Clinical pathological of Wegener's granulomatosis in middle-aged and elderly patients / 中华老年医学杂志

ABSTRACT

Objective To study the clinical pathological features of Wegener's granulomatosis (WG) in middle-aged and elderly patients,and enhance understanding of this disease.Methods Totally 21 patients with WG (11 males,10 females,aged 45 to 76 years,mean age 58.1 years) in our hospial from February 1999 to July 2012 were selected.The clinical and pathological data of WG patients were retrospectively analyzed.34 biopsies including 2 autopsies from different organs were paraffin embedded and stained by hematoxylin and eosin and histochemistry.13 renal biopsies were all examined by immunofluorescence and electron microscope.Results The average time from the onset of clinical symptoms to the diagnosis was 5.3 months (from 24 days to 11.0 months).Eyes,nose and salivary glands were the most commonly involved parts at the beginning of Wegener's granulomatosis (52.4％,11 cases).The percentages of the skin,lung and renal involvement were 14.3％ (3 cases),81.0％ (17 cases) and 71.4％ (15 cases),respectively.Among 21 patients,18 patients were examined with anti-neutrophil cytoplasmic antibody (ANCA).c-ANCA was positive in 72.2 ％ patients (13 cases,13/18),p-ANCA was positive in 16.7％ patients (3 cases,3/18),and ANCA was negative in 11.1％ patients (2 cases,2/18).3 major pathological manifestations were observed7 kinds of vasculitis including capillaritis,acute vasculitis,chronic vasculitis,fibrinoid necrosis in vasculitis,necrotizing granulomatous vasculitis,non-necrotizing granulomatous vasculitis and cicatricial vascular changes; 4 kinds of granulomatous inflammation including scattered giant cells,palisading histiocytes,poorly formed granulomas and microabscess surrounded by granulomatousinflammation;2 kinds of parenchymal necrosis including geographic necrosis and microabscess.13 kinds of histopathologic features in 3 major manifestations were found from 2 autopsies,but various kinds histopathologic features presented in small biopsy samples.Minor manifestations such as diffuse pulmonary hemorrhage were found at the periphery of WG.Conclusions The wide variation and broad spectrum of pathologic features can occur in WG.Vasculitis,granulomatous inflammation and parenchymal necrosis are the most important histopathological features.The correct diagnosis of WG requires careful correlation of pathology with complicated clinical features.