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BACKGROUND: Interstitial lung diseases (ILD) include a wide range of diseases impacting lung parenchyma and leading to fibrosis and architectural distortion. Chronic cough and dyspnea are common symptoms which affect the quality of life (QoL) in ILD patients. The mechanisms of cough in ILD patients are still unknown. The aim of this study was to prospectively investigate histological, radiological, and physiological determinants of cough-related QoL in ILD patients who underwent transbronchial lung cryobiopsy (TBLC). METHODS: All patients (n = 111) filled in The Leicester Cough Questionnaire (LCQ) and The St George's Respiratory Questionnaire (SGRQ). They underwent lung function tests, forced vital capacity (FVC), forced vital expiratory volume in 1 s (FEV1), diffusion capacity to carbon monoxide (DLCO), high-resolution computed tomography (HRCT), and blood samples before diagnostic TBLC. Two experienced radiologists assessed the extents of following HRCT patterns: ground-glass opacities (GGO), honeycombing, reticulation, traction bronchiectasis, and emphysema. Histology of TBLC were re-analyzed by two experienced pulmonary pathologists and the presence of fibroblast foci, fibrosis, giant cells, granulomas, and honeycombing were recorded. RESULTS: In the median multivariate regression analysis, BMI (-0.19; 95% CI -0.37- -0.014; p 0.035), GGO (-0.38; 95% CI -0.61- -0.15; p 0.001), granulomas (-3.21; 95% CI -6.12- -0.30; p 0.031), and current smoking (2.49; 95% CI 0.12-4.86; p 0.040) showed independent associations with LCQ total score. BMI (1.3; 95% CI 0.20-2.42; p 0.021) and DLCO (-0.51; 95% CI -0.85 - -0.16; p 0.004) showed independent association with SGRQ total score. CONCLUSION: Determinants of cough-related QoL in ILD patients are multifactorial including physiological, radiological and histological parameters.
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Tos , Enfermedades Pulmonares Intersticiales , Calidad de Vida , Tomografía Computarizada por Rayos X , Humanos , Enfermedades Pulmonares Intersticiales/fisiopatología , Masculino , Femenino , Persona de Mediana Edad , Anciano , Estudios Prospectivos , Encuestas y Cuestionarios , Pulmón/diagnóstico por imagen , Pulmón/patología , Pulmón/fisiopatología , Pruebas de Función Respiratoria , Capacidad Vital , Índice de Masa Corporal , Volumen Espiratorio Forzado , Biopsia , Análisis MultivarianteRESUMEN
BACKGROUND: Most patients with idiopathic pulmonary fibrosis (IPF) complain of cough. IPF-associated cough is widely characterized as dry or non-productive. The aim of this study was to compare chronic cough in early stage IPF patients to cough in subjects with chronic cough from a community-based sample and, especially, to investigate whether cough in IPF is less productive than chronic cough in a community-based sample. METHODS: The IPF cough population consisted of 46 biopsy-confirmed patients who complained of chronic cough. Control population consisted of subjects with chronic cough, gathered by a community-based email survey sent to public service employees and the Finnish Pensioners' Federation. A case-control setting was applied by having four age, gender, and smoking-status matched subjects from the community sample for each IPF cough patient. A cough specific quality of life questionnaire (Leicester Cough Questionnaire (LCQ)) was filled in by all subjects. The LCQ questionnaire contains 19 questions, each question is scored from 1 to 7 and total score from 3 to 21 with a smaller value indicating more severe impairment. RESULTS: The sputum production frequency, as assessed by LCQ question 2, was 5.0 (3.0-6.0) in the IPF chronic cough population and 5.0 (3.0-6.0) in the community-based chronic cough population (median and interquartile range p= 0.72). The LCQ total score was 14.8 (11.5-18.1) in the IPF chronic cough population and 15.4 (13.0-17.5) in the community-based chronic cough population (p=0.76). The domain impact scores were physical, 4.9 (3.9-6.1) vs. 5.1 (4.5-5.6) (p=0.80); psychological, 4.6 (3.7-5.9) vs. 4.7 (3.9-5.7) (p=0.90); and social, 5.5 (3.7-6.5) vs. 5.5 (4.5-6.3) (p=0.84), respectively. Furthermore, cough response to paint or fumes, cough disturbing sleep, and cough frequency per day did not differ between the groups. CONCLUSION: Cough in early stage IPF patients was not distinguishable from chronic cough in the community-based population by LCQ. Especially, there was no difference in the self-reported frequency of cough-associated sputum production.
