Idiopathic bronchiolitis obliterans with organizing pneumonia (BOOP) is a specific clinicopathologic syndrome characterized by a pneumonia-like illness, with excessive proliferation of granulation tissue within bronchioles, alveolar ducts and alveoli. The changes are most numerous in alveolar ducts. The presence of intraluminal tufts of organizing connective tissue in alveolar ducts and more distal airspaces has been termed organizing pneumonia The radiologic manifestations are distinctive with bilateral, diffuse alveolar opacities predominantly in the subpleural and lower lung zone. Patchy migratory pneumonic foci or solely nodular infiltrates are rarely present in BOOP. BOOP is a diagnosis of importance because of its dramatic response to steroids.
Bronchioles ; Bronchiolitis Obliterans ; Connective Tissue ; Cryptogenic Organizing Pneumonia* ; Diagnosis ; Granulation Tissue ; Lung ; Pneumonia ; Steroids

Bronchiolitis obliterans organizing pneumonia (BOOP) is a pulmonary disorder that exhibits various radiographic findings. It often shows bilateral, patched, or ground glass interstitial infiltrate, but a solitary nodular pattern rarely appears. We report a case of an 8-year-old boy suffering from BOOP that showed a single nodular pattern of the lung in the chest radiography. We conclude that when there is a solitary nodule discovered in the lungs of children or adolescence, the differential diagnosis must include BOOP.
Adolescent ; Bronchiolitis ; Bronchiolitis Obliterans ; Child ; Cryptogenic Organizing Pneumonia ; Diagnosis, Differential ; Glass ; Humans ; Lung ; Solitary Pulmonary Nodule ; Stress, Psychological ; Thorax

BACKGROUND & METHOD: Bronchiolitis obliterans organizing pneumonia(BOOP) is a specific clinicopathologic condition characterized by chronic inflammatory interstitial infiltrates. Cryptogenic form of BOOP presents subacute clinical course of flu-like illness, such as cough, fever, dyspnea with exertion and other constitutional symptoms. Pathologically it shows the presence of granulation tissue filling the lumen of terminal and respiratory bronchioles, extending into distal airspaces. Recently, we reviewed 24 cases of idiopathic type of BOOP, 5 cases of our hospital and another 19 cases on Korean literatures, and compared with reviewed data from foreign literatures. RESULTS: Mean age was 54 years old and there was female preponderance in domestic reports. Their common presenting symptoms were dyspnea and cough, and mean duration of illness was 41 days. On chest examination, inspiratory crackle was a common finding. The laboratory findings were nonspecific except hypoxemia. Lung function studies revealed restrictive defect or combined obstructive and restrictive pattern in most patients. Bilateral patchy and nonsegmental alveolar opacities constituted characterized radiographic finding, highlighted on high resolution computed tomogram. It showed a favorable prognosis with an excellent responsiveness to corticosteroid therapy. The clinical features and laboratory findings were similar between domestic and foreign cases except female preponderance in Korean cases. CONCLUSION: If the clinical course is atypical or pregressive under proper treatment, clinicians should reevaluate clinical features and radiographic findings under the consideration of BOOP. Tissue confirmation is recommended for the definitive diagnosis of BOOP.
Anoxia ; Bronchioles ; Bronchiolitis Obliterans* ; Bronchiolitis* ; Cough ; Cryptogenic Organizing Pneumonia* ; Diagnosis ; Dyspnea ; Female ; Fever ; Granulation Tissue ; Humans ; Korea* ; Lung ; Middle Aged ; Prognosis ; Respiratory Sounds ; Thorax

