Bronchiolitis ; epidemiology ; pathology ; therapy ; Bronchiolitis Obliterans ; epidemiology ; pathology ; therapy ; Child ; Haemophilus Infections ; epidemiology ; pathology ; therapy ; Humans

We report a patient who suffered from bronchiolitis obliterans organizing pneumonia(BOOP) after Ivor Lewis operation for esophageal cancer. The patient presented low grade fever, dry cough and mild dyspnea at 4 day after operation. Chest roentgenograms and chest CT revealed bilateral patchy and infiltrative shadows. The respiratory symptoms worsened and respiratory failure developed with mild elevation of WBC count despite of conservative treatment. An open lung biopsy was done and the biopsy specimen showed bronchiolitis obliterans organizing pneumonia(BOOP). After several weeks of steroid therapy, there were marked clinical, physiological and roentgenographic improvements. Our experience suggests that BOOP may be one of the underlying pathology in a number of patients presenting with ARDS after thoracotomy. Since steroid therapy may improve survival in these patients, thoracic surgeons should heighten their index of suspicion for this entity. Early histologic diagnosis should be considered in patients with treatment-resistant ARDS after thoracotomy.
Biopsy ; Bronchiolitis Obliterans* ; Bronchiolitis* ; Cough ; Cryptogenic Organizing Pneumonia* ; Diagnosis ; Dyspnea ; Esophageal Neoplasms ; Fever ; Humans ; Lung ; Pathology ; Respiratory Insufficiency ; Thoracotomy* ; Thorax ; Tomography, X-Ray Computed

OBJECTIVE: Critically ill patients with solid tumors complicated with paraneoplastic pemphigus are usually treated in intensive care units (ICU) for perioperative management after surgical treatment. In this study, the clinical characteristics and predictors of long-term prognosis of these critically ill patients were analyzed. METHODS: the clinical and laboratory data of 63 patients with solid tumors complicated with paraneoplastic pemphigus admitted to ICU from 2005 to 2020 were retrospectively analyzed, and the survival status of the patients were followed up. RESULTS: Among the 63 patients, 79.4% had Castleman disease as the primary tumor, and 20.6% with other pathological types; 69.8% had severe-extensive skin lesions, and 30.2% had other skin lesions; the patients with bronchiolitis obliterans accounted for 44.4%, and 55.6% were not merged. Postoperative fungal infection occurred in 23.8% of the patients, and 76.2% without fungal infection. The median follow-up time was 95 months, and 25 patients died during the study period. The 1-year, 3-year and 5-year survival rates were 74.6% (95%CI 63.8%-85.4%), 67.4% (95%CI 55.6%-79.2%) and 55.1% (95%CI 47.9%-62.3%), respectively. The log-rank univariate analysis showed that the patients had age>40 years (P=0.042), preoperative weight loss>5 kg (P=0.002), preoperative albumin < 30 g/L (P < 0.001), paraneoplastic pemphigus complicated with bronchiolitis obliterans (P=0.002), and perioperative fungal infection (P < 0.001) had increased mortality. Cox univariate analysis showed that preoperative weight loss >5 kg (P=0.005), preoperative albumin < 30 g/L (P < 0.001), paraneoplastic pemphigus complicated with bronchiolitis obliterans (P=0.009), preoperative bacterial pulmonary infection (P=0.007), prolonged surgical time (P=0.048), postoperative oxygenation index (P=0.012) and low albumin (P=0.010) and hemoglobin concentration (P=0.035) in ICU, acute physiology and chronic health evaluation (APACHE Ⅱ) score (P=0.001); sequential organ failure assessment (SOFA) score (P=0.010), and postoperative fungal infection (P < 0.001) were risk factors for long-term survival. Cox regression model for multivariate analysis showed that preoperative weight loss > 5 kg (HR 4.44; 95%CI 1.47-13.38; P=0.008), and preoperative albumin < 30 g/L (HR 4.38; 95%CI 1.72-11.12; P=0.002), bronchiolitis obliterans (HR 2.69; 95%CI 1.12-6.50; P=0.027), and postoperative fungal infection (HR 4.85; 95%CI 2.01-11.72; P < 0.001) were independent risk factors for postoperative mortality. CONCLUSION The 5-year survival rate of critically ill patients undergoing surgery for paraneoplastic pemphigus combined with solid tumors is approximately 55.1%, with preoperative weight loss > 5 kg, albumin < 30 g/L, bronchiolitis obliterans and postoperative fungal infection were associated with an increased risk of near- and long-term postoperative mortality.
Adult ; Albumins/therapeutic use* ; Bronchiolitis Obliterans/pathology* ; Critical Illness ; Hemoglobins ; Humans ; Neoplasms/complications* ; Paraneoplastic Syndromes/pathology* ; Pemphigus/drug therapy* ; Retrospective Studies ; Weight Loss

