Bronchiolitis obliterans organizing pneumonia in Korea

Abstract An analysis of the clinical features in 23 cases of bronchiolitis obliterans organizing pneumonia (BOOP) in Korea is presented. Six were men and 17 were female, with a male-tofemale ratio of 1:2.4. Idiopathic BOOP was present in 18 of these patients, connective tissue disease-associated BOOP in five and all of them were females. The most frequent symptoms were dyspnoea and coughing in both groups; and crackles were the most prominent physical findings. Leukocytosis was observed in seven of the idiopathic BOOP group and all in the connective tissue disease-associated BOOP group. In most cases, FVC, FEV¹, diffusing capacity and arterial O₂ pressure were reduced. In roentgenographic study, patchy air space consolidation was the major finding and subpleural predominance was observed in the majority of patients in both groups. Migration of lesions were identified in only two patients with idiopathic BOOP. Steroid treatment was effective in all of idiopathic BOOP. In contrast to previous reports, an analysis of the 23 Korean BOOP patients showed several interesting points. First, a female predominance was observed. Second, migration of lesion was rare. Third, it did not show any different prognosis in patients with reticular pattern on roentgenogram compared with patients with patchy air space consolidation on roentgenogram. Whether these differences were due to ethnic or environmental factors is to be determined.     

Bronchiolitis obliterans after bone marrow transplantation

Bronchiolitis obliterans occurred in the setting of chronic graft-versus-host disease 1 year after allogeneic bone marrow transplantation for chronic myelogenous leukemia. The severe obstructive pulmonary disease followed an episode of interstitial pneumonitis. The etiology and possible relationship to graft-versus-host disease of this rare pulmonary lesion following bone marrow transplantation are discussed.     

Bronchiolitis obliterans in children

Bronchiolitis obliterans (BO) in children is a relatively rare diagnosis. The increase in lung and bone marrow transplantation in children, however, has led to a heightened interest in BO, as this is one of the important complications of those procedures. This article will discuss BO as an entity that can follow any of several illnesses or toxic exposures, in addition to following allogeneic lung or bone marrow transplantation. The complex and incompletely understood pathology, pathogenesis, and molecular pathology involved in BO remain the subject of ongoing investigations. As the prognosis for BO is uncertain and treatment is often unsuccessful, the continued need for the recognition of surrogate markers for BO in patients at risk and the development of better forms of therapy are paramount. This review will describe our current understanding of BO, and will call attention to those research areas that require continuing efforts in order to prevent or treat this entity.     

Bronchiolitis obliterans organizing pneumonia during low-dose amiodarone therapy

Two cases of amiodarone-induced pulmonary toxicity during a relatively short period of low-dose amiodarone treatment are reported. The toxicity risk of amiodarone is determined by cumulative factors in individual patients.     

Bronchiolitis obliterans organising pneumonia: a clinical and radiological review

Background: The clinical presentation, course, and radiological spectrum of bronchiolitis obliterans organising pneumonia (BOOP) is still being characterised to aid differentiation from other causes of organising pneumonia. Aims: To define the clinical presentation, response to therapy, and radiological spectrum of BOOP. Methods: Fifteen cases of BOOP were retrospectively reviewed. The clinical presenting features, treatment and outcome of each patient were determined. Three independent readers and chest X-rays (CXRs) were blinded. CXRs were scored by a semi-quantitative method. Modal scores were calculated for type and profusion of opacification of each CXR. Results: The mean age of presentation was 64 years and the median duration of follow-up was 12.5 months. Thirteen patients received corticosteroid therapy. Outcome was varied. One patient had progressive loss of lung function, five had persisting symptoms with stable abnormal lung function, and nine were asymptomatic with near normal lung function. Five patients had a disease relapse. Symptom length prior to presentation, duration and intensity of treatment were not associated with outcome (ρ= 0.23–0.9). Radiological opacities were alveolar in 73%, large localised infiltrates in 13%, nodular in 20% and mobile in 33% of CXR series. There was no relationship between overall profusion, type of CXR opacities and patient outcome, treatment duration or treatment intensity (ρ= 0.42–1.0). Caonclusions: The clinical spectrum of BOOP includes mild subacute, chronic progressive, and acute life threatening illness. Prognosis and response to treatment is variable. The diversity of radiological findings and clinical presentations should prompt consideration of the diagnosis in patients with undiagnosed respiratory tract symptoms and persisting or varying radiological abnormalities.     

Bronchiolitis obliterans organizing pneumonia following chlorambucil treatment for chronic lymphocytic leukemia

A 70-yr-old man developed cough, chest pain, hemoptysis and a pulmonary infiltrate shortly after initiation of treatment with chlorambucil for chronic lymphocytic leukemia. Chlorambucil was discontinued and an open lung procedure was performed with complete excision of the pulmonary lesion. The biopsy specimens showed histological alteration consistent with the diagnosis of bronchiolitis obliterans organizing pneumonia (BOOP). There was no evidence of other known causes usually associated with BOOP. The patient recovered completely after surgery. This report suggests that BOOP must be considered in the differential diagnosis of respiratory symptoms and pulmonary infiltrates in patients treated with chlorambucil.     

