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

Sweden pediatric population

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, MÃ¼ller 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.

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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

c-ANCA
Duplicate

91%

99%

for active disease

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

ACR Criteria

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	Sensitivity	Specificity	Comments, Study
c-ANCA Duplicate	63%	100%	for inactive disease Study: Ann Intern Med. 1995 Dec 15;123(12):925-32. PMID: 7486487
c-ANCA and anti-PR3	73%	99%	specificity vs healthy people Study: Kidney Int. 1998 Mar;53(3):743-53. PMID: 9507222
c-ANCA Duplicate	64%	95%	specificity vs healthy people Study: Kidney Int. 1998 Mar;53(3):743-53. PMID: 9507222
anti-MPO	24%	91%	specificity vs healthy people Study: Kidney Int. 1998 Mar;53(3):743-53. PMID: 9507222
anti-PR3	66%	87%	specificity vs healthy people Study: Kidney Int. 1998 Mar;53(3):743-53. PMID: 9507222

Poorly Diagnostic Findings

p-ANCA

21%

81%

specificity vs healthy people

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

Findings With Unspecified Accuracy

Finding	Sensitivity	Specificity	Comments, Study
Sensorineural hearing loss (SNHL)	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	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	No accuracy specified.		Study: no study specified.