Mounier-Kuhn syndrome in poorly controlled asthma.

INTRODUCTION: Mounier-Kuhn syndrome or tracheobronchomegaly, is a rare condition that consists of abnormal dilation of the trachea and main bronchi due to a pathological arrangement of smooth muscle fibers in this area. CASE REPORT: We present the case of a 46-year-old woman with poorly controlled asthma and recurrent infections, who was diagnosed with Mounier-Kuhn syndrome through a computed tomography scan revealing an unusual enlargement of the trachea with associated bronchiectasis. RESULTS: The diagnosis of Mounier-Kuhn syndrome is radiological, involving measurement of the trachea where a diameter >25 mm in men and >21 mm in women is observed. While diagnosis is sometimes incidental, there is an association with respiratory diseases such as asthma or COPD, hence clinical suspicion is important in patients with poorly controlled underlying conditions who present with recurrent infections, inadequate secretion management, or even hemoptysis. CONCLUSIONS: Despite its rarity, this syndrome significantly impacts patients' quality of life. Diagnosis and management involve comprehensive evaluations including computed tomography, with a multidisciplinary approach including pulmonologists and radiologists. Exploring its clinical features, associations with other respiratory diseases and treatment options is crucial in managing this rare respiratory condition.

Treatment Patterns of Monoclonal Antibodies in Patients With Severe Uncontrolled Asthma Treated by Pulmonologists in Spain.

Introduction and objectives: The use of monoclonal antibody (mAb)-based therapies is becoming the new standard of care for severe uncontrolled asthma (SUA). Even though patients may qualify for one or more of these targeted treatments, based on different clinical criteria, a global vision of mAb prescription management in a large sample of hospitals is not well characterised in Spain.The objective was to give a global vision of mAb prescription management in a large sample of hospitals in Spain. Materials and methods: We used an aggregate data survey method to interview pulmonology specialists in a large sample of Spanish centres (90). The following treatment-related information was obtained on patients treated with mAbs: specific mAbs prescribed, treatment interruption, switch and restart and the reasons for these treatment changes. Results: mAb prescription was more frequent in females (13.3% females vs 7.4% males; p < 0.001). There were no differences in prevalence by hospital complexity level. In contrast, there were differences by geographical area. Omalizumab was the most prescribed mAb (6.2%), followed by mepolizumab (2.9%). Discontinuation of Omalizumab (due to a lack of effectivity) and switches from this mAb to mepolizumab were more frequent. Very few restarts to the first treatment were observed after a switch from ≥2 mAbs. Conclusions: Omalizumab appeared as the most prescribed mAb in SUA but was also the most withdrawn; a specific and objective characterisation of patients with SUA, along with asthma phenotyping, and together with further evaluation of safety and effectiveness profiles, will lead to future progress in the management of SUA with mAbs.

Introducción y objetivos: El uso de terapias basadas en anticuerpos monoclonales (mAb) se está convirtiendo en el nuevo estándar de atención para el asma grave no controlada (AGNC). A pesar de que los pacientes pueden optar a uno o varios de estos tratamientos dirigidos, con base en diferentes criterios clínicos, en España no se ha caracterizado bien una visión global de la gestión de la prescripción de mAb en una gran muestra de hospitales.El objetivo fue dar una visión global de la gestión de la prescripción de mAB en una amplia muestra de hospitales en España. Materiales y métodos: Se utilizó un método basado en una encuesta de datos agregados para entrevistar a especialistas en Neumología en una amplia muestra de centros españoles (90). Se obtuvo la siguiente información relacionada con el tratamiento de los casos tratados con mAbs: mAbs específicos prescritos, interrupción del tratamiento, cambio y reinicio, y las razones de estos cambios de tratamiento en consultas de Neumología. Resultados: La prescripción de mAB fue más frecuente en mujeres (13,3% mujeres vs. 7,4% hombres; p < 0,001). No hubo diferencias de prevalencia por nivel hospitalario. En cambio, hubo diferencias por área geográfica. Omalizumab fue el mAb más prescrito (6,2%), seguido de mepolizumab (2,9%). La interrupción y los cambios (debido a la falta de efectividad) también fueron más frecuentes para omalizumab. Conclusiones: Omalizumab fue el mAb más prescrito en el manejo de AGNC, pero también fue el mAB que presentó más retiradas; una caracterización específica y objetiva de los pacientes con AGNC, mediante fenotipificación de asma, junto con una evaluación adicional de los perfiles de seguridad y efectividad, conducirá a nuevos avances en el manejo del AGNC con mABs.

