Clinical And Pathological Features Analysis Of 25 Wegener's Granulomatosis Patients And Detecting EB Virus For Its Differential Diagnosis

Objectives1. To study the clinical, laboratory and pathological features of Wegener's granulomatosis(WG) and its response to therapy so as to improve our management.2. To evaluate the value of detecting EB virus (EBV) in upper respiratory tract tissue with lesion by PCR in differentiating WG from nasal T/NK-cell lymphoma.Methods1. Clinical data of 25 WG patients together with 20 biopsy specimens were reviewed. Nine patients were followed up.2. DNA was extracted from biopsy specimens with paraffin imbedding in upper respiratory tracts of eleven WG, sixteen nasal T/NK-cell lymphomas and fifteen chronic nasosinusitis or nasal polyps patients. EBV-DNA was detected in those by PCR.Results Parti:1. The patients' ages at the beginning of disease were from 13 to 72 years. The average was 38?6 years. The average course from initiation of clinical symptoms to diagnosis was 7.8 + 9.0 months.2. Multiple systems and organs involvements were found. Ear, nose and throat involvements were the most common(96.0%), followed by lung and kidney. The main clinical manifestations were specific symptoms in related systems and organs.3. Seven out of 12 were positive in ANCA(58.3%).4. Pathological manifestations included parenchymal necrosis, granulomas, multinucleated giant cells, microabscesses, vasculitisand so forth. Parenchymal necrosis was the most common manifestations in Ear, nose and throat. But in lung and subcutaneous tissue, vasculitis was the most dominant findings.5. Most patients' symptoms improved significantly after glucocorticoids and cyclophosphamide therapy.Part 2:1. The EBV-DNA positive rate was 10.0% in WG group, 60.0% in nasal T/NK-cell lymphoma group and 9.1% in chronic nasosinusitis or nasal polyps group respectively.2. The difference of positive rate between WG and nasal T/NK-cell lymphoma group has statistics significance (P=0.018) . It hasn't between WG and chronic nasosinusitis or nasal polyps group(P=1.0) . Conclusion1. WG is one of autoimmune diseases that can affect several systems and organs. The upper and lower respiratory tracts and kidneys are the most popular parts involved. The main pathological changes are necrotizing granuloma and small vessel vasculitis. But it has no absolutely specific pathological change. ANCA is positive in most active patients. Most patients have good response to glucocorticoids and cyclophosphamide and their prognosises are good.2. WG has no correlation with EBV infection. We can differentiate WG and nasal T/NK-cell lymphoma by detecting EBV-DNA in upper respiratory tract tissue with lesion.