Bronchiolitis and Bronchiolar Disorders.

Bronchioles are noncartilaginous small airways with internal diameter of 2 mm or less, located from approximately the eighth generation of purely air conducting airways (membranous bronchioles) down to the terminal bronchioles (the smallest airways without alveoli) and respiratory bronchioles (which communicate directly with alveolar ducts and are in the range of 0.5 mm or less in diameter). Bronchiolar injury, inflammation, and fibrosis may occur in myriad disorders including connective tissue diseases, inflammatory bowel diseases, lung transplant allograft rejection, graft versus host disease in allogeneic stem cell recipients, neuroendocrine cell hyperplasia, infections, drug toxicity (e.g., penicillamine, busulfan), inhalation injury (e.g., cigarette smoke, nylon flock, mineral dusts, hard metals, Sauropus androgynous); idiopathic, common variable immunodeficiency disorder, and a host of other disorders or insults. The spectrum of bronchiolar disorders is wide, ranging from asymptomatic to fatal obliterative bronchiolitis. In this review, we discuss the salient clinical, radiographic, and histological features of these diverse bronchiolar disorders, and discuss a management approach.

A new endoscopic standardized grading system for macroscopic central airway complications following lung transplantation: the MDS classification.

OBJECTIVES: After lung transplant, between 9 and 13% of bronchial anastomoses develop complications severe enough to warrant therapeutic intervention. These complications include stenosis, dehiscence, granulation tissue, bronchomalacia and fistula. Most of these have already been included in a classification or another, but none of these have been universally accepted. Moreover, no grading system has integrated all of these complications. The Groupe Transplantation (GT) (Transplant Group), from the Société de Pneumologie de Langue Française (SPLF) [French Language Pulmonology Society], maintains a prospective national registry of lung transplants performed in France. The GT has mandated the Groupe d'Endoscopie de Langue Française (GELF), also from the SPLF, to develop an endoscopic classification, in order to describe the macroscopic aspect of the bronchial anastomoses, and downhill airways, using a standardized and exhaustive grading system. METHODS: An endoscopic classification that would take into account the three major aspects of the description of bronchial anastomoses was elaborated. The first parameter is the macroscopic aspect (M), the second, the diameter (D) of the anastomosis and the third, the sutures (S) of the anastomosis. This classification was then submitted to expert bronchoscopists from nine centres, responsible for lung transplants in France, for their opinion, using a five-item questionnaire, according to the Delphi methodology. RESULTS: After the first round of consultation, all experts (100%) agreed on Questions 1 and 4. Answers were positive for Questions 2 (59%), 3 (56.25%) and 5 (70%). A modified classification, incorporating propositions from the first round, was then submitted. This second round allowed a consensus to be reached between all experts: the MDS classification. Each parameter (M, D and S) can be classified from 0 to 3. For M and D, it is possible to determine the extent of abnormalities downhill from the anastomosis into four subgroups (a, b, c or d). For S, the localization of abnormalities can be divided between two subgroups (e and f). CONCLUSION: The MDS classification, established by a consensus of French experts in bronchoscopy, could represent a standardized, universally acceptable system to describe central airway complications after lung transplant.

Classification of broncholiths and clinical outcomes.

BACKGROUND AND OBJECTIVE: We evaluated effective treatments of broncholithiasis based on its radiographical and bronchoscopic features. METHODS: This retrospective study conducted at Samsung Medical Center, Korea enrolled patients who were suspected of having broncholithiasis based on chest computed tomography (CT). The broncholiths were classified as intraluminal, mixed (both intraluminal and extraluminal) and extraluminal based on chest CT and bronchoscopic findings. RESULTS: The study enrolled 46 patients between 1995 and 2009. Symptoms included cough (n = 21, 45.7%), hemoptysis (n = 19, 41.3%) and purulent sputum (n = 11, 23.9%). Cough was more common in intraluminal boncholiths than in other type of broncholiths (P = 0.03). Based on chest CT, there were 15 (32.6%) intraluminal, 15 (32.6%) mixed and 16 (34.8%) extraluminal broncholiths. All 15 intraluminal broncholiths were removed completely via flexible (n = 2) or rigid (n = 13) bronchoscopy. For the 15 mixed broncholiths, seven (46.7%) bronchoscopic interventions were performed, but complete removal of the broncholiths was not accomplished. Six (40%) mixed and four (25%) extraluminal broncholiths were treated by surgical resection for symptom control. None of the patients who underwent surgical resection suffered morbidity or postoperative mortality. CONCLUSIONS: The treatment of broncholithiasis should be based on chest CT and bronchoscopic findings. Intraluminal broncholiths can be removed via bronchoscopy, while surgery should be considered for symptomatic mixed or extraluminal broncholiths.

