The Clinical Characteristics and Outcomes of Follicular Bronchiolitis in Chinese Adult Patients.

Follicular bronchiolitis (FB) is a rare interstitial lung disease (ILD) and has been reported in diverse clinical contexts. Six FB patients demonstrated by surgical lung biopsy (SLB) were reviewed between 2009 and 2017 from Nanjing Drum Tower Hospital in China. The average age of subjects was 42 years old (range: 31-55 years). The clinical symptoms were very mild. The laboratory findings showed elevated Erythrocyte sedimentation rate (ESR) and serum globulin and anemia. Pulmonary function tests were normal in four cases. Five cases had underlying diseases, such as, Sjo¨gren's syndrome, multi-centric castlemans' disease, idiopathic pneumonia with autoimmune features and abscess. Five cases presented as interstitial lung disease (ILD) on chest imaging with centrilobular or peribronchiolar nodules, ground glass opacities, interlobular septal thickening, cysts and bronchiectasis. Isolated mass was in one patient. The histopathology suggested the changes of FB in all subjects. Prednisone and/or cyclophosphamide were used in four cases, one did the surgery and the other was clinically monitored. All cases were alive at the end of follow up. The adult patients of FB usually have mild symptoms, ILD and underlying diseases. The definite diagnosis needs SLB. The prognosis is depended on their underlying conditions.

[Surgical Case of the Composite Tumor of Primary Lung Cancer and Follicular Bronchiolitis].

A case was a 72-year-old female who was pointed out abnormal findings on her chest X-ray taken at the medical checkup. Chest computed tomography revealed a tumor with 3.1 cm in diameter at the right lower lobe and multiple small nodules in both lung lobes. We initially suspected lung cancer with intrapulmonary metastases. However, only tumor at the right lower lobe increased in size and the size of other small nodules was not changed. Therefore, we performed the right lower lobectomy with mediastinal nodal dissection and the partial resection of right middle lobe to establish the diagnosis. Pathological assessment revealed that the tumor at right lower lobe was adenocarcinoma with follicular bronchiolitis, and small nodules in the lower lobe were intrapulmonary lymph nodes, and the nodules in the middle lobe were lymph node infiltration with follicular bronchiolitis. The composite tumor of primary lung cancer and follicular bronchitis is very rare, and the cancer stage could be overdiagnosed.

Lung transplantation for diffuse panbronchiolitis: 5 cases from a single centre.

Diffuse panbronchiolitis is a rare complex genetic disease predominantly affecting East Asians, and is characterized by chronic inflammation of the respiratory bronchioles and sinobronchial infection. Although long-term macrolide therapy has been shown to significantly improve the survival in patients with diffuse panbronchiolitis, some patients continue to deteriorate, eventually requiring lung transplantation. However, lung transplantation for diffuse panbronchiolitis has rarely been reported and the outcome in these patients remains unknown. We describe our experience of lung transplantation for diffuse panbronchiolitis. A total of 5 patients received long-term macrolide therapy and had airway colonization by Pseudomonas aeruginosa preoperatively. Three patients had undergone sinus surgery for chronic rhinosinusitis before the transplantation. Bilateral cadaveric lung transplantation was performed in 4 patients, and living-donor lung transplantation in 1. After the lung transplantation, 1 patient developed an A3 acute rejection episode; however, none of the recipients developed severe pneumonia or any fatal infections. One recipient developed chronic lung allograft dysfunction 3 years after the transplantation; however, none developed recurrence of diffuse panbronchiolitis. All of the 5 patients were still surviving after a median follow-up period of 4.9 years (3.7-12.3 years). Lung transplantation is a viable option for the treatment of progressive diffuse panbronchiolitis resistant to long-term macrolide therapy.

A case of diffuse panbronchiolitis, associated with severe pulmonary hypertension, managed with bilateral lung transplantation from a brain-dead donor.

Diffuse panbronchiolitis (DPB) is a chronic respiratory disease that mainly involves the respiratory bronchioles, and has historically been associated with a very poor prognosis. The development of long-term low dose macrolide therapy in the 1980s has dramatically improved the prognosis of DPB. Nevertheless, some cases are resistant to macrolide therapy, and ultimately develop severe respiratory failure and pulmonary hypertension; in such cases lung transplantation is a viable treatment option. Here we report the case of a 40-year-old patient with a 20-year history of DPB, who underwent bilateral lung transplantation due to severe respiratory failure with pulmonary hypertension.

