[Wegener's granulomatosis].

Wegener's granulomatosis (WG) is a multiorgan disease with a predilection for the upper airways (E: ear, eye, nose, throat) and lower airways (L: lung) and the kidneys (K):ELK classification. Typical histological features of WG include inflammation of small and medium sized vessels, necrosis and granuloma formation. The histological findings of WG are divided into 4 stages ie, I: degeneration II: acute inflammation III: granulation IV: scar. The recognition of the association between C (proteinase-3) antineutrophil cytoplasmic antibody (ANCA) and WG is the only tool available to aid in the assessment of disease activity, other than crude laboratory parameters of inflammation, such as, erythrocyte sedimentation rate and C reactive protein. Stage adapted immunosuppressive therapy (glucocorticoids and cyclophosphamide) has significantly altered the outcome of this once fatal disease.