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Combination of Tests

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Wegener's Granulomatosis: Sensitivity and Specificity

Introduction: An excellent summary article is available:

Wegener's Granulomatosis: Current Trends in Diagnosis and Management

PMID: 17483685

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Tags: Autoimmune Ear Nose and Throat Vasculitis Tag this Diagnosis.

Prevalence

Population / CalculatorPrevalence Comments / Study / Link
Sweden pediatric population 0% Study found incidence of WG in children and young adults in Sweden to be less than 1 in 2 million per year.

PMID 10752759

Stegmayr BG, Gothefors L, Malmer B, Müller Wiefel DE, Nilsson K, Sundelin B.

Wegener granulomatosis in children and young adults. A case study of ten

patients. Pediatr Nephrol. 2000 Mar;14(3):208-13. PubMed PMID: 10752759.

More, Edit...

The sensitivity and specificity of findings for Wegener's Granulomatosis are listed below. See the left navigation bar to change the display.

Combination of Tests

Finding SensitivitySpecificity Comments, Study
ACR Criteria Edit 88%92%

2 of the 4 criteria must be met from the 1990 Criteria for the Classification of Wegener's Granulomatosis:

1. Nasal or oral inflammation

  • Development of painful or painless oral ulcers or purulent or bloody nasal discharge
  • 2. Abnormal chest radiograph

  • Chest radiograph showing the presence of nodules, fixed infiltrates, or cavities
  • 3. Urinary sediment

  • Microhematuria (>5 red blood cells per high power field) or red cell casts in urine sediment
  • 4. Granulomatous inflammation on biopsy

  • Histologic changes showing granulomatous inflammation within the wall of an artery or in the perivascular or extravascular area (artery or arteriole)
  • Study: PMID 2202308

    Leavitt RY, Fauci AS, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis. Arthritis Rheum. Aug 1990;33(8):1101-1107.

    Untagged

    Finding SensitivitySpecificity Comments, Study
    anti-MPO Edit 24%91%

    specificity vs healthy people

    Study: Kidney Int. 1998 Mar;53(3):743-53. PMID: 9507222

    anti-PR3 Edit 66%87%

    specificity vs healthy people

    Study: Kidney Int. 1998 Mar;53(3):743-53. PMID: 9507222

    c-ANCA
    Duplicate Edit
    91%99%

    for active disease

    Study: Ann Intern Med. 1995 Dec 15;123(12):925-32. PMID: 7486487

    c-ANCA
    Duplicate Edit
    63%100%

    for inactive disease

    Study: Ann Intern Med. 1995 Dec 15;123(12):925-32. PMID: 7486487

    c-ANCA
    Duplicate Edit
    64%95%

    specificity vs healthy people

    Study: Kidney Int. 1998 Mar;53(3):743-53. PMID: 9507222

    c-ANCA and anti-PR3 Edit 73%99%

    specificity vs healthy people

    Study: Kidney Int. 1998 Mar;53(3):743-53. PMID: 9507222

    Erythematous painful pinna Edit No accuracy specified.

    Study: no study specified.

    Leukopenia Edit No accuracy specified.

    Leukopenia is NOT a feature of WG. A Leukocyte count < 4000/mm3 can be seen in patients with lymphomatoid granulomatosis which evolves into lymphoma. It is often confused with WG.

    Study: PMID 6336643

    See pages 79-80:

    Fauci AS, Haynes BF, Katz P, Wolff SM. Wegener's granulomatosis: prospective

    clinical and therapeutic experience with 85 patients for 21 years. Ann Intern

    Med. 1983 Jan;98(1):76-85. Review. PubMed PMID: 6336643.

    p-ANCA Edit 21%81%

    specificity vs healthy people

    Study: Kidney Int. 1998 Mar;53(3):743-53. PMID: 9507222

    Sensorineural hearing loss (SNHL) Edit No accuracy specified.

    2.8 to 13% incidence of SNHL in WG patients.

    Study: Hearing loss in Wegener's granulomatosis.

    Bakthavachalam S, Driver MS, Cox C, Spiegel JH, Grundfast KM, Merkel PA.

    Otol Neurotol. 2004 Sep;25(5):833-7.

    PMID 15354019