener's granulomatosis", although it is not an essential feature. Unfortunately, many biopsies can be non-specific and 50% provide too little information for the diagnosis of Wegener's.
Diagnostic Criteria
In 1990, the American College of Rheumatology accepted classification criteria for Wegener's. 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 Wegener's.[1]
- 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 Wegener's granulomatosis demands:[2]
- 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.[3]
References
- ↑ 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.
- ↑ 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.
- ↑ Bruce IN, Bell AL. A comparison of two nomenclature systems for primary systemic vasculitis. Br J Rheumatol1997;36:453-8. PMID 9159539.