ObjectiveTo investigate the clinical characteristics of polymyositis (PM)/dermatomyositis (DM) with acute interstitial pneumonia (AIP) as the presenting symptoms, and identify characteristics of such disease. MethodsA retrospective analysis was conducted on the hospitalized patients with PM/DM with AIP as the presenting symptoms, from October 2009 to June 2015 in the Departemnt of Respiratory Medicine, Guangzhou Institute of Respiratory Diseases. ResultsThey were two males and six females with a mean age of 54.8±7.5 years. The common clinical features included fever (8 cases), shortness of breath (8 cases), rapidly progressive exertional dyspnea (8 cases), dry cough (6 cases), decreased muscle strength (8 cases), and typical rash (7 cases). Electromyography showed neurogenic or myogenic leision in these 8 cases. Muscle biopsy revealed myositis in 7 cases. High resolution CT (HRCT) revealed widespread ground glass patterns in all patients. All patients received noninvasive positive pressure mechanical ventilation on the first hospital day. High dose of methylprednisolone or combination with intravenous cyclophosphamide were initiated on 2.3±1.4 hospital day. Six patients survived to hospital discharge and two patient died. ConclusionsThe most common symptoms in patients of PM/DM with AIP are shortness of breath, progressive exertional dyspnea, and dry cough. Typical rash is seen in most of the patients.The diagnosis can be established by combinating the characteristics of HRCT, electromyography and muscle biopsy. Earlier intervention with noninvasive positive pressure mechanical ventilation and immunosuppressive may improve clinical outcome in patients of PM/DM with AIP.
ObjectiveTo evaluate the effectiveness of different biopsy methods in the diagnosis of idiopathic interstitial pneumonia (IIP). MethodsA retrospective analysis was performed in patients hospitalized and diagnosed as IIP between January 2010 to December 2013 in the Department of Respiratory Medicine, the First Affiliated Hospital of Guangzhou Medical University. The patients were divided based on initial diagnoses. The performance of invasive examinations including both transbronchoscopic lung biopsy (TBLB) and surgical lung biopsy (SLB) and final diagnoses were recorded. ResultsThree hundred and ninety-one patients were included in the study with mean age of 62.5±11.9 years. There were 299 patients receiving TBLB, 43 patients receiving SLB, and 49 patients receiving non-invasive examinations. Among 299 patients who underwent TBLB, parenchymal tissue was not obtained in 132 (44.1%) but successfully obtained in 167 (55.9%) patients, with 79 (47.3% out of 167) clinically helpful. TBLB-based specific diagnoses were as follows:30 cases of non-specific interstitial pneumonia (NSIP), 22 cases of cryptogenic organizing pneumonia (COP), 18 cases of idiopathic pulmonary fibrosis (IPF), 4 cases of acute interstitial pneumonia (AIP), 2 cases of respiratory bronchiolitis-interstitial lung disease (RB-ILD) and lymphocytic interstitial pneumonia (LIP) for each, 1 case of desquamative interstitial pneumonia (DIP), respectively. Among 43 patients who underwent SLB, parenchymal tissue was obtained in all cases with 40(93.0% out of 43) pathologically diagnostic, including 20 cases of NSIP, 12 cases of IPF, 3 cases of COP and LIP for each, and 2 cases of RB-ILD and DIP for each. Finally, among 181 patients who failed to obtain parenchymal tissue or did not undergo invasive examination, specific diagnoses could be established in 40 patients with all finally diagnosed as IPF. ConclusionsThe pathological diagnosis, either based on TBLB or SLB, is very important in the diagnostic procedure of IIP. The biopsy technique of hospital and patient conditions should be considered when making the decision of biopsy method.