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Tos , Fibrosis Pulmonar Idiopática , Humanos , Estudios de Casos y Controles , Calidad de Vida , AutoinformeRESUMEN
BACKGROUND: The disease course of idiopathic pulmonary fibrosis (IPF) is progressive and occasionally, other types of interstitial lung disease (ILD) may progress similarly to IPF. This study aimed to evaluate risk factors for disease progression within 24 months in patients with various ILDs. METHODS: This prospective study obtained 97 patients with a suspected ILD who underwent a transbronchial lung cryobiopsy. The extent of several high-resolution computed tomography (HRCT) patterns was assessed. Due to the inclusion criteria the study population presented a low extent of honeycombing and definite usual interstitial pneumonia (UIP) pattern on HRCT suggesting an early stage of ILD. Disease progression within 24 months despite treatment was defined as a relative decline of ≥ 10% in forced vital capacity (FVC), or a relative decline in FVC of ≥ 5% and one of the three additional criteria: (1) a decline in diffusion capacity to carbon monoxide (DLCO) ≥ 15%; (2) increased fibrosis on HRCT; (3) progressive symptoms, or progressive symptoms and increased fibrosis on HRCT. The same definition was utilized in patients with IPF and other ILDs. Risk factors for disease progression were evaluated in a multivariable logistic regression model. RESULTS: Disease progression was revealed in 52% of the patients with ILD, 51% of the patients with IPF, and 53% of the patients with other types of ILD. A high extent of reticulation on HRCT (Odds ratio [OR] 3.11, 95% Confidence interval [CI] 1.21-7.98, P = 0.019) and never smoking (OR 3.11, CI 1.12-8.63, P = 0.029) were associated with disease progression whereas platelet count (OR 2.06 per 100 units increase, CI 0.96-4.45, P = 0.065) did not quite reach statistical significance. CONCLUSION: Higher extent of reticulation on HRCT and never smoking appeared to associate with the risk of disease progression within 24 months in ILD patients without honeycombing. Approximately half of the patients with ILD revealed disease progression, and similar proportions were observed in patients with IPF and in other types of ILD.
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Fibrosis Pulmonar Idiopática , Enfermedades Pulmonares Intersticiales , Progresión de la Enfermedad , Fibrosis , Humanos , Fibrosis Pulmonar Idiopática/diagnóstico , Enfermedades Pulmonares Intersticiales/diagnóstico por imagen , Estudios Prospectivos , Tomografía Computarizada por Rayos X/métodosRESUMEN
BAL cell differential counts of 133 therapy naive ILD patients and 43 patients during acute exacerbation of ILD (AE-ILD) were retrospectively evaluated. In the 20 patients who underwent BAL both at baseline and during an AE-ILD, there was an increase in neutrophils but a decrease in macrophages and eosinophils in the BAL obtained during AE-ILD. A detectable number of basophils at the baseline was a novel risk factor for earlier death and the occurrence of AE-ILD. Total BAL cell count >160 × 109/L during AE-ILD was correlated with a more favorable prognosis. BAL cell counts <20% of lymphocytes or > 20% of neutrophils during AE-ILD were associated with shorter survival. AE-ILD exerted significant changes in BAL cell profiles in individual patients. Several BAL-parameters correlated with survival of ILD patients; of these, baseline basophils and total cell count during AE-ILD were novel prognostic markers.