OBJECTIVETo recognize the clinical features of the bronchiolitis obliterans.

METHODClinical manifestation, chest X-ray, computed tomography (CT) and pulmonary function of 4 cases with bronchiolitis obliterans were retrospectively analyzed.

RESULTTwo cases were after Stevens-Johnson syndrome (SJS), the other 2 were after severe pneumonia, including one suffered from adenovirus pneumonia. Cough, tachypnea and wheezing persisted in all the 4 patients. The symptoms lasted for at least 6 weeks, in one case for over one year. Crackles and wheezing were present in all the 4 cases. Hyperinflation was seen in chest radiographs in all cases. On pulmonary CT/high-resolution CT (HRCT), patchy opacity and bronchial wall thickening were seen in each patient. Areas of air trapping were seen in three cases. Bronchiectasis was seen in 2 cases, atelectasis and mosaic perfusion were seen respectively in one case. PO(2) was low in all the four cases. Wheezing was not responsive to beta(2) agonist and other bronchodilating therapy. Prednisone was used at a dose of 1 mg/(kg.d) in 3 cases. Two cases were followed up for 3 months. The clinical condition of one case was improved, whose wheezing and bronchiolar constriction disappeared, cough and dyspnea were also relieved. However, the condition of one patient was not improved, although the wheezing disappeared. The HRCT of these two cases showed no improvement.

CONCLUSIONClinical symptoms of BO were cough, tachypnea, and wheezing after acute lung injury. Crackles and wheezing were the most common signs in the BO. Chest radiographs showed hyperinflation. Pulmonary CT showed bronchial wall thickening, bronchiectasis, atelectasis, and mosaic perfusion. Pulmonary function tests suggested obstruction of small airway.

Bronchiolitis Obliterans ; etiology ; pathology ; physiopathology ; Child ; Child, Preschool ; Humans ; Infant ; Male ; Pneumonia ; complications ; Pneumonia, Viral ; complications ; Prognosis ; Respiratory Function Tests ; Stevens-Johnson Syndrome ; complications ; Tomography, X-Ray Computed