Obstructive bronchiolar disease identified by CT in the non-transplant population: Analysis of 29 consecutive cases

Background and objective: Obstructive bronchiolar disease or constrictive bronchiolitis, particularly in non‐transplant patients, is poorly understood. This study identified the associated diseases, presenting features, and clinical course of obstructive bronchiolar disease identified by CT in the non‐transplant adult population. Methods: Retrospective single‐centre study of 29 consecutive patients clinically diagnosed to have an obstructive bronchiolar disease based on the presence of respiratory symptoms and abnormal CT findings consisting of mosaic perfusion pattern with air trapping. Results: The median age was 54 years (range, 25–80 years); 20 were women (69%) and four patients (14%) had a smoking history. All 29 patients presented with respiratory symptoms, predominantly dyspnoea. The most common cause of obstructive bronchiolar disease was rheumatoid arthritis (34%). Other causes included hypersensitivity pneumonitis, multiple carcinoid tumorlets, Sjögren's syndrome, paraneoplastic pemphigus, inflammatory bowel disease and Swyer–James syndrome. The underlying cause was not identifiable in nine patients (31%), that is, cryptogenic constrictive bronchiolitis. An obstructive pattern was seen on pulmonary function testing in most patients (86%) with the exception of those with hypersensitivity pneumonitis and extreme obesity. Management usually included corticosteroid therapy, inhaled and oral, and bronchodilator therapy. Additional medications included macrolides, cytotoxic agents and other immunomodulator therapy. Pharmacologic therapy did not provide improvement in pulmonary function in the majority of patients but the follow‐up data were limited. Conclusions: Diverse causes and underlying diseases are associated with obstructive bronchiolar disease diagnosed radiologically in the non‐transplant adult population. Rheumatoid arthritis‐associated and cryptogenic constrictive bronchiolitis are found in over one‐half of these patients. Most patients with obstructive bronchiolar disease do not appear to improve with currently available therapy.     

Bronchiolitis obliterans organizing pneumonia as an initial manifestation in systemic lupus erythematosus

Bronchiolitis obliterans organizing pneumonia (BOOP) is a rare complication of adult systemic lupus erythematosus (SLE). This is the first report of a pediatric patient with BOOP as an initial presentation of SLE. She had dyspnea, cough, arthralgia, and erythema on her face. Laboratory examinations revealed pancytopenia, low serum levels of complements, and positivity for anti-nuclear antibody, anti-double stranded DNA antibody, and anti-SM antibody. Her respiratory symptoms, pulmonary function tests, and radiologic findings showed significant improvement after treatment with oral prednisolone. Although it is a rare complication among the pleuro-pulmonary manifestations in SLE, BOOP can be the first presentation, even in pediatric patients.     

激素治疗验证闭塞性细支气管炎伴机化性肺炎临床诊断（附3例报告）

目的探讨激素治疗闭塞性细支气管炎伴机化性肺炎的意义。方法对3例临床上拟诊COPD的患者,给予糖皮质激素治疗,观察患者病情及肺部影像学变化。结果接受激素治疗后,3例患者病情缓解,肺部病变逐渐好转,1年后随访,肺部病变完全或基本吸收。结论激素治疗有效可作为COPD的临床诊断依据。     

Bronchiolitis obliterans organizing pneumonia in a patient with chronic myelogenous leukemia developing after initiation of interferon and cytosine arabinoside

A 59-yr-old man developed fevers, shortness of breath, persistent cough and weight loss, shortly after initiation of therapy with interferon-alpha 2a and cytosine arabinoside for treatment of chronic myelogenous leukemia. Radiologic pulmonary infiltrates and lung tissue biopsy were consistent with bronchiolitis obliterans organizing pneumonia (BOOP). After discontinuation of the chemotherapeutic drugs, the pneumonic symptoms and chest roentgenogram infiltrates resolved. This report suggests that treatment with interferon-alpha, in combination with cytosine arabinoside, may produce the rare complication of BOOP.     

Upregulation of elastin expression in constrictive bronchiolitis obliterans

Constrictive bronchiolitis obliterans is a fibrotic disease of small airways characterized by progressive obliteration of the airway lumen, with resulting obstructive pulmonary physiology. While previous work has demonstrated the collagenous nature of the constrictive fibrotic lesions, elastin expression in the disease has been poorly characterized. Elastin is a critical component of the pulmonary extracellular matrix, and is responsible for the reversible deformability characteristic of the alveoli, pulmonary blood vessels, and airways. Elastin is a long-lived protein with virtually no active protein production occurring after lung development is completed during early childhood. We report a novel case of cryptogenic bronchiolitis obliterans in which elastin gene expression is actively upregulated in affected airways, and accompanied by myofibroblast hyperplasia and disorganized elastic fiber deposition. In addition, deposition of new elastic fibers by myofibroblasts is noted in the alveoli surrounding the affected bronchioles.     