What is the added value of FeNO as T2 biomarker?

There is increasing evidence about the role of nitric oxide in type 2 (T2) immune response. Fraction of exhaled nitric oxide (FeNO) is a product of airways inflammation and it is increased in patients with asthma. Since Gustaffson published the first article about this biomarker in the 1990s, interest has continued to grow. Compared with other T2 biomarkers such as blood eosinophil count, induced sputum, or serum periostin, FeNO has some remarkable advantages, including its not invasive nature, easy repeatability, and possibility to be performed even in patients with severe airway obstruction. It is considered as an indicator of T2 inflammation and, by the same token, a useful predictor for inhaled steroid response. It is difficult to determine the utility of nitric oxide (NO) for initial asthma diagnosis. In such a heterogenous disease, a single parameter would probably not be enough to provide a complete picture. There is also an important variability among authors concerning FeNO cutoff values and the percentage of sensibility and specificity for diagnosis. Its high specificity indicates a potential role to "rule in" asthma; however, its lower sensibility could suggest a lower capacity to "rule out" this pathology. For this reason, if a diagnosis of asthma is being considered, FeNO should be considered along with other tests. FeNO has also shown its utility to detect response to steroids, adherence to treatment, and risk of exacerbation. Even though there is not enough quality of evidence to establish overall conclusions, FeNO could be an alternative procedure to diagnose or exclude asthma and also a predictive tool in asthma treated with corticosteroids.

The Response to Biologics is Better in Patients with Severe Asthma Than in Patients with Asthma-COPD Overlap Syndrome.

Although biologics have demonstrated to be effective in T2-high asthma patients, there is little experience with these drugs in asthma-COPD overlap (ACO). The aim of this study was to compare the effectiveness of biologics in these two conditions. We included 318 patients (24 ACO and 297 asthma) treated with monoclonal antibodies and followed for at least 12 months. Omalizumab was the most frequently employed biologic agent both in patients with ACO and asthma. Asthma control test (ACT) scores after at least 12 months of biologic therapy were not significantly different between groups. The percentage of patients with ≥1 exacerbation and ≥1 corticosteroid burst was significantly higher in ACO patients (70.8 vs 27.3 and 83.3% vs 37.5%, respectively), whereas the percentage of "controlled" patients (with no exacerbations, no need for corticosteroids and ACT ≥ 20) was significantly lower (16.7% vs 39.7%). In conclusion, this report suggests that patients with ACO treated with biologics reach worse outcomes than asthma patients.

Monoclonal antibody treatment for severe uncontrolled asthma in Spain: analytical map.

BACKGROUND: Monoclonal antibodies (mABs) have become available to treat more efficiently patients with severe uncontrolled asthma (SUA). However, the use of mABs is lower than expected given the prevalence of SUA, with significant disparities in the use of these treatments. OBJECTIVE: To evaluate the proportion of patients with SUA treated with mABs in Spain, and to analyze some of the factors that could determine these prescription patterns. METHODS: An analysis was performed on the data provided from the Hospitals National Health System (NHS) 2018 catalogue where Chest Diseases Department and a Hospital Pharmacy were available. Random sampling was performed to determine the sample size, stratifying proportionally by geographic area and hospital level. Characteristics of the participating sites, as well as the prescribing of mABs were collected, which included geographic area, hospital levels, prescribing medical specialities, types of clinics, and mABs prescribed. RESULTS: Data from 90 hospitals were analyzed (Response rate 64.3%). Level 4 hospitals, the Canary Islands geographical area, and the presence of a high complexity Asthma Healthcare Unit (ACU) were associated with a higher probability that the SUA was treated with mABs. CONCLUSION: The map of the prescribing of mABs for SUA in Spain shows a significant variation by geographic area, hospital level, type of clinic, and the accreditation level of the ACUs. At the current time, there appears to be significant under-prescribing of these treatments.

