Clinical features of microscopic polyangiitis with pulmonary involvement

Li Fei; Xie Jiang; Shang Mingyu; Zhu Guangfa

doi:10.3760/j.issn.1673-4777.2019.04.017

Abstract：Objective To analyze the clinical characteristics of pulmonary involvement in patients with microscopic polyangiitis (MPA).Methods Clinical data of 60 cases of MPA with pulmonary involvement admitted to Beijing Anzhen Hospital,Capital Medical University from June 2009 to July 2018 were retrospectively reviewed.General information,clinical manifestations,laboratory results,chest CT,pulmonary function,renal pathology,therapeutic choice and outcomes were analyzed.Results There were 38 males and 22 females;the onset age was 40-88 years and the mean age was (69 ± 11) years.Pulmonary involvement was the initial manifestation in 30 patients.Common symptoms were cough and expectoration(42 cases),haemoptysis(13 cases) and dyspnea (24 cases).Positive rate of antimyeloperoxidase-antineutrophil cytopasmic antibody (ANCA) was 91.7％ (55/60).Positive rate of antiproteinase-3-ANCA was 11.7％ (7/60).Chest CT manifestations consisted of reticular markings(40 cases),ground glass opacity (35 cases),patchy infiltration (30 cases),pleural thickening (11 cases),pleural effusion(11 cases),nodules(8 cases)and usual interstitial pneumonitis(3 cases).The most frequent abnormality of lung function test was reduced diffusing capacity for carbon monoxide(24/25) and restrictive ventilation dysfunction (7/25).Incidence of pulmonary hypertension was 19.2％ (10/52) and the mean pulmonary arterial systolic pressure was (50 ± 10) mmHg.In 48 patients receiving medical treatments in hospital,34 patients had glucocorticoids plus cyclophosphamide and 14 patients were treated with glucocorticoids;17 patients had high dose methylprednisolone pulse therapy.Three patients died of pulmonary infection-induced respiratory failure and infectious shock;the other patients showed good therapeutic response.Conclusions Lung lesion is a common comorbidity and frequent initial manifestation in patients with MPA.Clinical manifestations are nonspecific;typical CT manifestations are ground glass opacity and pulmonary interstitial abnormality.Serum ANCA and histopathologic biopsy can help diagnose MPA with pulmonary involvement.