[Secondary non-tuberculous mycobacterium infection in patients with bronchiectasis caused by Marfan syndrome].

In this article, we reported a 28-year-old female patient who presented with intermittent hemoptysis, cough, and sputum production. Laboratory tests showed no abnormalities in the blood counts or inflammatory markers, and the sputum cultures were negative. A chest computed tomography scan showed bronchiectasis associated with infection in the middle and lower lobes of the right lung and right pleural thickening. We performed bronchoalveolar lavage by bronchoscopy in the dorsal segment of the right lower lobe and found Mycobacterium avium intracellulare complex (MAC) by Next Generation Sequencing (NGS) of bronchoalveolar lavage fluid (BALF). The patient's symptoms improved significantly after anti-mycobacterium treatment and the extent of infection was reduced on imaging. To further identify the cause of bronchiectasis, the patient is tall and thin, with slender limbs. Cardiac color ultrasound showed the widening of aortic sinus. Her genetic testing of blood samples revealed the gene mutation in the FBN1 gene (c.4349G>A). Based on these results, she was diagnosed with Marfan syndrome.

[Clinical features of 12 cases of respiratory diseases associated with inflammatory bowel diseases].

Objective: To investigate the clinical features of respiratory diseases associated with inflammatory bowel diseases(IBD). Methods: A retrospective analysis was performed for 12 cases of respiratory diseases associated with IBD admitted to Beijing Chaoyang Hospital from January 2011 to May 2017. Their clinical data, including symptoms, laboratory, spirometry, radiological findings as well as therapy and clinical course, were collected. Results: These 12 patients, including 11 males and 6 smokers, were 37-75 years old, with an average age of (61±12) years. There were 11 patients with ulcerative colitis(UC) and 1 with Crohn's disease(CD). The interval between the diagnosis of IBD and the occurrence of respiratory diseases ranged from 5 months to 30 years. The patients did not receive therapy for IBD when respiratory diseases were diagnosed. The respiratory diseases associated IBD included bronchiectasis (n=3), interstitial lung diseases (n=5, with 2 cases of organizing pneumonia by pathology), pulmonary embolism(n=3) and pulmonary arterial stenosis (n=1). Of the 12 cases, 9 presented with respiratory symptoms, including cough, expectoration and dyspnea. One patient presented with abdominal symptoms and 2 cases were found by physical examinations. Chest CT features were manifested as bronchiectasis, reticular patterns, patchy opacity and pulmonary artery filling defect. Conclusions: Respiratory diseases associated with IBD are various, which can involve airways, interstitium and pulmonary vessels. Respiratory diseases seem to be more common in patients with UC as compared to those with CD.

[IgG(4)-related disease involving the trachea and paratracheal soft tissue: a case report and literature review].

Objective: To investigate the clinical data of a patient with IgG(4)-related disease involving the trachea and paratracheal soft tissue and review the literature so as to improve the understanding level of the disorder. Methods: To analyze the clinical manifestation, laboratory examination, imaging, histopathology, treatment and prognosis of a patient with IgG(4)-related disease trachea and paratracheal soft tissue involved, who was admitted to the Department of Respiratory and Critical Care Medicine at Beijing Chaoyang Hospital. The relevant literatures were reviewed. Results: A 18-year-old female was admitted with chief complaint of cough, dyspnea, and neck mass. Neck CT suggested that tracheal stenosis was caused by surrounded soft tissue. Paratracheal mass biopsy showed dense collagen fibers with infiltration of many lymphocytes and plasma cells. Immunohistochemical stain found that IgG(4)-positive plasma cells were >50/high power field (HPF) and a ratio of IgG(4)/IgG positive cells was over 40% .The level of serum IgG(4) was significantly increased (2 930 mg/L). She was diagnosed as IgG(4)-related disease. The patient was treated with 80 mg intravenous methylprednisolone per day for three days, then prednisone 40 mg daily oral. Her dyspnea was significantly relieved.One month later, CT scan showed that the cervical tracheal stenosis was significantly improved. We identified 20 cases of IgG(4)-related disease involving the trachea and paratracheal soft tissue from databases, in which only 1 case was similar as this patient. The other 19 cases were of extratracheal involvement. Elevated serum IgG(4) was detected in 11/12 patients. Most patients were treated with glucocorticoid, some combined with immunosuppressive agents and rituximab. The clinical outcome was good. Conclusion: IgG(4)-related disease involving the trachea and paratracheal soft tissue is a rare condition. Serum IgG(4) level and histopathology should be considered for diagnosis. Glucocorticoid is effective.

Changes in the BODE index, exacerbation duration and hospitalisation in a cohort of COPD patients.

INTRODUCTION: We investigated the relationship between frequency of exacerbation and duration and change in functional status, as measured by the BODE index in chronic obstructive pulmonary disease (COPD) patients. METHODS: This was a longitudinal cohort study of 56 patients with moderate to severe COPD. Body mass index, spirometry, Modified Medical Research Council (MMRC) dyspnoea score and six-minute walk distance (6MWD) were measured annually when the patients were clinically stable. Data on frequency and duration of COPD exacerbations occurring in the community and requiring hospitalisation were collected prospectively. Early stage exacerbations were identified through the use of individualised patient action plans and further reinforced by fortnightly phone contact. RESULTS: At the two-year follow-up, the BODE index increased in 33 patients, remained stable in 18 and decreased in five patients. Patients with increased BODE index had significantly higher hospital presentation rates and longer total bed-days compared to those with stable BODE index. Among the 33 patients with increased BODE index, 20 had lower 6MWD and higher MMRC scores, indicating deteriorating functional status, and 13 had higher levels of airway obstruction. Between these two subgroups, patients with deteriorating functional status had higher exacerbation frequency, longer exacerbation duration and higher inpatient bed-days. Linear regression showed that total annual duration of exacerbation was predictive of change in 6MWD. CONCLUSION: Change in the BODE index is a sensitive measure of deteriorating functional status in COPD patients. Duration of exacerbation has greater impact on functional status than frequency of exacerbation episodes.