Lung Ultrasound and Clinical Progression of Acute Bronchiolitis: A Prospective Observational Single-Center Study.

Background and Objectives: Recent literature suggests that lung ultrasound might have a role in the diagnosis and management of bronchiolitis. The aim of the study is to evaluate the relationship between an ultrasound score and the clinical progression of bronchiolitis: need for supplemental oxygen, duration of oxygen therapy and hospital stay. Materials and Methods: This was a prospective observational single-center study, conducted in a pediatric unit during the 2017-2018 epidemic periods. All consecutive patients admitted with clinical signs of acute bronchiolitis, but without the need for supplemental oxygen, underwent a lung ultrasound in the first 24 h of hospital care. The lung involvement was graded based on the ultrasound score. During clinical progression, need for supplemental oxygen, duration of oxygen therapy and duration of hospital stay were recorded. Results: The final analysis included 83 patients, with a mean age of 4.5 ± 4.1 months. The lung ultrasound score in patients that required supplemental oxygen during hospitalization was 4.5 ± 1.7 (range: 2.0-8.0), different from the one of the not supplemented infants (2.5 ± 1.8; range: 0.0-6.0; p < 0.001). Ultrasound score was associated with the need for supplemental oxygen (OR = 2.2; 95% CI = 1.5-3.3; p < 0.0001). Duration of oxygen therapy was not associated with LUS score (p > 0.05). Length of hospital stay (coef. = 0.5; 95% CI = 0.2-0.7; p < 0.0001) correlates with LUS score. Conclusion: Lung ultrasound score correlates with the need of supplemental oxygen and length of hospital stay in infants with acute bronchiolitis.

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.

Small Airway Disease: A Step Closer to Etiology-Based Classification of Bronchiolitis.

Three major histologic patterns of bronchiolitis: obliterative bronchiolitis, follicular bronchiolitis, and diffuse panbronchiolitis, are reviewed in detail. These distinct patterns of primary bronchiolar injury provide a useful starting point for formulating a differential diagnosis and considering possible causes. In support of the aim toward a cause-based classification system of small airway disease, a simple diagnostic algorithm is provided for further subclassification of the above 3 bronchiolitis patterns according to the major associated etiologic subgroups.

Adult bronchiolitis--a clinical and pathological interpretative classification.

INTRODUCTION: Bronchiolitis is a heterogeneous group of diseases of an inflammatory nature, centered on small conducting airways and often associated with other pulmonary disorders. No single classification scheme for bronchiolar diseases has been widely accepted. In this retrospective study, it was decided to apply a new clinical and pathological interpretative classification. OBJECTIVES: To propose a new clinical and pathological interpretative classification for adult bronchiolitis, based on statistical analysis of a population of 193 patients with histopathological diagnosis of bronchiolitis. MATERIALS AND METHODS: A retrospective study analyzed the epidemiological characteristics, co-morbidities and radiological findings present in a group of patients with histopathological diagnosis of bronchiolitis. RESULTS: This trial involved 193 cases collected over a period of eleven years; 48 (24.9%) patients had simultaneous pulmonary disease; non-pulmonary diseases, such as cardiovascular diseases, type II Diabetes mellitus and dyslipidemia were present in 57 cases. The image study was extremely important in order to integrate clinical and pathological aspects. In this study respiratory bronchiolitis related to smoking dominated. The radiological findings confirmed the secondary nature of the histopathological features, with prevalence of ground-glass patterns, pneumothorax and patterns of interstitial involvement, as described in the literature. It was also verified that clinical behavior of different forms of bronchiolitis was important to distinguish the various types, since they could progress without typical anatomopathological aspects. CONCLUSION: This trial showed that the vast majority of diagnosis obtained corresponded to bronchiolitis as secondary to pulmonary pathology. In most cases, morphological findings had to be complemented with clinical and radiological characteristics, in order to obtain the final diagnosis.