PURPOSE: Interstitial lung disease(ILD) is a rare and poorly characterized disorder in children with poor prognosis. To understand the ILD in children, we reviewed our experience with 21 patients who were diagnosed interstitial lung disease during 9-year period at Asan Medical Center retrospectively. METHODS: Severity-of-illness score was measured by the Denver protocol. We evaluated underlying diseases, clinical manifestations, high resolution computed tomography findings, lung pathology and clinical responses after steroid therapy and prognosis. Fifteen patients were performed open lung biopsy, but six patients were diagnosed bronchiolitis obliterans by HRCT. RESULTS: The median ages at onset of ILD was 1 year 5 month old. Common clinical findings were tachypnea(90.0%), hypoxemia(90.0%). 14 patients among 15 patients were diagnosed specifically after open lung biopsy. Interstitial pneumonitis was 6 cases, including 3 nonspecific interstitial pneumonitis, 2 cases of desquamative interstitial pneumonitis and one usual interstitial pneumonitis. Other diagnosis included idiopathic pulmonary fibrosis, cytomegalovirus pneumonitis, diffuse aspiration bronchiolitis, pulmonary lymphangiomatosis, BOOP(bronchiolotis obliterans organizing pneumonia) and pulmonary histiocytosis. Six patients showed clinical and HRCT findings consistent with bronchilitis obliterans. Common radiologic findings were mosaic perfusion(12/21), bronchial wall thickening(9/21). 13 patients among 15 patients with methylprednisolone pulse therapy showed improvement of clinical symptoms. The severity-of-illness score was improved significantly after methylprednisolone pulse therapy. CONCLUSION: Pediatric ILD includes heterogeneous disorders. Open lung biopsy was helpful to make early diagnosis. Severity-of-illness score is a noninvasive and useful measure of disease progression or response to therapy. Methylprednisolone pulse therapy may be effective to treat ILD.
Biopsy ; Bronchiolitis ; Bronchiolitis Obliterans ; Child* ; Chungcheongnam-do ; Cytomegalovirus ; Diagnosis ; Disease Progression ; Early Diagnosis ; Histiocytosis ; Humans ; Idiopathic Pulmonary Fibrosis ; Infant ; Lung Diseases, Interstitial* ; Lung* ; Methylprednisolone ; Pathology* ; Pneumonia ; Prognosis ; Retrospective Studies

Anti-Inflammatory Agents ; therapeutic use ; Bronchi ; pathology ; Bronchiolitis Obliterans ; pathology ; Humans ; Lung ; pathology ; Male ; Methylprednisolone ; therapeutic use ; Middle Aged ; Pneumonia ; pathology ; Pulmonary Fibrosis ; complications ; drug therapy ; pathology ; Respiratory Distress Syndrome, Adult ; etiology

A case of dermatomyositis presented as bronchiolitis obliterans organizing pneumonia has been rarely reported. We describe a 46-year-old female patient with dermatomyositis without elevation of creatine kinase presented as bronchiolitis obliterans organizing pneumonia. She was treated with prednisolone and azathioprine. Over a 2-year follow-up she has had no elevation of creatine kinase. The patient remains asymptomatic and has no medication for dermatomyositis and bronchiolitis obliterans organizing pneumonia two years after initial treatment. It has been suggested that the prognosis of dermatomyositis without creatine kinase elevation may be poor. Because the prognosis of bronchiolitis obliterans organizing pneumonia is generally believed to be good, we tentatively suggest that the normal value of creatine kinase in dermatomyositis does not always seem to herald a poor prognosis, an associated malignancy or severe interstitial lung disease.
Azathioprine/administration +ACY- dosage ; Biopsy, Needle ; Bronchiolitis Obliterans Organizing Pneumonia/pathology ; Bronchiolitis Obliterans Organizing Pneumonia/diagnosis+ACo- ; Case Report ; Creatine Kinase/blood+ACo- ; Dermatomyositis/pathology ; Dermatomyositis/enzymology ; Dermatomyositis/drug therapy ; Dermatomyositis/diagnosis+ACo- ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Human ; Middle Age ; Prednisone/administration +ACY- dosage ; Tomography, X-Ray Computed

A case of inflammatory pseudotumor of the lung occurring in a six-year-old boy is reported with clinicopathologic findings, including its ultrastructure. The patient had had frequent upper respiratory tract infections, and one and half year before the discovery of the lung mass, he suffered from pneumonia of the right lung, which was serologically proven to be a mycoplasma pneumoniae infection. Exploratory thoracotomy revealed a large mediastinal mass that was removed together with the right middle and lower lobes of the lung. The mass arose from the lung with an endobronchial element. Microscopically, the mass was composed of a variety of inflammatory and mesenchymal cells, including plasma cells, histiocytes, lymphocytes, and fibroblast-like spindle cells. Ultrastructurally, the spindle-shaped mesenchymal cells were either fibroblasts or myofibroblasts. At the time of diagnosis of the inflammatory pseudotumor of the lung, the serum titer of antimycoplasma antibody rose again, and the lung parenchyma adjacent to the mass showed interstitial pneumonia with features of bronchiolitis obliterans. The present case suggests that the inflammatory pseudotumor of the lung could be a postinflammatory lesion associated with mycoplasma pneumoniae infection.
Antibodies, Bacterial/blood ; Bronchiolitis Obliterans/complications ; Child ; Diagnosis, Differential ; Fibroma/etiology/*pathology/surgery ; Humans ; Lung Neoplasms/etiology/*pathology/surgery ; Male ; Mycoplasma pneumoniae/immunology/*pathogenicity ; Pneumonia, Mycoplasma/complications/microbiology/*pathology ; Tomography, X-Ray Computed