Rinitis, poliposis nasal y su relación con el asma / Asthma, rhinitis, and nasal polyp multimorbidities

El objetivo de esta revisión es facilitar al neumólogo el manejo de las enfermedades de la vía respiratoria superior ligadas a la vía respiratoria inferior, especialmente al asma, unidas por una epidemiología, una clínica y un mecanismo inflamatorio comunes e interrelacionados. El documento recoge las definiciones de los diferentes fenotipos nasosinusales ligados al asma: rinitis alérgica o no alérgica y rinosinusitis crónica con o sin pólipos nasales. Asimismo se recogen los criterios diagnósticos y su nivel de gravedad. Se dedica especial atención a los dos síndromes principales asociados al asma: 1) rinitis alérgica, la patología más frecuente, y 2) rinosinusitis crónica con pólipos nasales, la patología más ligada al asma grave. En síntesis, en el manejo del asma debe valorarse siempre la vía respiratoria superior con la finalidad de un diagnóstico unificado y un tratamiento integral de la vía respiratoria única

The aim of this review is to assist pulmonologists in the management of diseases involving both the upper and lower respiratory tract that are linked by a common, interrelated epidemiology, clinical signs and symptoms, and inflammatory mechanism − asthma, in particular. The document discusses the definitions of the various sinonasal phenotypes associated with asthma: allergic and non-allergic rhinitis and chronic rhinosinusitis with or without nasal polyps. Diagnostic criteria and severity levels are also listed. Particular attention has been given to the 2 main syndromes associated with asthma: (I) allergic rhinitis, the most common, and (II) chronic rhinosinusitis with nasal polyps, the disease most closely associated with severe asthma. To summarize, the upper respiratory tract should always be evaluated in order to achieve a single diagnosis and comprehensive treatment of the "united airway"

Asthma, rhinitis, and nasal polyp multimorbidities. / Rinitis, poliposis nasal y su relación con el asma.

The aim of this review is to assist pulmonologists in the management of diseases involving both the upper and lower respiratory tract that are linked by a common, interrelated epidemiology, clinical signs and symptoms, and inflammatory mechanism - asthma, in particular. The document discusses the definitions of the various sinonasal phenotypes associated with asthma: allergic and non-allergic rhinitis and chronic rhinosinusitis with or without nasal polyps. Diagnostic criteria and severity levels are also listed. Particular attention has been given to the 2 main syndromes associated with asthma: (i)allergic rhinitis, the most common, and (ii)chronic rhinosinusitis with nasal polyps, the disease most closely associated with severe asthma. To summarize, the upper respiratory tract should always be evaluated in order to achieve a single diagnosis and comprehensive treatment of the "united airway".

Actinomicosis pulmonar en paciente con neumonía eosinófila crónica en tratamiento con omalizumab / Pulmonary Actinomycosis in a Patient with Chronic Eosinophilic Pneumonia treated with Omalizumab

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Normativa sobre asma grave no controlada / Guidelines for Severe Uncontrolled Asthma

Desde la publicación, hace ya 9 años, de la última normativa de la Sociedad Española de Neumología y Cirugía Torácica (SEPAR) sobre asma de control difícil(ACD), se han producido avances en los conocimientos de la enfermedad asmática, que hacen necesario realizar una puesta al día de los datos disponibles e incorporarlos tras su análisis en el nivel de evidencia y recomendación más adecuado. Recientemente han aparecido documentos de consenso y guías de práctica clínica (GPC) que abordan este problema. En esta normativa se hará mención explícita a lo que la previa guía de ACD definía como «verdadera asma de control difícil»; es decir, al asma que tras haber verificado su diagnóstico, realizado un abordaje sistematizado para descartar factores ajenos a la propia enfermedad que conducen a un mal control de la misma («falsa asma de control difícil»), y realizar una estrategia de tratamiento adecuado (escalones 5 y 6 de la Guía española para el manejo del asma [GEMA]), no se consigue alcanzar el control: «asma grave no controlada» (AGNC). En esta línea la normativa propone una revisión de la definición, un intento de clasificación de las diferentes manifestaciones de este tipo de asma, una propuesta del abordaje diagnóstico por pasos y un tratamiento dirigido según fenotipo, conjuntamente con un apartado específico sobre este arquetipo de asma en la infancia, con el objetivo de que pueda servir de ayuda a los profesionales sanitarios y repercutir en el cuidado de estos pacientes