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Basófilos/inmunología , Líquido del Lavado Bronquioalveolar/citología , Líquido del Lavado Bronquioalveolar/inmunología , Enfermedades Pulmonares Intersticiales/inmunología , Macrófagos/inmunología , Neutrófilos/inmunología , Enfermedad Aguda , Anciano , Anciano de 80 o más Años , Recuento de Células , Femenino , Humanos , Enfermedades Pulmonares Intersticiales/mortalidad , Masculino , Persona de Mediana Edad , Pronóstico , Estudios RetrospectivosRESUMEN
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial pneumonia with an unpredictable course. The aims of this study were to retrospectively re-evaluate a cohort of patients with IPF according to the 2011 international IPF guidelines and 1) to characterize the subgroups of patients when classified according to their observed survival times and 2) to evaluate whether Composite Physiologic Index (CPI), Gender-Age-Physiology (GAP) Index or clinical variables could predict mortality. METHODS: Retrospective data was collected and patients were classified into subgroups according to their observed lifespans. Differences in clinical variables, CPI and GAP stages as well as in comorbidities were investigated between the subgroups. Predictors of mortality were identified by COX proportional hazard analyses. RESULTS: A total of 132 patients were included in this study. The disease course was rapid (≤ 2 years) in 30.0%, moderate (2-5 years) in 28.0% and slow (≥ 5 years) in 29.0% of the patients. Pulmonary function tests (PFT) and CPI at baseline differentiated significantly between the rapid disease course group and those patients with longer survival times. However, the predictive accuracy of the investigated clinical variables was mainly less than 0.80. The proportions of patients with comorbidities did not differ between the subgroups, but more patients with a rapid disease course were diagnosed with heart failure after the diagnosis of IPF. Most patients with a rapid disease course were categorized in GAP stages I and II, but all patients in GAP stage III had a rapid disease course. The best predictive multivariable model included age, gender and CPI. GAP staging had slightly better accuracy (0.67) than CPI (0.64) in predicting 2-year mortality. CONCLUSIONS: Although the patients with a rapid disease course could be differentiated at baseline in terms of PFT and CPI, the predictive accuracy of any single clinical variable as well as CPI and GAP remained low. GAP staging was unable to identify the majority of patients with a rapid disease progression. It is challenging to predict disease progression and mortality in IPF even with risk prediction models.
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Progresión de la Enfermedad , Fibrosis Pulmonar Idiopática/clasificación , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Fibrosis Pulmonar Idiopática/complicaciones , Masculino , Persona de Mediana Edad , Estudios RetrospectivosRESUMEN
BACKGROUND: The most common cause of death of patients with idiopathic pulmonary fibrosis (IPF) has been reported to be the lung disease itself and mortality from IPF appears to be increasing. However, the causes of death in patients with IPF taking into account differences between genders and smoking histories as well as disease progression, have not been previously explored. METHODS: Retrospective data from hospital register and death certificates from national database of IPF patients treated in Kuopio University Hospital (KUH) from 2002 to 2012 were collected. Mortality was also explored from the death registry database via ICD-10 code J84 revealing the numbers of deaths from pulmonary fibrosis in Finland from 1998 to 2015. RESULTS: Out of 117 deaths, 26.5% were females and 73.5% males in KUH. The most common underlying causes of death were IPF 67.5% and ischemic heart diseases 14.8%. More males died for reasons other than IPF (39.5%) compared to females (12.9%) (p = 0.007). Pneumonia as the immediate cause of death was more common in males (27.9%) than in females (3.2%) (p = 0.004) and in ex-smokers (32.7%) compared to non-smokers (9.3%) (p = 0.007). Death register based mortality from pulmonary fibrosis is increasing in Finland. CONCLUSIONS: Even though the overall mortality was higher in males with IPF, the disease-specific mortality for IPF was higher in females i.e. in males, comorbidities were more often the underlying causes of death. Pneumonia-triggered acute exacerbations of IPF may be associated with smoking and gender since females and non-smokers were less likely to succumb to pneumonia. We conclude that disease progression at the end of life may vary depending on smoking habits and gender.
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Certificado de Defunción , Fibrosis Pulmonar Idiopática , Isquemia Miocárdica/epidemiología , Neumonía/epidemiología , Factores Sexuales , Fumar/epidemiología , Anciano , Anciano de 80 o más Años , Causas de Muerte , Comorbilidad , Progresión de la Enfermedad , Femenino , Finlandia/epidemiología , Humanos , Fibrosis Pulmonar Idiopática/diagnóstico , Fibrosis Pulmonar Idiopática/mortalidad , Fibrosis Pulmonar Idiopática/fisiopatología , Masculino , Persona de Mediana Edad , Mortalidad , Factores de Riesgo , Brote de los SíntomasRESUMEN
BACKGROUND: Cigarette smoking has been associated with the risk of idiopathic pulmonary fibrosis (IPF). Certain comorbidities have been associated with reduced survival although some studies have indicated that current smokers have a longer survival than ex-smokers. Comorbidities in relation to smoking history have not been previously analyzed. METHODS: Retrospective data was collected and patients were categorized according to gender and smoking habits. Comorbidities and medications were collected. Predictive values for mortality were identified by COX proportional hazard analyses. RESULTS: We examined 45 non-smokers (53.3% female), 66 ex-smokers (9.1% female) and 17 current smokers (17.6% female) with IPF. Current smokers were younger at baseline (58.1 ± 8.74 years) compared to non-smokers (71.4 ± 8.74, p < 0.001) and ex-smokers (72.5 ±7.95, p <0.001). Median survival of non-smokers and current smokers was longer (55.0 and 52.0 months, respectively) than that of ex-smokers (36.0 months) (p=0.028 and 0.034, respectively). In age and severity adjusted analyses, smoking was not related to survival. Cardiovascular diseases (CVD) (72.7 %) were the most common comorbidities, current smokers had more chronic obstructive pulmonary disease (COPD) and lung cancer compared to ex-smokers (p<0.001). CVD, COPD and use of insulin were related to poorer survival in adjusted analyses. CONCLUSIONS: Smoking seems to influence the course of disease in IPF since current smokers developed the disease at a younger age in comparison to non-smokers and ex-smokers. No significant differences in the major comorbidities were detected between IPF patients with different smoking histories. The mechanism through which smoking influences IPF progression requires further investigation.