BACKGROUNDBronchiolitis obliterans syndrome (BOS) often develops in transplant patients and results in injury to the respiratory and terminal airway epithelium. Owing to its rising incidence, the pathogenesis of BOS is currently an area of intensive research. Studies have shown that injury to the respiratory epithelium results in dysregulation of epithelial repair. Airway epithelial regeneration is supported by stromal cells, including fibroblasts. This study aimed to investigate whether the supportive role of lung fibroblasts is altered in BOS.

METHODSSuspensions of lung cells were prepared by enzyme digestion. Lung progenitor cells (LPCs) were separated by fluorescence-activated cell sorting. Lung fibroblasts from patients with BOS or healthy controls were mixed with sorted mouse LPCs to compare the colony-forming efficiency of LPCs by counting the number of colonies with a diameter of ≥50 μm in each culture. Statistical analyses were performed using the SPSS 17.0 software (SPSS Inc., USA). The paired Student's t-test was used to test for statistical significance.

RESULTSLPCs were isolated with the surface phenotype of CD31-CD34-CD45- EpCAM+Sca-1+. The colony-forming efficiency of LPCs was significantly reduced when co-cultured with fibroblasts isolated from patients with BOS. The addition of SB431542 increased the colony-forming efficiency of LPCs to 1.8%; however, it was still significantly less than that in co-culture with healthy control fibroblasts (P < 0.05).

CONCLUSIONThe epithelial-supportive capacity of fibroblasts is impaired in the development of BOS and suggest that inefficient repair of airway epithelium could contribute to persistent airway inflammation in BOS.

Animals ; Benzamides ; pharmacology ; Bronchiolitis Obliterans ; metabolism ; pathology ; Cells, Cultured ; Coculture Techniques ; Dioxoles ; pharmacology ; Fibroblasts ; cytology ; drug effects ; metabolism ; physiology ; Flow Cytometry ; Humans ; Mice ; Stem Cells ; cytology ; drug effects ; metabolism

Bronchiolitis obliterans syndrome (BOS) after hematopoietic stem cell transplantation (HSCT) is a major cause of morbidity and mortality with limited treatment options. Lung transplantation (LTX) has been rarely reported as a treatment option for selected HSCT recipients with this problem. In the present study, we reported six patients who underwent LTX due to BOS after HSCT (two females, four males) from January 2012 to December 2014 in our center. The median time from HSCT to diagnosis of BOS was 2.5 years (ranging from 1 to 5 years). At a median time of 4 years (ranging from 2 to 5 years) after diagnosis of BOS, four patients received bilateral sequential LTX, and two patients received single LTX. One of the recipients suffered from mild acute rejection after LTX, another suffered from primary lung graft dysfunction on post-operation day 2, and three experienced fungal infections. The median time for follow-up after LTX was 19.5 months (ranging from 12 to 39 months). At present, all patients are alive with good functional capacity and no relapse of BOS and hematologic malignancy conditions. Patients who received bilateral LTX have better pulmonary functions than patients who received single LTX.
Adolescent ; Adult ; Bronchiolitis Obliterans ; etiology ; surgery ; Female ; Graft Rejection ; Hematopoietic Stem Cell Transplantation ; adverse effects ; Humans ; Lung ; diagnostic imaging ; pathology ; Lung Transplantation ; Male ; Mycoses ; Tomography, X-Ray Computed ; Young Adult