Since the publication, 9 years ago, of the latest SEPAR (Spanish Society of Pulmonology and Thoracic Surgery) Guidelines on Difficult-to-Control Asthma (DCA), much progress has been made in the understanding of asthmatic disease. These new data need to be reviewed, analyzed and incorporated into the guidelines according to their level of evidence and recommendation. Recently, consensus documents and clinical practice guidelines (CPG) addressing this issue have been published. In these guidelines, specific mention will be made of what the previous DCA guidelines defined as «true difficult-to-control asthma». This is asthma that remains uncontrolled after diagnosis and a systematic evaluation to rule out factors unrelated to the disease itself that lead to poor control («false difficult-to-control asthma»), and despite an appropriate treatment strategy (Spanish Guidelines for the Management of Asthma [GEMA] steps 5 and 6): severe uncontrolled asthma. In this respect, the guidelines propose a revised definition, an attempt to classify the various manifestations of this type of asthma, a proposal for a stepwise diagnostic procedure, and phenotype-targeted treatment. A specific section has also been included on DCA in childhood, aimed at assisting healthcare professionals to improve the care of these patients

Guidelines for severe uncontrolled asthma.

Since the publication, 9 years ago, of the latest SEPAR (Spanish Society of Pulmonology and Thoracic Surgery) Guidelines on Difficult-to-Control Asthma (DCA), much progress has been made in the understanding of asthmatic disease. These new data need to be reviewed, analyzed and incorporated into the guidelines according to their level of evidence and recommendation. Recently, consensus documents and clinical practice guidelines (CPG) addressing this issue have been published. In these guidelines, specific mention will be made of what the previous DCA guidelines defined as "true difficult-to-control asthma". This is asthma that remains uncontrolled after diagnosis and a systematic evaluation to rule out factors unrelated to the disease itself that lead to poor control ("false difficult-to-control asthma"), and despite an appropriate treatment strategy (Spanish Guidelines for the Management of Asthma [GEMA] steps 5 and 6): severe uncontrolled asthma. In this respect, the guidelines propose a revised definition, an attempt to classify the various manifestations of this type of asthma, a proposal for a stepwise diagnostic procedure, and phenotype-targeted treatment. A specific section has also been included on DCA in childhood, aimed at assisting healthcare professionals to improve the care of these patients.

Normativa sobre el estudio de la hiperrespuesta bronquial inespecífica en el asma / Guidelines for the Study of Nonspecific Bronchial Hyperresponsiveness in Asthma

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Factores relacionados con el mayor porcentaje de ingresos por asma en mujeres. Estudio FRIAM / Factors Related With the Higher Percentage of Hospitalizations Due to Asthma Amongst Women: the FRIAM Study

Introducción: Las mujeres ingresan por exacerbaciones de asma con mayor porcentaje que los varones, sin que se conozcan claramente los motivos. El objetivo del estudio es explorar las posibles causas que expliquen este hecho. Pacientes y métodos: Estudio prospectivo multicéntrico en pacientes ingresados por asma durante 2 años en servicios de neumología. Mediante cuestionario se recogieron: características demográficas, variables previas al ingreso y durante el mismo que incluían adherencia, ansiedad-depresión, hiperventilación, y control del asma. Resultados: Se recogieron datos de 183 ingresos: 115 mujeres (62,84%). Las mujeres eran de mayor edad (52,4±18,3/43,4±18,7; p=0,02); había mayor porcentaje con prescripción de corticosteroides inhalados (63,2/47,1%; p=0,03) y síndrome de hiperventilación (57,3/35,9%; p=0,02), y una estancia media mayor (7,3±3,4/5,9±3,6; p=0,02). El porcentaje de fumadoras era menor (21,2/38,8%; p=0,01), y el FEV1 al ingreso era más bajo (58,2%±15,9/67,5%±17,4; p=0,03).En el rango de 40 a 60 años se demostró una asociación entre ser mujer y la variable ingresos previos. De forma independiente, también se asociaron el sexo y la obesidad, con un OR de 16,1 (IC 95%, 1,6-156,7) y de 4,8 (IC 95%, 1,06-22), respectivamente. Conclusiones: El porcentaje de ingresos por asma fue mayor en mujeres. Ser mujer entre 40 y 60 años se asocia con ingresos previos y constituye un factor de riesgo para ingreso por asma, en el que la hipótesis hormonal durante el climaterio podría explicar parte de los hechos. La poliposis y la obesidad son factores de riesgo, independientemente del sexo(AU)