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Fibrosis Pulmonar Idiopática/etiología , Fibrosis Pulmonar Idiopática/mortalidad , Fumar/efectos adversos , Fumar/mortalidad , Anciano , Anciano de 80 o más Años , Comorbilidad , Femenino , Humanos , Fibrosis Pulmonar Idiopática/diagnóstico por imagen , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Tasa de Supervivencia/tendenciasRESUMEN
BACKGROUND: The long-term prognosis of chronic cough and its determinants need to be clarified. METHODS: This is a prospective, observational cohort study. Eighty-nine unselected subjects with chronic (> 8 weeks' duration) cough were carefully investigated: Clinical examination, symptom questionnaire, Leicester Cough Questionnaire (LCQ), skin prick tests, ambulatory peak expiratory flow monitoring, spirometry before and after 0.4 mgs of salbutamol, exhaled nitric oxide concentration measurement, hypertonic saline cough provocation test, and histamine bronchial provocation test. After five years, a letter was sent to the subjects containing questions about continuation of cough, smoking, indoor exposures, presence of co-morbidities, and current medication. It also contained LCQ and Cough Clinic diagnostic questionnaire. Sixty-eight subjects (76%) responded. RESULTS: At five years, continuing regular cough was present in 31 (46%) of the subjects and continuing impairment in cough-related quality of life (less than 1.3 points' improvement in LCQ) in 32 (47%). Continuing regular cough was associated with presence of chronic rhinitis or esophageal reflux disease, baseline mild airway responsiveness to histamine, and baseline strong cough responsiveness to hypertonic saline. Continuing impairment in cough-related quality of life was associated with high body mass index, absence of atopy, absence of pets, and high number of background disorders (esophageal reflux disease, asthma, or chronic rhinitis). CONCLUSIONS: Almost half of subjects with chronic cough suffered of the disorder at five years from initial assessment. Several possible determinants of poor prognosis could be identified.
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Asma/epidemiología , Tos/diagnóstico , Reflujo Gastroesofágico/epidemiología , Rinitis/epidemiología , Anciano , Albuterol/uso terapéutico , Pruebas de Provocación Bronquial , Enfermedad Crónica , Comorbilidad , Tos/tratamiento farmacológico , Femenino , Volumen Espiratorio Forzado , Humanos , Modelos Logísticos , Masculino , Persona de Mediana Edad , Óxido Nítrico/análisis , Pronóstico , Estudios Prospectivos , Calidad de Vida , Espirometría , Encuestas y CuestionariosRESUMEN
BACKGROUND: Risk predicting models have been applied in idiopathic pulmonary fibrosis (IPF), but still not validated in patients with rheumatoid arthritis-associated interstitial lung disease (RA-ILD). The purpose of this study was to test the suitability of three prediction models as well as individual lung function and demographic factors for evaluating the prognosis of RA-ILD patients. METHODS: Clinical and radiological data of 59 RA-ILD patients was re-assessed. GAP (gender, age, physiologic variables) and the modified interstitial lung disease (ILD)-GAP as well as the composite physiologic indexes (CPI) were tested for predicting mortality using the goodness-of-fit test and Cox model. Potential predictors of mortality were also sought from single lung function parameters and clinical characteristics. RESULTS: The median survival was 152 and 61 months in GAP / ILD-GAP stages I and II (p = 0.017). Both GAP and ILD-GAP models accurately estimated 1-year, 2-year and 3-year mortality. CPI (p = 0.025), GAP (p = 0.008) and ILD-GAP (p = 0.028) scores, age (p = 0.002), baseline diffusion capacity to carbon monoxide (DLCO) (p = 0.014) and hospitalization due to respiratory reasons (p = 0.039), were significant predictors of mortality in the univariate analysis, whereas forced vital capacity (FVC) was not predictive. CPI score (HR 1.03, p = 0.018) and baseline DLCO (HR 0.97, p = 0.011) remained significant predictors of mortality after adjusting for age. CONCLUSIONS: GAP and ILD-GAP are applicable for evaluating the risk of death of patients with RA-ILD in a similar manner as in those with IPF. Baseline DLCO and CPI score also predicted survival.
