A Report Of30Cases Of ANCA Associated Small Vasculitis Performance In Lung Diseases

Objective：To study the clinical manifestations and characteristics ofanti-neutrophil cytoplasmic antibodies (ANCAs) associated small vasculitis(AASV) in lung diseases. Moreover, providing support for the front-lineclinical doctors with diagnosis and treatment of AASV patients timely.Methods:A retrospective analysis of the clinical data of30patients diagnosed with AASV (including Wegener granulomatosis、microscopic polyangiitis and Churg-strauss Syndrome) at the Second Hospitalof Jilin University form January2007to April2011. Comparison of generalinformation, clinical manifestations, laboratory tests, pulmonary imagingfindings, pathology, efficacy, and get the correlation analysis throughcombining with the literature in recent years.Results:The patients age24to77years old, and the average age is59.06±2.40.18patients are females, with the mean age of62.59±3.45years old,and the others are males, with the mean age of54.34±2.92years old. Theduration is from2weeks to2years for different patients.12out of30caseshave rapid-onset. Pure with respiratory symptoms as the initial symptom in7cases, with respiratory symptoms and fever as the first13cases, to urologicalsymptoms as initial symptoms of8cases, and the digestive symptoms2cases.There are8cases of Wegener granulomatosis (WG),22cases ofmicroscopic polyangiitis (MPA), without Churg-strauss Syndrome (CSS) cases.Neutrophils increased in17cases;23cases have lower hemoglobin, whichcombined with renal abnormalities in15cases; erythrocyte sedimentation rate were increased in21cases; C-reactive protein grow up in19patients; arterialblood gas analysis and respiratory failure in9cases, were associated withimpairment of lung diffusion function. Imaging findings of: lesion site wide,with bilateral involvement was seen, most in lower lung field, a few in the lung,but no obvious specificity. After tne therapy of via glucocorticoid combinedimmunosuppressive, most lesions can be gradually absorbed. In30patients,8cases have similar complaints, symptoms and imaging findings as idiopathicinterstitial pneumonia. Moreover,3cases were misdiagnosed as IPF and takingprednisone for several months. In8cases there are3cases get lung biopsy, andin which2cases show endoscopic manifestations of herpes infiltration ofinflammatory cells, and1case performed as cryptogenic organizing pneumonia.In30patients a total of7deaths, of which6died of respiratory failure,1casesof severe circulatory function, renal insufficiency, active treatment ineffective,and eventually died of multiple organ failure. The first treatment after partialremission in16cases, not relieved in1cases,6cases diagnosed after transfer toother hospitals to continue treatment,2cases due to uremia hemodialysistreatment for law.Conclusions:Of all ages are likely to be suffered with AASV. Theincidence rate of MPA is about3times of the one of WG, the higher prevalenceof MPA may be one of the characteristics of AASV in China. WG patients weremore likely had respiratory symptoms than MPA, and MPA patients were morelikely involved kidney than the WG. Imaging has no specific changes, but oftenmore critical than clinical manifestations. The lung pathology of AASV can beexpressed as idiopathic pulmonary fibrosis. As the improvement of means ofinspection, AASV has a increasing incidence. Varied the clinical presentation is,extensive the lesions are. Lung and kidney are the most vulnerable organ whichAASV affected. So for long-term fever and multiple organ damaged in patients should be checked as soon as possible with ANCA diagnosis, timely treatmentand improve prognosis.