Introduction: The reason why there is a higher hospitalization rate due to asthma exacerbations amongst women is unclear. The objective of this study is to explore the possible causes that may explain this fact. Methods: A multi-center, prospective study including asthma patients hospitalized in the pulmonary medicine departments during a two-year period. By means of a questionnaire, the following data were collected: demographic characteristics and treatment compliance, anxiety-depression, hyperventilation and asthma control, both prior to and during the hospitalization. Results:183 patients were included, 115 (62.84%) of whom were women. The women were older (52.4±18.3/43.4±18.7; P=.02), were more frequently prescribed inhaled corticosteroids (63.2%/47.1%; P=.03) and had a higher rate of hyperventilation syndrome (57.3/35.9; P=.02) and a longer mean hospital stay (7.3±3.4/5.9±3.6; P=.02). The percentage of smokers among the women was lower (21.2%/38.8%; p=0.01) and the FEV1 was lower at admittance (58.2% ±15.9/67.5%±17.4; P=.03).In the 40 to 60-year-old age range, an association was demonstrated between being female and the «previous hospitalizations» variable (OR, 16.1; 95% CI, 1.6-156.7); sex and obesity were also independently associated (OR, 4.8; 95% CI, 1.06-22).Conclusions: In this cohort, the rate of hospitalization for asthma was higher in women than in men. Being a woman between the ages of 40 and 60 is associated with previous hospitalizations and is a risk factor for asthma-related hospitalization. This situation could partially be explained by the hormonal changes during menopause, where polyposis and obesity are independent risk factors(AU)

Factors related with the higher percentage of hospitalizations due to asthma amongst women: the FRIAM study.

INTRODUCTION: The reason why there is a higher hospitalization rate due to asthma exacerbations amongst women is unclear. The objective of this study is to explore the possible causes that may explain this fact. METHODS: A multi-center, prospective study including asthma patients hospitalized in the pulmonary medicine departments during a two-year period. By means of a questionnaire, the following data were collected: demographic characteristics and treatment compliance, anxiety-depression, hyperventilation and asthma control, both prior to and during the hospitalization. RESULTS: 183 patients were included, 115 (62.84%) of whom were women. The women were older (52.4 ± 18.3/43.4 ± 18.7; P=.02), were more frequently prescribed inhaled corticosteroids (63.2%/47.1%; P=.03) and had a higher rate of hyperventilation syndrome (57.3/35.9; P=.02) and a longer mean hospital stay (7.3 ± 3.4/5.9 ± 3.6; P=.02). The percentage of smokers among the women was lower (21.2%/38.8%; p=0.01) and the FEV(1) was lower at admittance (58.2% ± 15.9/67.5% ± 17.4; P=.03). In the 40 to 60-year-old age range, an association was demonstrated between being female and the 'previous hospitalizations' variable (OR, 16.1; 95% CI, 1.6-156.7); sex and obesity were also independently associated (OR, 4.8; 95% CI, 1.06-22). CONCLUSIONS: In this cohort, the rate of hospitalization for asthma was higher in women than in men. Being a woman between the ages of 40 and 60 is associated with previous hospitalizations and is a risk factor for asthma-related hospitalization. This situation could partially be explained by the hormonal changes during menopause, where polyposis and obesity are independent risk factors.