Wegener's granulomatosis: an analysis of 33 patients seen over a 10-year period

Abstract

This retrospective study examined 33 patients with Wegener's granuloma seen between 1990 and 1999 in the Ayrshire and Arran region of Scotland. There was an estimated annual incidence of 10/million/year. The diagnosis in this series was based on the presence of one or more of the following: a positive histology, a positive c-ANCA or a typical clinical course. Twenty-eight patients were diagnosed based on either a positive histology and/or c-ANCA, whereas the remaining five were diagnosed based on the clinical course. c-ANCA was positive in 79.3% but correlated poorly with disease activity. Nasal biopsies were positive in 40%, whereas 94% of renal biopsies were positive, thereby making nasal biopsies unreliable in the diagnosis. Significantly elevated levels of erythrocyte sedimentation rate (ESR) averaging 77 mm/h were found in 32 patients at diagnosis. This showed fluctuation with disease activity. Thirteen patients died, 12 within 5 years. The best prognostic indicator statistically was age.

Relapsing polychondritis associated with bilateral stapes footplate fixation: a case report

“…Biopsy is therefore to be discouraged as it only adds to the mutilating nature of the disease. Such non-specific findings from biopsies are also common in other chronic conditions such as Wegener's granulomatosis [10]. Some authors have also emphasized that there is a significant correlation between elevated ESR and disease activity [2,4].…”

Orbital Granulomatosis With Polyangiitis (Wegener Granulomatosis): Clinical and Pathologic Findings

“…12 Laboratory tests have an essential role in current diagnosis of GPA. Serologic indicators of generalized inflammation, including erythrocyte sedimentation rate and C-reactive protein levels, are frequently elevated in GPA; conversely, C3 and C4 complement levels may be reduced.…”

A 12 MINUTE CONSULTATION: 12 minute consultation: a patient with nasal crusting

“…Involvement of the septum and turbinate together may indicate undisclosed cocaine abuse. The majority of septal perforations are anterior and will be detected on anterior rhinoscopy 16,29 . The site and size of the perforation should be determined, with particular attention paid to the regularity of the border, the presence of crusting, bleeding and granulation tissue at the perforation’s edges and the general appearance of the septum.…”

“…The range of investigations for nasal crusting is summarised in Table 2 4,5,7,10,29–33 . The aim is to detect serious underlying conditions and their systemic consequences.…”

“…The range of investigations for nasal crusting is summarised in Table 2. 4,5,7,10, [29][30][31][32][33] The aim is to detect seri-ous underlying conditions and their systemic consequences. As with all screening tests, sensitivity is often low and caution is therefore needed in their interpretation; a high clinical suspicion in a patient with an obviously destructive nasal lesion, significant nasal crusting or systemic illness should prompt onward referral for specialist investigation even in the absence of positive screening tests.…”