Anthracofibrosis or anthracostenosis.

Anthracofibrosis is a bronchial stenosis due to local mucosal fibrosis that also presents anthracotic pigment in the mucosa. The cause has not been well clarified, although there is a frequent association with tuberculosis and the exposure to smoke from biofuel or biomass combustion. It is an entity that has not been reported in Spain, although the influx of people from rural areas of developing countries or rural areas of our own country should make us contemplate this entity in the differential diagnosis of our patients. We present 3 cases detected in Spain (2 of them natives) diagnosed by bronchoscopy and bronchial biopsy, which are techniques necessary to confirm the diagnosis. There is no specific treatment, except for tuberculostatic treatment in cases with coexisting tuberculosis.

[Classification of occupational bronchial diseases: matters of controversy, open problems].

Current national classification of occupational bronchitis lacks to consider drastic changes in understanding pathogenesis of bronchial diseases over the recent decade, that leads to misunderstanding between occupational therapists and general practitioners in using this classification. The authors suggested a variant to classify occupational bronchial diseases: chronic occupational nonobstructive (simple) bronchitis and occupational COPD, that correspond to approaches accepted by international medical community. Tracheobronchial dyskinesia, asthmatic bronchitis and chronic pneumonia should be excluded from the classification.

[Clinical classification of 288 cases of bronchial tuberculosis based on an expert consensus].

OBJECTIVE: To study the clinical application and significance of the recently published expert consensus on endobronchial tuberculosis (EBTB). METHODS: A retrospective analysis of 288 cases of EBTB hospitalized in Tianjin Haihe Hospital from May 2005 to April 2010 was carried out. The classification and typing of the disease were based on a consensus report recently published by Chinese Journal of Tuberculosis and Respiratory Diseases. Chi-square test was performed to analyze the differences between groups. RESULTS: Of the 288 cases of EBTB, 47.9% (138/288) was classified as Type I (Inflammatory infiltrative), 33.3% (96/288) as Type II (ulcerous necrotic), 5.2% (15/288) as Type III (granulomatous hyperplastic), 7.3% (21/288) as Type IV (scar stricture) and 0.4% (1/288) as Type V (Bronchomalacia), respectively. There were 17 cases (5.9%) classified as a mixed type with a combination of Type IV or Type V disease with 1 or more of the other types. 37.5% (108/288) of the patients were young females, while young and middle-aged patients with type I and type II diseases accounted for 74.7% (215/288) of the cases, much more than old aged patients (6.6%, 19/288). 97.2% (n=280) of the cases suffered from secondary pulmonary tuberculosis. In 107 cases, the disease was located in the left, 162 cases in the right, while in 109 cases the right upper lobe bronchus was involved, and right main bronchus in 36 cases, 3 cases and 58 cases in left upper lobe with and without lingular segment, 10 cases in lingular segment only. Chest CT showed that local mucosal thickening of the trachea or bronchus was evident in 40.3% (116/288); toothed or spike protuberance in 30.9% (89/288), bronchial obstruction in 11.1% (32/288), and bronchial stenosis in 87.9% (253/288). The negative rate of sputum in the first month after interventional therapy was 60.2% (56/93), significantly higher than that in non-interventional therapy group (23.1%, 18/78). CONCLUSION: The new consensus report on EBTB was clinically useful for classification and typing of the disease, and for the selection of treatment modalities.