Follicular bronchiolitis, an unusual cause of haemoptysis in giant cell arteritis.

The occurrence of follicular bronchiolitis (FB), lymphoid hyperplasia of the bronchus-associated lymphoid tissue, is reported in association with several systemic rheumatic diseases. However, the occurrence of FB in patients with giant cell arteritis (GCA) is not described. A 64-year-old man with long-standing GCA subsequently presented with haemoptysis. A mass in the lower lobe of the left lung was seen on chest x-ray and computed tomography. A wedge resection was performed, and histological examination revealed bronchiectasis, granulation tissue and hyperplasia of peribronchiolar lymphoid aggregates, consistent with FB. FB should be included in the differential diagnosis of haemoptysis in patients with systemic rheumatic diseases.

[Effects of ESS on sinobronchial syndrome].

Sinobronchial syndrome (SBS) is characterized by chronic sinusitis with chronic infection of the bronchus. Chronic bronchitis, bronchiectasis and diffuse panbronchiolitis are also examples of complicating lower airway disease. In Japan, the surgical treatment of sinusitis is not considered to improve the lower airway diseases. Most published reports describe the Caldwell-Luc method. However ESS (Endoscopic Sinus Surgery) can heal sinusitis while maintaining the morphology and function of the paranasal sinus. In addition, its surgical results are satisfactory. This report describes the improvement of patients with SBS who underwent ESS. Twelve patients with sinobronchial syndrome who underwent ESS at our department between 1989 and 1993 were enrolled in the study. Subjective improvement in sinusitis and the lower airway diseases were evaluated using a questionnaire. Objective improvements in sinusitis were evaluated using endoscopic findings of the ethmoid sinuses, while objective improvements in the lower airway diseases were evaluated by measuring the vital capacity and FEV 1.0%. These examinations were performed one year after the operation; the results of long-term follow-up examinations performed more than 6 years after the operation were also studied. In all of the patients, subjective nasal symptoms and objective endoscopic findings were satisfactory, with improvement rates of 91.7% and 83.3%, respectively. Regarding the lower airway symptoms, all the patients subjectively rated the symptoms as having improved, and an improvement in the FEV 1.0% was improved in all of the cases. In the present study, the lower airway symptoms improved after ESS. ESS enables the paranasal sinuses to be treated while maintaining the morphology of the paranasal sinus. Consequently, surgical results are better. The improvement of chronic sinusitis reduces the direct inflow of postnasal drippings into the bronchus. Restoring the normal functions of the nasal sinus defends the lower airway by warning, and humidifying the inspiratory air and removing dusts; these functions are though to have a favorable effect on the lower airway.

Alarming hypoxemia during one-lung ventilation in a patient with respiratory bronchiolitis-associated interstitial lung disease.

PURPOSE: To report a patient with respiratory bronchiolitis-associated interstitial lung disease (RB-ILD) who developed severe hypoxemia during one-lung ventilation (OLV). CLINICAL FEATURES: A 27-yr-old female, ex-smoker presented with productive cough and dyspnea of 18-month duration. The chest x-ray revealed diffuse abnormalities involving both lungs consisting of interstitial emphysema with irregular shadowing. Preoperative PaO(2) was 88 mmHg and pulmonary function tests showed moderate obstructive disease. The patient underwent right open lung biopsy. After induction of anesthesia, a left double lumen tube was inserted and its position verified with auscultation and fibreoptic bronchoscopy. Upon initiation of OLV, the patient developed severe hypoxemia and the PaO(2) dropped from 500 mmHg during two-lung ventilation (TLV) to 50 mmHg. Hypoxemia was readily corrected by resuming TLV. CONCLUSION: The severe hypoxemia during OLV in this patient with RB-ILD may be attributed to impaired hypoxic pulmonary vasoconstriction. Other causes were not excluded. Caution is warranted when initiating OLV in these patients.

Successful lung volume reduction surgery in a child with severe airflow obstruction and hyperinflation due to constrictive bronchiolitis.