Validation of a scale to assess the severity of bronchiolitis in a population of hospitalized infants.

BACKGROUND: Although assessment of the severity of bronchiolitis using severity scores is important both in daily practice and as an outcome measure in clinical trials, many of these scores have not been formally validated or have been only partially validated. METHODS: We conducted a prospective cohort study on a sample of children diagnosed with bronchiolitis. Two physicians independently assessed all of the children on the modified Wood's Clinical Asthma Score (M-WCAS) and on the Tal et al. severity score and collected the information required to assess the criterion validity, construct validity, inter-rater agreement, sensitivity to change, and usability of the M-WCAS. RESULTS: The median (interquartilic range [IQR]) of the age of the 54 patients included in the study was 5 (2-9) months. Thirty (55.6%) of the patients were males and 24 (44.4%) were female. The scores of the M-WCAS correlated positively with the scores of the Tal et al. severity score (ρ = 0.761, p < 0.001). The scores of the M-WCAS in patients who required subsequent admission to the PICU were significantly higher than those in patients who required admission only to the pediatric medical floor (PMF) [4.5 (3.6-5.2) vs. 2.5 (1.5-2.5), p < 0.001]. The inter-rater agreement for the raters was found to be κ = 0.897 (p < 0.001), 95% CI (0.699-1.000). The scores of the M-WCAS in patients at admission to the PMF were significantly higher than those obtained immediately before discharge from the hospital [2.5 (1.9-2.5) vs. 1.0 (0.5-1.6), p < 0.001). CONCLUSIONS: Our results suggest that the M-WCAS severity score has adequate criterion validity, adequate construct validity, adequate inter-rater agreement, adequate sensitivity to change, and appropriate usability for infants hospitalized for acute bronchiolitis.

Bronchiolitis: adopting a unifying definition and a comprehensive etiological classification.

Bronchiolitis is an inflammatory and potentially fibrosing condition affecting mainly the intralobular conducting and transitional small airways. Secondary bronchiolitis participates in disease process of the airways and/or the surrounding lobular structures in the setting of several already defined clinical entities, mostly of known etiology, and occurs commonly. Primary or idiopathic bronchiolitis dominates and characterizes distinct clinical entities, all of unknown etiology, and occurs rarely. Secondary bronchiolitis regards infections, hypersensitivity disorders, the whole spectrum of smoking-related disorders, toxic fumes and gas inhalation, chronic aspiration, particle inhalation, drug-induced bronchiolar toxicities, sarcoidosis and neoplasms. Idiopathic or primary bronchiolitis defines clinicopathologic entities sufficiently different to be designated as separate disease entities and include cryptogenic constrictive bronchiolitis, diffuse panbronchiolitis, diffuse idiopathic pulmonary neuroendocrine cell hyperplasia, neuroendocrine hyperplasia in infants, bronchiolitis obliterans syndrome in lung and allogeneic hematopoietic cell transplantation, connective tissue disorders, inflammatory bowel disease and bronchiolitis obliterans organizing pneumonia. Most of the above are pathological descriptions used as clinical diagnosis. Acute bronchiolitis, though potentially life threatening, usually regresses. Any etiology chronic bronchiolitis contributes to morbidity and/or mortality if it persists and/or progresses to diffuse airway narrowing and distortion or complete obliteration. Bronchiolitis in specific settings leads to bronchiolectasis, resulting in bronchiectasis.

[The distal airways in systemic disease]. / Voies aériennes distales et maladies de système.