The pathologist's approach to small airways disease.

The small airways constitute a significant compartment of the lung, and diseases therein are not uncommon, occurring in a wide variety of diseases, either as a primary bronchiolar disorder or as a component of local or systemic disease affecting multiple anatomical compartments. Unlike the idiopathic interstitial pneumonias, there is as yet no consensus classification system for bronchiolar disorders, and a multidisciplinary approach to their classification is discussed. Whereas some conditions show relatively characteristic histological features, others show non-specific or subtle changes that rely on a combination of clinical, radiological and pathological information for accurate final diagnosis. Furthermore, certain diseases of the small airways have been better characterized recently, and several provisional new entities have also been described, some of these being reviewed here.

Description of a multidimensional classification system for patients with expiratory central airway collapse.

BACKGROUND AND OBJECTIVE: Patients with expiratory central airway collapse present with various symptoms, aetiologies, morphologies, extent and severity of airway collapse. The aim of this study was to delineate a multidimensional classification system and common language for evaluating patients with expiratory central airway collapse. METHODS: The classification system was based on the morphology and origin of the airway abnormality as well as stratification parameters such as functional status, extent and severity of airway collapse. Patients with expiratory central airway collapse who underwent clinical and bronchoscopic examination before and after treatment were identified from a bronchoscopy database. The classification was applied to the study patients before and after treatment to assess the utility of this stratification approach. RESULTS: Eighteen patients were studied. Four had excessive dynamic airway collapse and 14 had tracheobronchomalacia. Post-treatment, functional status improved by one class in 12 patients, by two classes in four patients and remained unchanged in two. Severity of airway collapse improved in 15 and remained unchanged in three patients. The extent of abnormality diminished in 14 patients and did not change in four. CONCLUSIONS: Using this classification, the morphologies and aetiologies of expiratory central airway collapse are identified, and an objective stratification of patients according to degree of functional impairment, extent of disease and severity of airway collapse is possible.

A proposed classification system of central airway stenosis.

Tracheobronchial stenosis, a serious problem in adults and children, has multiple causes and has been treated in many ways. While developing an international multicentre study to evaluate efficacy of airway stents, it was realised that no adequate description of central airway stenosis regarding type, location and degree has been published. Thus, comparing results of different treatment modalities in different centres has been difficult due to a lack of uniformity of classification. Reports are typically descriptive and precise classification schemes have not adequately addressed either for the trachea or the main bronchi. A standardised classification scheme was proposed with descriptive images and diagrams for rapid and uniform classification of central airway stenosis. The present authors' system divides stenosis into structural and dynamic types and further classifies the disease by degree of stenosis, location and transition zone. Multiple sites can be described and each is transformed into a simple numerical scoring system prompted by a diagram, which can be easily captured for subsequent uniform analysis across sites. A pilot validation of the system, with 18 pulmonologists of varying training background, showed strong precision and agreement between observers. Such a system will enhance the ability to study the effectiveness of treatment modalities for central airway stenosis.

Tracheobronchomalacia and excessive dynamic airway collapse.

Tracheobronchomalacia and excessive dynamic airway collapse are two separate forms of dynamic central airway obstruction that may or may not coexist. These entities are increasingly recognized as asthma and COPD imitators. The understanding of these disease processes, however, has been compromised over the years because of uncertainties regarding their definitions, pathogenesis and aetiology. To date, there is no standardized classification, diagnosis or management algorithm. In this article we comprehensively review the aetiology, morphopathology, physiology, diagnosis and treatment of these entities.

Classification and approach to bronchiolar diseases.