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Artritis Reumatoide/complicaciones , Enfermedades Pulmonares Intersticiales/diagnóstico , Enfermedades Pulmonares Intersticiales/mortalidad , Índice de Severidad de la Enfermedad , Factores de Edad , Anciano , Estudios de Cohortes , Femenino , Finlandia/epidemiología , Humanos , Masculino , Persona de Mediana Edad , Modelos Teóricos , Pronóstico , Modelos de Riesgos Proporcionales , Tasa de Supervivencia , Tomografía Computarizada por Rayos X , Capacidad VitalRESUMEN
BACKGROUND: In rheumatoid arthritis-associated interstitial lung disease (RA-ILD), occurring in 10 % of patients with patients with RA, usual interstitial pattern (UIP) has shown to associate with poor prognosis but more detailed data about the course of the disease in different subtypes is limited. Our aim was to compare the disease course of patients with RA-ILD categorized into either UIP or other types of ILDs. METHODS: Clinical and radiological information of 59 patients with RA-ILD were re-assessed and re-classified into UIP or non-UIP groups, followed by a between-group comparison of demographic data, lung function, survival, cause of death and comorbidities. RESULTS: The majority of patients (n = 35/59.3 %) showed a radiological UIP-like pattern in high resolution computed tomography. The median survival was 92 months (95 % CI 62.8-121.2) in the UIP-group and 137 months (95 % CI 31.0-243.0) in the non-UIP-group (p = 0.417). Differences in course of disease were found in the number of hospitalizations for respiratory reasons (mean 1.9 ± 2.6 in UIP vs. 0.5 ± 0.9 in non-UIP group, p = 0.004), the use of oxygen therapy (8/22.9 % UIP patients vs. 0 non-UIP patients, p = 0.016), number of deaths (23/65.7 % vs. 10/41.7 %, p = 0.046) and decline in diffusion capacity (56 ± 20.6 vs. 69 ± 20.2, p = 0.021). Dyspnea and inspiratory crackles were detected more often in the UIP group. RA-ILD was the most common primary cause of death (39.4 % of cases). Hypertension, coronary artery disease, chronic obstructive pulmonary disease, heart insufficiency, diabetes and asthma were common comorbidities. ILD preceded RA diagnosis in 13.6 % of patients. CONCLUSIONS: The course of the disease in RA-UIP patients is different from the other RA-ILD subtypes. Several comorbidities associated commonly with RA-ILD, although ILD was the predominant primary cause of death.
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Artritis Reumatoide/complicaciones , Enfermedades Pulmonares Intersticiales/clasificación , Enfermedades Pulmonares Intersticiales/diagnóstico por imagen , Anciano , Causas de Muerte , Comorbilidad , Femenino , Finlandia , Humanos , Enfermedades Pulmonares Intersticiales/complicaciones , Masculino , Persona de Mediana Edad , Tomografía Computarizada por Rayos XRESUMEN
The prevalence of chronic cough is 10 to 15%. It has a strong negative impact on the patients' quality of life and it often causes depression. Many patients find medications unhelpful. Successful management of chronic cough requires the identification of the underlying condition like chronic rhinosinusitis, asthma, and asthma-like syndrome, and esophageal reflux disease. If the underlying condition cannot be identified or if the drug trials fail to help, the patient probably suffers from idiopathic chronic cough. A new paradigm has been introduced in which chronic cough is regarded as a primary condition.