The association of inflammatory involvement of the distal airways or bronchiolitis and systemic diseases is essentially observed in Sjögren's syndrome, rheumatoid arthritis and chronic inflammatory bowel disease. Bronchiolitis may be mainly cellular in nature, often involving lympho-monocytic cells, and sometimes associated with lymphoid follicles, as in Sjögren's syndrome. It may also, particularly in rheumatoid arthritis, be constrictive, with peribronchiolar fibrosis. This type is associated with a worse prognosis, with possible progression to chronic respiratory insufficiency. The diagnosis of bronchiolitis should be suspected in any atypical form of asthma, or recurrent "bronchitis", and it is essential to look for extrarespiratory symptoms and auto-antibodies to establish the diagnose of systemic disease. The CT appearances coupled with the evaluation of pulmonary function parameters usually lead to the diagnosis. In severe and/or rapidly progressive cases treatment-combining corticosteroids with immunosuppressive drugs may be prescribed, but often with disappointing results. In these cases, lung transplantation should be considered in young patients.

Lymphocytic interstitial pneumonia and other benign lymphoid disorders.

Nonneoplastic pulmonary lymphoid disorders consist of a complex spectrum of diseases for pathologists and pulmonologists alike. Advances in our understanding of these disorders in recent years have led to revisions in the classification scheme. This review summarizes the clinicoradiological and pathological features of several benign pulmonary lymphoid disorders as well as the current knowledge regarding their pathogenesis. The disorders discussed include lymphocytic interstitial pneumonitis, follicular bronchiolitis, nodular lymphoid hyperplasia, inflammatory pseudotumor, Castleman disease, immunoglobulin G4-related disease in the lung, and posttransplant lymphoproliferative disease.

[Bronchiolitis. Part 1--anatomic features, classification, clinical presentation and imaging]. / Bronchiolitis. Teil 1--Anatomische Grundlagen, Klassifikation, klinische Präsentation und Bildgebung.

The term "bronchiolitis" refers to a broad spectrum of common conditions related to the small airways associated with a miscellaneous aetiology, histology, clinical features and course. Due to their variability, bronchiolar disorders are generally difficult to diagnose. History (smoking, collagen vascular disease, inhalational injury, medication usage, and organ transplant) may point towards a bronchiolar process. In addition, signs of systemic and pulmonary infection and evidence of air trapping may provide diagnostic hints. Although clinical presentation, physical examination, pulmonary function tests (obstructive ventilatory defect), and plain chest radiographs may demonstrate abnormalities suggesting small airways involvement, they are often non-specific and rarely diagnostic. In contrast, the high-resolution CT (HR-CT) scanning of the chest provides three distinct HR-CT patterns that assist in the diagnosis and differential diagnosis of bronchiolar conditions: (i) a tree-in-bud pattern, (ii) ill-defined centrilobular ground-glass nodules, and (iii) a mosaic attenuation pattern (best visible on expiratory images). The present paper summarises the current knowledge, the classification, imaging, and the clinical presentation of bronchiolar disorders.

Evaluación clínico-radiológica y clasificación de la bronquiolitis del adulto / Bronchiolar disorders: clinical-radiological assessment and classification

Bronchiolar disorders are generally difficult to diagnose. A detailed clinical history may point toward a specific diagnosis. Pertinent clinical questions include history of smoking, collagen vascular disease, inhalation injury, medication use and organ transplantation. It is important also to evaluate possible systemic and pulmonary signs of infection, evidence of air trapping, and high-pitched expiratory wheezing, which may suggest small airways involvement. Pulmonary function tests and plain chest radiography may demonstrate abnormalities; however, they rarely prove sufficiently specific to obviate bronchoscopic or surgical biopsy. High-resolution CT (HRCT) scanning of the chest is often an important diagnostic tool to guide diagnosis in these difficult cases, because different subtypes of bronchiolar disorders may present with characteristic image findings. Some histopathologic patterns of bronchiolar disease may be relatively unique to a specific clinical context but others are nonspecific with respect to either etiology or pathogenesis. Primary bronchiolar disorders include acute bronchiolitis, respiratory bronchiolitis, follicular bronchiolitis, mineral dust airway disease, constrictive bronchiolitis, diffuse panbronchiolitis, and other rare variants. Prominent bronchiolar involvement may be seen in several interstitial lung diseases, including hypersensitivity pneumonitis, collagen vascular disease, respiratory bronchiolitis-associated interstitial lung disease, cryptogenic organizing pneumonia, and pulmonary Langerhans’ cell histiocytosis. Large airway diseases that commonly involve bronchioles include bronchiectasis, asthma, and chronic obstructive pulmonary disease. The clinical and prognostic significance of a bronchiolar lesion is best determined by identifying the etiology, underlying histopathologic pattern and assessing the correlative clinic-physiologic-radiologic context.