PURPOSE OF REVIEW: Bronchiolar abnormalities are relatively common and occur in a variety of clinical contexts. There have been an increasing number of terms, some of which are redundant, used in referring to various forms of bronchiolar disorders. The purpose of this review is to provide an updated classification scheme to facilitate the clinical approach to patients with suspected bronchiolar disease. RECENT FINDINGS: The spectrum of bronchiolar disorders is broader and more heterogeneous than previously recognized. In deciphering the potential significance of bronchiolar abnormalities, it is useful to distinguish those disorders in which the bronchiolar involvement is the dominant pathologic process, that is, primary bronchiolar disorders, from parenchymal or large airway diseases in which the bronchioles may become secondarily involved. Primary bronchiolar disorders include respiratory bronchiolitis, acute bronchiolitis, constrictive bronchiolitis, follicular bronchiolitis, diffuse panbronchiolitis, mineral dust airway disease, and a few other variants. Interstitial lung diseases with a prominent bronchiolar involvement include smoking-related interstitial lung diseases, cryptogenic organizing pneumonia, and hypersensitivity pneumonitis. Bronchiolitis can also be seen in large airway diseases such as chronic bronchitis, bronchiectasis, and asthma. SUMMARY: In the clinical approach to a patient with bronchiolar disease, primary bronchiolar disorders should be distinguished from predominantly parenchymal or large airway processes with bronchiolar involvement. The number of patterns of bronchiolar response to injury is limited and these patterns are generally non-specific in regard to cause. Appropriate diagnosis and management of patients with bronchiolar disorders depend on judicious correlation of clinical, physiologic, and morphologic manifestations.

Otras enfermedades obstructivas: bisinosis, bronquitis crónica y EPOC de origen laboral y bronquitis eosinofílica / Other obstructive diseases: byssinosis, chronic bronchitis and occupational COPD and eosinophilic bronchitis

Además del asma ocupacional y las enfermedadesderivadas de la inhalación aguda, otras enfermedadesobstructivas también reconocen un origen laboral.Aunque en la actualidad la bisinosis es una enfermedadrara en España, describimos las características de lamisma por su interés histórico dentro de las enfermedadesrespiratorias de origen laboral y porque todavíasigue vigente en los países en vías de desarrollo. Labronquitis crónica también puede estar relacionadacon la exposición laboral a polvos y humos, con frecuenciadenominada “bronquitis industrial”. La relaciónocupacional de la EPOC ha sido más controvertidaa lo largo de la historia, pero en la actualidad estarelación está aceptada; describimos la evidencia actualque sustenta esta relación. En último lugar, describimosla bronquitis eosinofílica sin asma, entidad descritapor primera vez en 1989 y que en ocasiones tambiénpuede tener un origen laboral, compartiendo agentesetiológicos con el asma ocupacional

Besides occupational asthma and diseases derivedfrom acute inhalation, other obstructive diseases alsohave an occupational origin. Although at presentbyssinosis is a rare disease in Spain, we describe itscharacteristics because of its historical interestamongst occupational respiratory diseases andbecause it is still relevant in developing countries.Chronic bronchitis can also be related to exposure atwork to dust and smoke, and is often referred to as“industrial bronchitis”. Historically, the relation ofCPOD to occupation has been subject to controversy,but nowadays this relationship is accepted; wedescribe the present evidence supporting thisrelationship. Finally, we describe eosinophilicbronchitis without asthma, an entity that wasdescribed for the first time in 1989 and that cansometimes have an occupational origin, sharingaetiological agents with occupational asthma

Symptomatic improvement in dyspnea following tracheobronchial metallic stenting for malignant airway obstruction.

PURPOSE: To investigate the value and limitation of Gianturco expandable metallic stenting for patients with dyspnea due to stenotic tracheobronchial lesions associated with malignancies. MATERIAL AND METHODS: We treated 55 lesions of 44 patients with obstructing stenotic tracheobronchial lesions related to end-stage malignancies by Gianturco expandable metallic stents (EMSs). RESULTS: In 42 of 44 patients, the dyspnea subjectively improved after the procedure (95.5%). An improvement over one grade of the Hugh-Jones classification was shown in 79.5% (35/44); in 80% (20 of 25 patients) with intraluminal tumor and in 78.9% (15 of 19 patients) with extrinsic compression. Seven of the 44 patients developed dyspnea related to re-stenosis of 10 lesions and 1 of these patients developed dyspnea related to re-re-stenosis during follow-up. The mean duration of survival was 4.3 months in patients who underwent stenting. No significant differences in survival rates and primary patency rates were seen in patients with extrinsic compression compared to patients with intraluminal tumors. CONCLUSION: Gianturco EMS therapy was valuable in patients who suffered from dyspnea due to airway stenosis causing obstruction.