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Tos , Enfermedad Crónica , Tos/complicaciones , Tos/epidemiología , Tos/etiología , Tos/prevención & control , Depresión/etiología , Humanos , Prevalencia , Calidad de VidaRESUMEN
Typical symptoms of bronchiectasis include cough, sliminess of the airways and recurrent respiratory infections which lead to lung injury and impaired quality of life when untreated. High-resolution computer tomography is the most important examination to detect bronchiectasias. Cornerstones of the treatment are elucidation of the etiology of the disease, taking care of vaccinations, management of physical fitness, treating sliminess, and careful treatment of periods of exacerbation with antibiotics according to bacterial growth and susceptibility testing determined from sputum. Hypertonic saline inhalation can be used to potentiate the treatment of sliminess and achieve a positive effect on the quality of life.
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Bronquiectasia/diagnóstico , Bronquiectasia/terapia , Administración por Inhalación , Antibacterianos/uso terapéutico , Humanos , Calidad de Vida , Solución Salina Hipertónica/uso terapéutico , Esputo/microbiología , Tomografía Computarizada por Rayos XRESUMEN
One out of five working persons with asthma has work-related respiratory symptoms. When exploring the symptoms of a working-age patient it is essential to survey the job description and working conditions. Early intervention in the factors aggravating the respiratory symptoms will decrease morbidity, maintain working capacity and improve the quality of life. Occupational health service and the employer play a central role in identifying and decreasing the exposure factors in the working environment as well as in patient guidance for asthma therapy and protecting from the stimuli. The working capacity of an asthmatic person can be improved by applying vocational rehabilitation.
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Asma Ocupacional/etiología , Asma Ocupacional/prevención & control , Exposición Profesional/efectos adversos , Asma Ocupacional/epidemiología , Humanos , Perfil Laboral , Salud Laboral , Calidad de Vida , Rehabilitación Vocacional , Factores de RiesgoRESUMEN
Irritant-induced asthma is a rare disease, usually being caused by an accidental or other exceptionally strong exposure to substances irritating the respiratory passages. High-dose inhaled corticosteroid medication is immediately started at the emergency call service. If severe exposure is suspected, it is important to monitor the patient at least for a couple of days in hospital. Immediately after the acute stage diagnostic investigations are carried out, including a metacholine or histamine challenge test, since demonstration of airway hyperreactivity is of diagnostic and prognostic significance. The asthma may remain permanent.
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Asma/inducido químicamente , Irritantes/envenenamiento , Administración por Inhalación , Corticoesteroides/administración & dosificación , Pruebas de Provocación Bronquial , Humanos , Exposición por Inhalación , PronósticoRESUMEN
Multidisciplinary meeting (MDM) is a core element in the diagnosis of interstitial lung diseases (ILD). The aim of the study was to investigate the implementation and key elements related to ILD MDMs in Finnish specialized care, which is characterized by long travel distances and a large number of small centers treating patients suffering from ILDs. An electronic questionnaire was sent to ILD experts working at five academic centers of Finland regarding the implementation of ILD MDMs with the focus on utilization of virtual communication. Responses were received from all academic centers of Finland (n = 5) whose catchment areas cover all of Finland. ILD MDMs were organized in each center approximately every two weeks and the core participants included a radiologist, respiratory physicians, junior staff, pathologist and a rheumatologist. All non-academic centers could refer their patients to be evaluated in ILD MDM of an academic center. Virtual communication was utilized by all academic centers in the implementation of ILD MDMs, being most common among small centers located in Eastern and Northern Finland. Virtual access to ILD MDM of an academic center was available in most parts of Finland, enabling small centers to benefit from the ILD expertise of academic centers.
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Background: Acute onset of interstitial lung disease (ILD) has been described in patients with idiopathic inflammatory myositis (IIM), but controlled studies about this issue are sparse. The aim of this study was to compare disease onset, demographics, and high-resolution computed tomography (HRCT) patterns in IIM-ILD and other connective tissue disease (CTD)-ILDs. Methods: Clinical and radiological data of 22 IIM-ILD and 132 other CTD-ILD patients was retrospectively gathered from hospital registries between January 2000 and November 2019. Data was re-assessed and compared using a multivariate analysis. Results: Compared to other CTD-ILDs, the patients with IIM-ILD were younger (59.7 vs. 68.0 years, P=0.023), more often non-smokers (71.4% vs. 45.7%, P=0.029) and displayed radiological nonspecific interstitial pneumonia/organizing pneumonia (NSIP/OP) overlap pattern more frequently (27.3% vs. 1.5%, P<0.001). The onset of ILD was acute with patients needing intensive care significantly more often in IIM-ILD than in other CTD-ILDs (22.7% vs. 2.3%, P<0.001). In most patients ILD was diagnosed before or simultaneously with IIM presentation unlike in other CTD-ILDs (90.9% vs. 47.7%, P<0.001). In multivariate analysis, NSIP/OP overlap pattern, acute onset disease treated in intensive care unit and ILD preceding or being diagnosed simultaneously with CTD were significantly associated with IIM-ILD. The multivariate model, supplemented with age, had excellent diagnostic performance identifying IIM-ILD [area under curve (AUC) 0.845]. Conclusions: Unlike other CTD-ILDs, IIM-ILD often develops acutely, simultaneously with the systemic disease. Therefore, clinicians should consider IIM-ILD as an option of differential diagnosis in patients with acute ILD and promptly test muscle enzymes as well as comprehensive autoantibody tests.