[Bronchiolar disorders: clinical-radiological assessment and classification]. / Evaluación clínico-radiológica y clasificación de la bronquiolitis del adulto.

Bronchiolar disorders are generally difficult to diagnose. A detailed clinical history may point toward a specific diagnosis. Pertinent clinical questions include history of smoking, collagen vascular disease, inhalation injury, medication use and organ transplantation. It is important also to evaluate possible systemic and pulmonary signs of infection, evidence of air trapping, and high-pitched expiratory wheezing, which may suggest small airways involvement. Pulmonary function tests and plain chest radiography may demonstrate abnormalities; however, they rarely prove sufficiently specific to obviate bronchoscopic or surgical biopsy. High-resolution CT (HRCT) scanning of the chest is often an important diagnostic tool to guide diagnosis in these difficult cases, because different subtypes of bronchiolar disorders may present with characteristic image findings. Some histopathologic patterns of bronchiolar disease may be relatively unique to a specific clinical context but others are nonspecific with respect to either etiology or pathogenesis. Primary bronchiolar disorders include acute bronchiolitis, respiratory bronchiolitis, follicular bronchiolitis, mineral dust airway disease, constrictive bronchiolitis, diffuse panbronchiolitis, and other rare variants. Prominent bronchiolar involvement may be seen in several interstitial lung diseases, including hypersensitivity pneumonitis, collagen vascular disease, respiratory bronchiolitis-associated interstitial lung disease, cryptogenic organizing pneumonia, and pulmonary Langerhans' cell histiocytosis. Large airway diseases that commonly involve bronchioles include bronchiectasis, asthma, and chronic obstructive pulmonary disease. The clinical and prognostic significance of a bronchiolar lesion is best determined by identifying the etiology, underlying histopathologic pattern and assessing the correlative clinic-physiologic-radiologic context.

Small airways diseases.

The histopathology of the small airways is frequently quite subtle, even in cases with clinically severe disease. In the present paper, we will demonstrate some recognizable straightforward pathological changes in the small airways, and also provide a list of clinico-pathological conditions that should be considered when each is encountered. In the second part of the paper, we will briefly overview some general histological patterns of lesions and specific diseases that may involve the small airways. The basic lesions will be subdivided into inflammatory (acute, chronic, granulomatous, with or without necrosis), proliferative (epithelial or mesenchymal), and remodeling reactions. Inflammatory and proliferative reactions may lead to a the third category of remodeling reactions, characterized by a variety of distortions of normal bronchiolar architecture, including occlusion, constriction, dilatation (with or without mucostasis), tortuosity and nodularity. In addition to this schematic distinction, it is important to recognize that these lesions are frequently combined together and evolve with one another. Each of the 3 reaction patterns may be exquisitely bronchiolar, or may extend in the surrounding parenchyma. In this case, it is important to distinguish between lesions that extend from the bronchiole to the parenchyma or vice versa (such as in organizing pneumonia patterns, where the main lesion is in the parenchyma). As most of these lesions are part of a dynamic process, it is important to recognize that a single causative agent may produce distinct pathologic features at different times in the natural history of the disease. In addition, the same clinical disease may result in a variety of pathologic lesions. Accordingly, there may be not always be an unequivocal relationship between the clinical disease/condition and specific histopathologic lesions in the small airways.

Bronquiolitis constrictiva: ¿qué conocemos acerca de esta enfermedad fibrosante de las vías aéreas?

