Granulomatosis Polyangiitis with Diffuse Alveolar Hemorrhage：a Case Report and Literature Review

Abstract

Background：Granulomatous polyangiitis(GPA)is a rare systematic disease that mainly affects the upper respiratory tract,lung and kidney.Diffuse alveolar hemorrhage(DAH)is a rare manifestation of the disease.Once it occurs,the situation is critical with a high mortality rate.Objective: To investigate the clinical manifestations,laboratory and imaging features,diagnosis and treatment as well as prognosis of GPA with DAH,enhancing clinicians' understanding of this disease to perform a diagnosis and deliver appropriate treatment early to improve the patient prognosis.Methods: The clinical data of a GPA patient with DAH admitted to Department of Rhrumatology and Immunology,Ruijin Hospital,Shanghai Jiao Tong University School of Medicine,was reviewed and analyzed.Besides,publicly published reports on GPA or GPA with DAH during January 1980 to February 2019 were retrieved from electronic databases of Wanfang Data Knowledge Service Platform,CNKI,and PubMed using "Wegener,vasculitis,granulomatous polyangiitis,pneumorrhagia,and alveolar hemorrhage" as keywords.Results: 12 cases were reported,combined with our case,there were 13 cases of GPA with DAH,including 7 males and 6 females,with an average age of(38.6±19.3)years,and an average duration of(72.1±62.7)days.Most of them were treated with high-dose intravenous corticosteroid pulse therapy combined with cyclophosphamide.The rate of mortality was 23.1%(3/13).Commonly seen clinical manifestations were cough(83.3%),dyspnea(83.3%),and hemoptysis(42.7%).Chest X-rays or CT scans showed multiple patches,ground-glass opacities or bilateral diffuse alveolar infiltrates and consolidations.Bronchoscopy may show airway hemorrhage,and elevated number of hemosiderin-laden macrophages could be found in bronchoalveolar lavage fluid.Conclusion: DAH is a rare manifestation of GPA,characterized mainly by dyspnea,cough,hemoptysis,rapid progress,and high mortality rate.Early treatment may improve the prognosis.