Lung volume reduction surgery (LVRS) may improve pulmonary function in patients with severe emphysema. However, its effects in other types of obstructive lung disease are unknown. To delay the need for lung transplantation, we performed LVRS in a 14-year-old boy with disabling airflow obstruction/hyperinflation secondary to postinfectious bronchiolitis nonresponsive to medical therapy. Within days after LVRS, a major improvement of symptoms and lung function occurred and persisted for > 1 year. Our observation suggests that LVRS may be a novel treatment option in selected patients with extreme hyperinflation even if the underlying disease is not emphysema.

Respiratory bronchiolitis: a clinicopathologic study in current smokers, ex-smokers, and never-smokers.

The clinical and pathologic features of 109 cases of respiratory bronchiolitis (RB) identified from review of 156 consecutive surgical lung biopsy specimens were studied. A total of 107 of the 109 cases (98%) occurred in smokers, including all 83 current smokers and 24 of 49 ex-smokers (49%). RB persisted in some patients for many years after stopping smoking, occurring in one third of patients 5 years after quitting, and in one patient 32 years afterwards. A correlation was found between degree of cytoplasmic pigmentation of macrophages and number of pack-years smoked and also between the presence of peribronchiolar fibrosis and number of pack-years. No correlation was found between pulmonary function test results and pathologic findings. A desquamative interstitial pneumonia-like reaction was observed in six individuals. One patient each with a desquamative interstitial pneumonia-like reaction and one with RB were diagnosed based on clinical findings with desquamative interstitial pneumonia and RB-associated interstitial lung disease, respectively. No histologic features distinguished desquamative interstitial pneumonia from a desquamative interstitial pneumonia-like reaction or RB-associated interstitial lung disease from RB. Five cases of variant RB were encountered that resembled RB except that macrophage cytoplasm lacked pigment. All occurred in never-smokers, and their significance is unknown. RB is an accurate histologic marker of cigarette smoking, and it may be found many years after smoking ceases. There are no reliable histologic features to distinguish RB-associated interstitial lung disease from RB or desquamative interstitial pneumonia-like reactions from desquamative interstitial pneumonia.

Long-term results of lung volume reduction surgery.

OBJECTIVE: Lung volume reduction surgery (LVRS) is effective in the short and intermediate term for the improvement of pulmonary function and subjective symptoms in selected patients with advanced emphysema. The purpose of this study was to examine the long-term functional results of LVRS and to investigate which subgroups would benefit in terms of long-term survival. METHODS: All records of the patients who underwent LVRS between 1994 and, 1998 at our hospital were reviewed. RESULTS: Eighty-eight consecutive patients underwent LVRS during the period. There were 62 men and 26 women with an average age of 56.1 years (range 34-72 years). Eleven patients with alpha1-antitrypsin deficiency were included. The perioperative mortality rate (<90 days) was 2.3% (n=2). Total lung capacity (7.5+/-0.3 l) and residual volume (4.8+/-0.3 l) at 3 years remained lower than baseline (9.2+/-0.2 l, 6.5+/-0.2 l, each) (P<0.001). The mean forced expiratory volume in 1 s (FEV(1)) at 3 years (0.86+/-0.08 l) was higher than baseline (0.78+/-0.02 l), but the difference did not reach statistical significance. The FEV(1) of the patients with alpha1-antitrypsin deficiency and of those with respiratory bronchiolitis returned to baseline at one year after LVRS and showed further deterioration. Overall survival rate at 5 years was 71.0% with the mean length of follow-up of 54.2 months. The survival difference was statistically significant between patients with preoperative FEV(1) >or=28.5% and those with FEV(1)<28.5% (P=0.0152). CONCLUSIONS: The improvement of total lung capacity and residual volume persisted long after the operation. Patients with alpha1-antitrypsin deficiency and those with bronchiolitis showed early deterioration of the lung function. Patients with higher preoperative FEV(1) had a survival benefit. The favorable long-term survival might justify LVRS for the treatment of selected patients with severe emphysema.

Respiratory bronchiolitis associated interstitial lung disease (RB-ILD) presenting with haemoptysis.

