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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.

Sensitive and Specific Findings

Finding SensitivitySpecificity Comments, Study
c-ANCA
Duplicate Edit
91%99%

for active disease

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

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.

    Specific Findings

    Finding SensitivitySpecificity Comments, Study
    c-ANCA
    Duplicate Edit
    63%100%

    for inactive disease

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

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

    specificity vs healthy people

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

    c-ANCA
    Duplicate Edit
    64%95%

    specificity vs healthy people

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

    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

    Poorly Diagnostic Findings

    Finding SensitivitySpecificity Comments, Study
    p-ANCA Edit 21%81%

    specificity vs healthy people

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

    Findings With Unspecified Accuracy

    Finding SensitivitySpecificity Comments, Study
    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

    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.

    Erythematous painful pinna Edit No accuracy specified.

    Study: no study specified.