Bronchiolar disease: spectrum and radiological findings.

Two types of bronchiole, the terminal bronchiole and the respiratory bronchiole, have structural and functional differences. The former is characterized as a conducting airway and the latter is closely related to a gas-exchange function as it has numerous alveoli on the wall. Therefore, the diseases occurring at bronchiole demonstrate different pathological, radiological and clinical pictures depending on which bronchiole is mainly involved. The disease that mainly involves the conducting airway is appreciated as a small airway disease. Constrictive bronchiolitis is a well-recognized entity classified in this category. Whereas the disease mainly involves the respiratory bronchiole and distal alveolar space, it is recognized as an interstitial and parenchymal disease. BOOP or RB-ILD is classified in this category. These two types of bronchiolar diseases reveal the contrast clinical pictures, including incidence, causative disease, response to the treatment, prognosis, respiratory function test as well as the radiological findings. This pictorial essay will illustrate the radiological features of the varieties of bronchiolitis.

Bronchoscopic assessment of the evolution of endobronchial tuberculosis.

BACKGROUND: We previously classified forms of endobronchial tuberculosis (EBTB) into seven subtypes by bronchoscopic finding: actively caseating, edematous-hyperemic, fibrostenotic, tumorous, granular, ulcerative, and nonspecific bronchitic. STUDY OBJECTIVE: To evaluate the value of this classification in predicting the therapeutic outcome of EBTB. DESIGN: A prospective study with serial bronchoscopy performed from the diagnosis of EBTB to the completion of antituberculosis chemotherapy. PARTICIPANTS: Eighty-one patients with biopsy-proven EBTB. INTERVENTIONS: Fiberoptic bronchoscopy was done every month until there was no subsequent change in the endobronchial lesions, every 3 months thereafter, and at the end of treatment. RESULTS: Twenty-two of the 34 cases of actively caseating EBTB changed into the fibrostenotic type, and the other 12 healed without sequelae. Seven of the 11 cases of edematous-hyperemic EBTB changed into the fibrostenotic type, and the other 4 healed. Nine of the 11 cases of granular EBTB, 6 cases of nonspecific bronchitic EBTB, and 2 cases of ulcerative EBTB resolved completely. However, the other two cases of granular EBTB changed into the fibrostenotic type. Seven cases of fibrostenotic EBTB did not improve despite antituberculosis chemotherapy. These various changes in bronchoscopic findings occurred within 3 months of treatment. In 10 cases of tumorous EBTB, 7 progressed to the fibrostenotic type. In addition, new lesions appeared in two cases, and the size of the initial lesions increased in another two cases, even at 6 months after treatment. CONCLUSIONS: The therapeutic outcome of each subtype of EBTB can be predicted by follow-up bronchoscopy during the initial 3 months of treatment, with the exception of the tumorous type. In tumorous EBTB, close and long-term follow-up is advisable because the evolution of the lesions during treatment is very complicated and bronchial stenosis may develop at a later time.

Bronchoscopic, radiologic and pulmonary function evaluation of endobronchial tuberculosis.

