gms | German Medical Science

Background: Wegener’s granulomatosis is primary necrotizing ANCA associated systemic vasculitides with preferential involvement of small and medium-sized vessels.

In same case with isolated form the diagnosis of Wegener’s granulomatosis may be difficult with unreliable PR3- ANCA serology and ambiguous histological results.

Patients and methods: The files of all patients with is primary necrotizing ANCA associated systemic vasculitides (n=10) between 1998 and 2004 were evaluated with respect to clinical presentation, histological examinations and relevant laboratory data.

Results: PR3- ANCA positively is found in about 40% of the patients with localized Wegener’s granulomatosis. The histological pattern of organ involvement was positive in 33%. A localised form of Wegener’s granulomatosis had been present in 2 cases, the generalised in 8 cases. Most organ involvements occurred in the ear, nose, throat tract and kidney. Manifestinations occurred to a lesser extent in the skin and central nervous system (blindness of both eyes). Treatment followed the Fauci scheme.

Conclusion: The diagnosis of Wegener’s granulomatosis in isolated or generalised forms is most successful using the PR3-ANCA.