Wegeners Granulomatosis

Pathogenesis and Pathophysiology. The almost universal involvement of the upper or lower airway in Wegener's granulomatosis (WG) has led to a theory that the condition results from exposure to an inhaled antigen with resultant granulomatous inflammation and altered immunoreactivity. y Cell-mediated immunity and immune complexes are felt to play significant roles. There may be genetic or other predisposing host factors. A strong association with cytoplasmic antineutrophil cytoplasmic antibodies (cANCA) suggests a pathogenic role. It has been shown in vitro that cANCA causes neutrophils to undergo a respiratory burst and degranulation, releasing toxic superoxides. y However, there is no direct evidence to confirm that it is an etiologic factor in WG.

Epidemiology and Risk Factors. The incidence of WG in the United States is about 1 in 30,000. Among 180 patients referred to the National Institute of Allergy and Infectious Diseases, 97 percent with WG were white with equal numbers of men and women. The mean age was 41 years (range 9 to 79 years) and 15 percent were less than 19 years of age.y The ease of identification of the disorder with cANCA has increased disease ascertainment.

Clinical Features and Associated Disorders. Criteria for the diagnosis of WG are listed in Tabie^G-S .'291 Ninety percent of patients with WG have upper and/or lower airway symptoms. Median duration of these symptoms at the time of diagnosis is 4.7 months. Nasal, sinus, and ear abnormalities are seen at presentation in 73 percent and during the illness in 92 percent. Lung disease develops in 85 percent of cases over the course of the illness, although it is asymptomatic in one third of cases. Eighteen percent of patients present with asymptomatic glomerulonephritis and 77 percent of patients develop glomerulonephritis, usually within the first two years of disease onset.y

Nervous system involvement is rare at initial presentation but mononeuritis multiplex develops eventually in 15 percent of patients. Central nervous system involvement occurs in 8 percent of patients and includes stroke, cranial nerve abnormalities, and diabetes insipidus. y In literature series, neurologic involvement with WG occurs in 22 to 54

_TABLE 50-5 -- CRITERIA FOR THE DIAGNOSIS OF WEGENER'S GRANULOMATOSIS_

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