OBJECTIVE: Endobronchial tuberculosis (EBTB) causes problems in both making a diagnosis and following up the endobronchial lesion, because the lesion is not evident in simple chest radiograph. Frequently, the diagnosis and follow up of EBTB lesion during treatment mainly depend on bronchoscopy. Chest computed tomography (CT) and pulmonary function test (PFT) have also been used in evaluating EBTB and differentiating it from the other diseases. Primary purpose of the present study was to observe the change of EBTB lesion during treatment and determine the optimal time and the indication of follow-up bronchoscopy. We also evaluate the usefulness of chest CT and PFT in EBTB. METHODOLOGY: Eighty-one biopsy-proven EBTB patients were enrolled from 1992 to 1997. Endobronchial tuberculosis was classified into seven subtypes: actively caseating, fibrostenotic, oedematous-hyperaemic, tumorous, ulcerative, granular, and non-specific bronchitic type according to bronchoscopic features by Chung's Classification. The change of bronchoscopic findings during treatment in each subtype of EBTB was evaluated prospectively. Follow-up bronchoscopy was done each month until there was no subsequent change in endobronchiat lesion, and every 3 months thereafter, and at the end of treatment. Chest CT and PFT were performed in 26 and 68 patients respectively, at initial bronchoscopy. RESULTS: Twenty-two of the 34 cases of actively caseating EBTB changed into the fibrostenotic type, and the other 12 healed without sequelae. Seven of the 11 cases of oedematous-hyperaemic EBTB changed into the fibrostenotic type, and the other four healed. Nine of the 11 cases of granular EBTB, six cases of non-specific bronchitic EBTB, and two cases of ulcerative EBTB resolved completely. However, the other two cases of granular EBTB changed into the fibrostenotic type. Seven cases of fibrostenotic EBTB did not improve despite antituberculosis chemotherapy. These various changes in bronchoscopic findings occurred within 3 months of treatment. In 10 cases of tumorous EBTB, seven progressed to the fibrostenotic type. In addition, new lesions appeared in two cases, and the size of the initial lesions increased in another two cases, even at 6 months after treatment. On chest CT findings of 26 EBTB patients, the length of bronchial involvement was measured from 10 to 55 mm. Bronchial stricture was noticed in 25 cases and the range of narrowing was from total occlusion to near normal, and there was wide variation in bronchial stricture even within same subtype of EBTB. The dominant feature of PFT in EBTB at the diagnosis was restrictive pattern. CONCLUSION: The therapeutic outcome of each subtype of EBTB can be predicted by follow-up bronchoscopy during the initial 3 months, with the exception of the tumorous type. In tumorous EBTB, the evolution of the lesions during treatment is very complicated, and bronchial stenosis may develop at a later time. Chest CT was useful in measuring the length of involved bronchus and degree of stricture in EBTB. PFT may be useful in differential diagnosis and follow up of EBTB.

Pediatric and adult tracheobronchomalacia.

Twelve cases of tracheobronchomalacia (TBM) cases were reviewed: five were pediatric, and seven were adult, two of which were due to relapsing polychondritis (RPC). In pediatric TBM, the malacic segments were short. Resection of the malacic segment in one case and laryngotracheoplasty with autologous costal cartilage in one case were unsuccessful. However, aortopexy gained good results. Two cases managed conservatively experienced gradual improvement of their symptoms. In adult TBM, plication of pars membranacea was not effective in one case. The insertion of a stent was minimally effective in one case, and distinctly in one polychondritic case. The other four cases managed conservatively have deteriorated gradually. From these findings, a new classification system is proposed.

Airway complications in lung transplantation.

This article reviews the literature on airway healing after lung transplantation. From a historical point of view, this has been the Achilles' heel of lung transplantation through two decades, from the first attempt at single-lung transplantation in 1963 to the clinical successes in the early 1980s. The overall incidence of lethal airway complications is estimated to be 2% to 3%, whereas that of late stricture is 7% to 14%. Comparison of experiences has been difficult without a universal classification; a new classification for airway and anastomotic complications and healing is proposed. Ischemia appears to be the most important factor influencing airway healing. Low-pressure collateral bronchial blood flow from the pulmonary artery may be affected by low cardiac output, reperfusion edema, or rejection; mucosal injury may be further increased by prolonged positive-pressure ventilation. Good bronchial healing appears to be possible without a protective wrap and with early use of steroids. The management of bronchial complications is challenging and requires endoscopic skills including knowledge of endobronchial laser photocoagulation and stent insertion techniques.

Endoscopic classification of tracheobronchial tuberculosis with healing processes.

We established an endoscopic classification of tracheobronchial tuberculosis with healing processes. According to this classification, the period of time needed for healing was found to be shorter in patients who were treated by aerosolized streptomycin than in those treated with the conventional triple-drug oral regimen.