OBJETIVOS: 1. Identificar la bronquiolitis constrictiva como una enfermedad fibrosante a diferencia de la bronquiolitis proliferativa, una condición inflamatoria de las vías aéreas. 2. Describir las causas de la bronquiolitis constrictiva y su asociación a enfermedades sistémicas. 3. Identificar los hallazgos clínicos, radiológicos y fisiológicos de la bronquiolitis constrictiva. 4. Conocer y entender el manejo y tratamiento de la bronquiolitis constrictiva. 5. Describir la evolución clínica de la bronquiolitos constrictiva. La bronquiolitis constrictiva es una enfermedad respiratoria que afecta las pequeñas vías aéreas y es importante reconocerla debido a su naturaleza fibrosante e irreversible. Los términos utilizados en el mundo para describir esta condicion son variables. En esta revisión, el término Bronquiolitis obliterans constrictiva (BOC) será usado para describir este síndrome que se presenta como una condicion fibrótica de las pequeñas vías aéreas. Patología generalmente utiliza el término bronquiolitis constrictiva y lo usará independientemente si hay o no obliteración total de las vías aéreas; clínicamente la enfermedad se manifiesta, especialmente, cuando hay obliteración de las pequeñas vías aéreas.

Smoking-related interstitial lung disease.

Pulmonary diseases associated with tobacco smoking are a complex group of disorders ranging from chronic obstructive pulmonary disease (COPD) to lung cancer. Interstitial lung diseases (ILDs) have only recently been linked to smoking. The ILDs related to smoking include respiratory bronchiolitis-associated interstitial lung disease, desquamative interstitial pneumonia, and pulmonary Langerhans cell histiocytosis. The relationship of smoking with each of these entities has been largely established on the weight of epidemiologic evidence. Although they have been retained as distinct and separate conditions in various classifications of interstitial lung diseases, these 3 entities share a number of clinical, radiologic, and pathologic features suggesting that they represent a spectrum of patterns of interstitial lung disease occurring in predisposed individuals who smoke. Evaluation of histologic features, particularly in surgical lung biopsy samples, is important in making the distinction between these disorders. However, even after tissue biopsy, it may sometimes be difficult to clearly separate these entities. The importance of making the distinction between them lies in the different clinical management strategies used. Further experimental evidence, including genetic information, may be important in improving our understanding of these diseases.

[Quality of spirometric measurements in children younger than 10 years of age in the light of the recommendations]. / Jakosc badania spirometrycznego u dzieci 10-letnich i mlodszych w swietle zalecen standaryzacyjnych.

INTRODUCTION: In 2005 the European Respiratory Society/American Thoracic Society (ERS/ATS) published an updated document on the standardization of spirometry (European Respiratory Journal 2005; 26: 319-338). It defines criteria for the acceptability of spirometric measurements. The aim of this retrospective study was to assess the adherence to those standards of flow-volume measurements in children younger than 10 years of age. MATERIAL AND METHODS: The analysis was carried out on the results obtained from 233 children aged 4.2-10 years, referred to a spirometric lab during a period of three months. RESULTS: 116 children (all but one preschool) did not cooperate; the results of the 117 who completed the procedure of flow-volume measurement were analysed using ERS/ATS criteria. 80.3% of the children had back extrapolated volume (Vbe) within the defined limit, but only 23.9% had forced expiratory time > 3 s. FEV(1) and FVC were repeatable in 78.6% of the children. When these three criteria were used together, the measurements were acceptable according to ATS/ERS recommendations in 17.1% of the children. Elimination of the forced expiratory time criterion has further increased their number to 63.2%. CONCLUSIONS: Specific recommendations for children should be developed, as the current requirements appear too restrictive, especially regarding the time of forced expiration.

[Non transplant-related constrictive bronchiolitis in adults]. / Bronchiolites constrictives de l'adulte, hors contexte de transplantation.