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

BACKGROUND: Idiopathic pulmonary fibrosis(IPF) is a fatal progressive fibrous disease of the lung of unknown etiology. Recently it has been classified into several distinct entities of the basis of pathologic and clinical characteristics, ie : usual interstitial pneumonia(UIP), desquamative interstitial pneumonia(DIP), acute interstitial pneumonia(AIP), bronchiolitis obliterans with organizing pneumonia(BOOP), and nonspecific interstitial pneumonia(NSIP). IPF is now applied only for UIP, which has the worst prognosis. The previous reports of 3-5 year median survival apears to be overoptimistic because other types with better prognosis like NSIP or BOOP might have been included. Therefore, this study was performed to determine the clinical course and the prognostic factors of UIP as diagnosed by surgical lung biopsy. METHODS: The subjects were 72 UIP patients (age 58.2±11.6 years, M:F=45:27, median follow up period:18.1 months (0.7-103.6) diagnosed by surgical lung biopsy at the Asan Medical Center (68 patients) and the Paik Hospital in Seoul (4 patients). Clinical scores (level of dyspnea:1-20 points), radiologic score (honey-combing : HC score 0-5 points, ground glass : GG score 0-5 points), and physiologic scores (FVC:1-12 points, FEV1:0-3 points, TLC:0-10 points, DDLC:0-5 points, AaDO2:0-10 points) were summed into a total CRP score. RESULTS: 1) The one year survival rate was 78.3%, while the rate for three year survival was 58.1%, and the median survival period was 42.5 months. 2) Short term (1 year) prognosis : The patients who died within one year of diagnosis (14 patients) had the higher initial total CRP score (28.6±8.3 vs. 16.6±9.7) than those who lived longer than one year (46 patients). The difference in the total CRP score was attributed to the symptom score (8.4±2.1 vs. 5.7±3.9) and the physiologic score (15.7±7.1 vs. 6.7±5.7) including FVC, DLCO, and AaDO2. 3) Long-term (3 year) prognosis : The total CRP score (12.2±6.7 vs. 28.7±7.9:including symptom score, FVC, DLCO, and AaDO2) at the time of diagnosis were also different for the long-term survivors and those who lived less than 3 years. 4) Cox regression analysis showed LCO (≥60%) (Hazard ratio:4.56, 95% CI:2.30-16.04) was the independent prognostic factors of UIP (P<0.05). CONCLUSION: These results suggest that DLCO at the time of diagnosis seem to be a prognostic markers of biopsy-proven UIP.
Biopsy ; Bronchiolitis Obliterans ; Chungcheongnam-do ; Cryptogenic Organizing Pneumonia ; Diagnosis ; Follow-Up Studies ; Glass ; Humans ; Idiopathic Pulmonary Fibrosis* ; Lung ; Prognosis ; Seoul ; Survival Rate ; Survivors

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

We describe a patient with rheumatoid arthritis(RA) who developed bronchiolitis obliterans organizing pneumonia(BOOP) during the treatment of bucillamine. A 51 year-old man was admitted to the hospital for an abnormal shadow on his chest radiogragh. He had been diagnosed as having RA 3 years previously and had been receiving 200 mg of bucillamine for 21 months. Two months prior to admission, he presented with a cough and his chest X-ray showed opacities in both lower lungs. He was treated with antibiotics for 2 months after the development of cough and lesions on the chest X-ray, but the symptoms and lung lesions became more aggravated. On admission, an HRCT revealed airspace consolidations in the subpleural space of both basal lungs and a CT-guided fine needle aspiration biopsy showed Masson's body filling air space, interstitial infiltration of acute and chronic inflammatory cells and type II cell hyperplasia, consistent with BOOP. Bucillamine was stopped and 50 mg of prednisolone was administered. His symptoms and infiltrations on the chest X-ray resolved. We suggest that bucillamine should be considered as a drug possibly associated with BOOP.
Anti-Inflammatory Agents, Non-Steroidal/therapeutic use ; Anti-Inflammatory Agents, Non-Steroidal/adverse effects* ; Arthritis, Rheumatoid/drug therapy* ; Biopsy, Needle ; Bronchiolitis Obliterans Organizing Pneumonia/diagnosis* ; Bronchiolitis Obliterans Organizing Pneumonia/chemically induced* ; Case Report ; Cysteine/therapeutic use ; Cysteine/analogs & derivatives ; Cysteine/adverse effects* ; Follow-Up Studies ; Human ; Male ; Middle Age ; Radiography, Thoracic ; Risk Assessment ; Tomography, X-Ray Computed