Wegener's granulomatosis consists of a clinical triad, with glomerulonephritis, a variable degree of small vessel vasculitis, and granulomatous vasculitis of the upper and lower respiratory tract.
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Criteria
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Comment
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nasal or oral inflammation
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development of painful or painless oral ulcers; or, purulent or bloody nasal discharge
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abnormal chest radiograph
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presence of nodules, fixed infiltrates, or cavities
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urinary sediment
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microhematuria ( > 5 RBCs per high power field), or red cell casts
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granulomatous inflammation on biopsy
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granulomatous inflammation within the wall of an artery, or in the perivascular or extravascular area (artery or arteriole)
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Additional findings:
(1) nonspecific signs of symptoms, with fever, malaise, weight loss, myalgias and arthralgias
(2) biopsy of a diagnostic area will show necrosis of small arteries and veins with fibrinoid necrosis, a mononuclear cell infiltrate, and necrotizing granulomas
(3) presence of autoantibodies such as rheumatoid factor, anti-endothelial cell antibodies and antineutrophil cytoplasmic antibodies (ANCA)
(4) increased circulating B-lymphocytes
(5) elevated serum immunoglobulin levels and acute phase reactants
(6) vascular deposits of immunoglobulins and/or complement
(7) complaints of nasal obstruction or sinusitis, with abnormal sinus radiographs
(8) otitis media
(9) bronchoalveolar lavage may contain ANCA in patients without radiographic infiltrates
(10) proteinuria
(11) glomerulonephritis ranging from minimal to severe, with renal compromise ranging from slight to rapidly progressive renal failure
Limitations:
• The disease may be limited to airway involvement only.
• Biopsies of nasopharynx, sinus mucosa, gingiva or skin may be negative or show only nonspecific inflammation. The lungs may be the only site showing diagnostic histopathologic change.
• The differential diagnosis included fungal infection, tuberculosis, syphilis berylliosis, nasopharyngeal carcinoma, malignant lymphoma, midline granulomas, other forms of vasculitis, streptococcal infection with glomerulonephritis, and Goodpasture's syndrome.
Interpretation:
• The presence of 2 or more criteria suggests the diagnosis of Wegener's granulomastosis.
Performance:
• The sensitivity is 88% and specificity 92%.