Respiratory bronchiolitis associated interstitial lung disease is an uncommon condition in current or ex-smokers. The presentation is non-specific, but haemoptysis is uncommonly reported in this condition. We report the case of a 25-year-old woman who presented with significant haemoptysis, dyspnoea, reduced transfer factor and normal clinical examination. In addition, a Medline literature search was performed to review the clinical features and prognosis of this disease. Other causes of haemoptysis were excluded with extensive investigation. The diagnosis was made on thoracoscopic lung biopsy. The patient had significant postoperative complications of prolonged air leak and hydropneumothorax requiring further surgery and prolonged hospital stay. Advice regarding smoking cessation was given. Her pulmonary physiology remains abnormal on follow up but symptoms have improved. Respiratory bronchiolitis-ILD may present with normal examination and radiology. Haemoptysis in this case may have been associated with the underlying disease but could have been incidental. Diagnosis, in general, requires lung biopsy. As in this patient, lung function does not appear to improve significantly on follow up.

[Pathologic-anatomic findings in surgical lung volume reduction of advanced emphysema]. / Pathologisch-anatomische Befunde bei der chirurgischen Lungenvolumenreduktion des fortgeschrittenen Emphysems.

Surgical lung volume reduction is a method for treatment of advanced pulmonary emphysema after all other therapeutic approaches have failed. 280 atypical lung resection specimen of 81 patients were examined pathologico-anatomically using routine stains. In all cases combinations of various forms of emphysema were found; in 65.8% there were also bullous changes (with > or = 1 cm diameter) focally. Acute bronchiolitis was seen in 41.8% of the specimens, an only slight (if at all) chronic bronchiolitis in 26.7% and bronchioloectasia in 45.4% of the cases. Focal intraalveolar aggregates of granulocytes were identified in 16.1%, and the process of permanent scarring resulting in "organised pneumonia" in 20.6% of the specimens. Occult neoplasms were found in 9.9% of the patients and specific changes in 27.2%. Bronchiolitis is relevant for postoperative prognosis and an indication for intensifying antibiotical and antiinflammatory therapy. Preoperative diagnostical procedures should be intensified to find out these patients. Inflammatory changes must be investigated in respect of etiology and pathogenesis.

[Successful treatment of diffuse aspiration bronchiolitis by cricopharyngeal myotomy].

A 62-year-old man was admitted to our hospital with complaints of coughing and fever. He had poliomyelitis at the age of 4 years, and had experienced difficulty in swallowing and chronic aspiration for the past 3 years. A chest roentgenogram and a high-resolution CT scan obtained on admission showed many small nodular shadows in the lower lung fields. Cricopharyngeal myotomy was done to decrease aspiration. The patient was then able to eat without aspiration, and the chest-roentgenographic and CT findings improved. Diffuse aspiration bronchiolitis was diagnosed from the clinical course and the radiographic findings. Cricopharyngeal myotomy can reduce the risk of aspiration in patients with diffuse aspiration bronchiolitis.

Recurrence of diffuse panbronchiolitis after lung transplantation.

Diffuse panbronchiolitis (DBP) is characterized by chronic inflammation of the upper and lower respiratory tract. DPB has been found almost exclusively in oriental populations. We describe the occurrence of a case of DPB in an African American patient who underwent bilateral sequential lung transplantation. Ten weeks after transplantation, DPB recurred in the lung allograft, with rapid and significant deterioration in graft function. Allograft function improved within a few weeks after beginning treatment with erythromycin. This early recurrence is suggestive of a systemic etiology of DPB. Although recurrence of other systemic diseases has been reported after lung transplantation, no previous patients have been reported with early functional deterioration based solely on disease recurrence.

Peristomal complications of paediatric tracheostomy.

Failure of decannulation after paediatric tracheostomy, once the underlying disorder has resolved, is almost always due to peristomal complications. Granulation tissue formation in the raw tissue of the stoma and its subsequent fibrosis requires removal (50 of the 293 tracheostomies from the Red Cross War Memorial Children's Hospital). It is suggested that this can be avoided by creating a formal skin-to-trachea stoma at the time of tracheostomy. Suprastomal depression of the anterior wall of the trachea (52/293) appears to be unavoidable when using standard tracheostomy tubes. Localised stomal site tracheomalacia and stenosis (numbers of this complication are unknown) results from damage to cartilage of the trachea either by incision or by necrosis from pressure of the tracheostomy tube. Trauma to the cartilage needs to be minimised by careful design of the tracheal incision. It is suggested that consideration should be given to creating a formal tracheostomy stoma for any paediatric tracheostomy that is likely to be required for more than a short period of time.