INTRODUCTION: The term bronchiolitis refers to inflammatory disorders of the bronchioles. Constrictive bronchiolitis is the type most frequently encountered. STATE OF THE ART/PERSPECTIVES: The main clinical manifestations include the development of exertional dyspnoea and fixed airflow obstruction. Chest x-ray findings are usually unhelpful, but CT scanning may reveal a mosaic pattern on expiration. Peripheral micronodules are less frequently seen. The causes of constrictive bronchiolitis are numerous. The diagnosis may be clear from the clinical context when a causative event or predisposing condition can be identified (lung or bone marrow transplantation, toxic fume or gas inhalation, rheumatoid arthritis); in other conditions, a stepwise approach to the diagnosis is usually recommended in order to exclude other causes of subacute or chronic obstructive disease. Formal diagnosis requires histological examination of surgical lung biopsies. Despite corticosteroid administration, respiratory failure usually develops. Specific inhibitors of pro-inflammatory cytokines may offer a new and promising therapeutic approach. CONCLUSIONS: If the clinical context or the radiology and clinical findings are not highly suggestive of a constrictive bronchiolitis, a surgical lung biopsy should be considered.

The interrater reliability of a validated bronchiolitis severity assessment tool.

BACKGROUND: We previously constructed and tested a bronchiolitis severity assessment tool in 2 independent hospitals. The model uses age, work of breathing, dehydration and tachycardia to successfully predict disease severity. OBJECTIVE: To prospectively measure the interrater reliability of a bronchiolitis severity assessment tool and of its component variables. DESIGN: Prospective observational survey. SETTING: A county teaching hospital emergency department serving a mixed urban and rural population with an emergency medicine residency program in 2-3-4 format. SUBJECTS: Thirty-two physicians evaluated a convenience sample of children aged less than 18 months presenting to the emergency department with a clinical diagnosis of bronchiolitis during a single season. METHODS: Two physicians independently examined each patient. Each physician completed a physical examination template that included the variables used in the severity assessment tool. Interrater agreement was measured for the variables work of breathing and dehydration and for the tool as a whole using a weighted kappa statistic. RESULTS: One hundred and forty-six cases were enrolled. Twenty-five were dropped for incomplete data collection. The actual weighted agreement on overall classification was 92%; expected, 73%, kappa = 0.676; P < 0.0001. The actual weighted agreement for dehydration was at 95%; expected, 92%, kappa = 0.305; P = 0.0001. The agreement for work of breathing was 95%; expected, 86%; kappa = 0.611; P < 0.0001. The overall model showed better interrater reliability than its individual components. CONCLUSIONS: Overall interrater reliability for this bronchiolitis severity assessment tool is substantial.

Bronchiolitis: the pathologist's perspective.

The term "bronchiolitis" refers to a broad morphologic spectrum of inflammatory events that are centered on small conducting airways. Bronchiolitis may be an isolated pathologic finding, although it is often a secondary consequence of diseases affecting other parts of the conducting apparatus or pulmonary acinus. Divergent causes of bronchiolitis may have similar microscopic findings. Hence, a pathologic diagnosis is nonspecific and not clinically meaningful unless placed in the context of relevant clinical and radiographic findings. Etiologically, most cases of bronchiolitis are infectious in nature or related to smoking. Increasingly, other causes of bronchiolitis have been recognized, including collagen vascular disease, idiopathic inflammatory bowel disease, or toxins/drugs. Many cases are idiopathic. Histologically, most examples of bronchiolitis demonstrate acute inflammation, usually corresponding to viral infection. Other well-defined histologic categories of bronchiolitis include bronchiolitis obliterans, in which there is fibroblast proliferation within airspaces, and follicular bronchiolitis, corresponding to lymphoid hyperplasia with formation of germinal centers. In recent years, several new forms of bronchiolitis have been recognized, including constrictive bronchiolitis, diffuse panbronchiolitis, and airway-centered fibrosis. This article highlights the clinical and pathologic features of these more recently described entities.