儿童闭塞性细支气管炎的诊治进展

闭塞性细支气管炎在儿童中是一种相对少见而严重的慢性阻塞性肺疾病,与小气道损伤有关,通常病变局限在终末细支气管。起病年龄主要发生于婴幼儿时期,多由感染引起。主要临床特点是反复或持续气促、喘息、咳嗽,运动耐力差,肺部有湿啰音和喘鸣音。对支气管扩张剂无反应。近年随着诊疗技术及临床医师对该病认识水平的提高,该病在儿童中报道发病例数增多。为进一步加强儿科医师对闭塞性细支气管炎的认识,本文就闭塞性细支气管炎的近年诊治进展作一简要综述。     

Adenovirus pneumonia in infants and factors for developing bronchiolitis obliterans: a 5-year follow-up

To describe clinical, pulmonary function, and chest tomography profiles in a 5-year follow-up of infants with adenovirus pneumonia and determine the factors that potentially contributed to the development of bronchiolitis obliterans (BO). We prospectively assessed 45 hospitalized infants with adenovirus pneumonia with additional follow-up for 5 years. At the end of the study, pulmonary function by impulse oscillometry technique (IOS) and chest tomography were performed in the 38 surviving patients (mean 5.7 years of age). We divided the population between those who developed chest tomography evidence of BO and those who did not. Most of the children developed adenovirus infection before 2 years of age. During the 5 years of follow-up, almost half (47.4%) developed BO. Children who developed BO had significantly more respiratory compromise (intensive care admission, need for mechanical ventilation and for oxygen therapy, and systemic corticosteroid and beta agonist use) during their adenovirus pneumonia episode than those who did not develop BO. Only 33.3% of children with BO had normal impedance compared with 85% in the no BO group. Children who developed BO had significantly higher levels of Zrs, R5, X5 and predicted Zrs, R5, and X5 and frequency. However, there were no differences in the beta 2 agonist response between the children with and without BO (94% vs. 80%, respectively). This study represents the spectra of adenovirus pneumonia ranging from relatively mild to severe and fatal cases. Children with severe pulmonary compromise are usually more prone to develop BO.     

CT densitovolumetry in children with obliterative bronchiolitis: correlation with clinical scores and pulmonary function test results,

OBJECTIVE:

To determine whether air trapping (expressed as the percentage of air trapping relative to total lung volume [AT%]) correlates with clinical and functional parameters in children with obliterative bronchiolitis (OB).

METHODS:

CT scans of 19 children with OB were post-processed for AT% quantification with the use of a fixed threshold of −950 HU (AT%950) and of thresholds selected with the aid of density masks (AT%DM). Patients were divided into three groups by AT% severity. We examined AT% correlations with oxygen saturation (SO₂) at rest, six-minute walk distance (6MWD), minimum SO₂ during the six-minute walk test (6MWT_SO₂), FVC, FEV₁, FEV₁/FVC, and clinical parameters.

RESULTS:

The 6MWD was longer in the patients with larger normal lung volumes (r = 0.53). We found that AT%950 showed significant correlations (before and after the exclusion of outliers, respectively) with the clinical score (r = 0.72; 0.80), FVC (r = 0.24; 0.59), FEV₁ (r = −0.58; −0.67), and FEV₁/FVC (r = −0.53; r = −0.62), as did AT%DM with the clinical score (r = 0.58; r = 0.63), SO₂ at rest (r = −0.40; r = −0.61), 6MWT_SO₂ (r = −0.24; r = −0.55), FVC (r = −0.44; r = −0.80), FEV₁ (r = −0.65; r = −0.71), and FEV₁/FVC (r = −0.41; r = −0.52).

CONCLUSIONS:

Our results show that AT% correlates significantly with clinical scores and pulmonary function test results in children with OB.     

Bronchiolitis obliterans organizing pneumonia (BOOP) in a case of smouldering adult T-cell leukaemia

Although various respiratory diseases have been reported in human T lymphotropic virus type-1 (HTLV-1) carriers or patients with adult T-cell leukaemia (ATL), there appears to be no report of the development of bronchiolitis obliterans organizing pneumonia (BOOP) in ATL or HTLV-1-related disorders. We describe a 51-year-old male with smouldering ATL who developed BOOP during a long-term follow up. Bronchoalveolar lavage (BAL) and transbronchial lung biopsy (TBLB) were performed in the right lower lobe B6 with infiltrative shadows. As a result of flow cytometric analysis of peripheral lymphocytes and BAL lymphocytes, histological examination of the biopsied lung specimen, and the clinical course, we excluded the pulmonary infiltration of ATL cells and bacterial infection. Thus, he was diagnosed as having BOOP and successfully treated with corticosteroid therapy. This is probably the first report of BOOP developing in ATL. Bronchiolitis obliterans organizing pneumonia should be considered in the differential diagnosis of pulmonary complications in HTLV-1 carriers or ATL patients since BOOP can be successfully treated by corticosteroids.