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INTRODUCTION: The previous data concerning the prevalence of idiopathic pulmonary fibrosis (IPF) and the frequency of antifibrotic drug use in Finland were based on research registries and medical records whereas nationwide data on the number of patients with IPF in specialised care and those on antifibrotic treatment have not been published. METHODS: We made an information request to the Finnish National Hospital Discharge Register (Hilmo) covering the whole population of Finland to find out the annual numbers of patients with IPF treated in specialised care in 2016-2021. The numbers of the patients initiating and using pirfenidone and nintedanib were requested from the Social Insurance Institution of Finland (Kela) for the same time period. RESULTS: The estimated prevalence of IPF in specialised care was 36.0 per 100 000 in 2021, having increased since 2016. The number of antifibrotic drug users and their proportion of outpatients with IPF had also risen during the follow-up period. In 2021, 35% of the patients with IPF used pirfenidone or nintedanib. The number of inpatients treated in specialised care because of IPF had declined during 2016-2021. CONCLUSIONS: The prevalence of IPF was higher than expected in Finnish specialised care and had increased during the 6-year follow-up time. The increase in the number of patients with IPF using antifibrotic drugs might have diminished the need for IPF-related hospitalisations.
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Fibrosis Pulmonar Idiopática , Humanos , Finlandia/epidemiología , Prevalencia , Fibrosis Pulmonar Idiopática/tratamiento farmacológico , Fibrosis Pulmonar Idiopática/epidemiologíaRESUMEN
Mast cells (MCs) are known to be involved in the pathogenesis of idiopathic pulmonary fibrosis (IPF), although their role in acute exacerbations of IPF has not been investigated. The aims of the study were to evaluate the numbers of MCs in fibrotic and non-fibrotic areas of lung tissue specimens of idiopathic pulmonary fibrosis (IPF) patients with or without an acute exacerbation of IPF, and to correlate the MC density with clinical parameters. MCs of IPF patients were quantified from surgical lung biopsy (SLB) specimens (n = 47) and lung tissue specimens taken at autopsy (n = 7). MC density was higher in the fibrotic areas of lung tissue compared with spared alveolar areas or in controls. Female gender, low diffusion capacity for carbon monoxide, diffuse alveolar damage, and smoking were associated with a low MC density. MC densities of fibrotic areas had declined significantly in five subjects in whom both SLB in the stable phase and autopsy after an acute exacerbation of IPF had been performed. There were no correlations of MC densities with survival time or future acute exacerbations. The MC density in fibrotic areas was associated with several clinical parameters. An acute exacerbation of IPF was associated with a significant decline in MC counts. Further investigations will be needed to clarify the role of these cells in IPF and in the pathogenesis of acute exacerbation as this may help to identify some potential targets for medical treatment for this serious disease.
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Fibrosis Pulmonar Idiopática , Recuento de Células , Femenino , Fibrosis , Humanos , Fibrosis Pulmonar Idiopática/patología , Pulmón/patología , Mastocitos/patologíaRESUMEN
BACKGROUND: The use of a transbronchial lung cryobiopsy (TBLC) is increasing as a diagnostic method of interstitial lung diseases (ILD). This study aimed to evaluate risk factors associated with clinically significant complications of TBLC in ILD patients. METHODS: Patients referred to Kuopio or Tampere university hospitals, in Finland, for a suspected ILD were included. The TBLC was performed in an outpatient setting for 100 patients. Patients were mechanically ventilated in general anesthesia. Fluoroscopy guidance and prophylactic bronchial balloon were used. Complications, such as bleeding, pneumothorax, infections, and mortality were recorded. Moderate or serious bleeding, pneumothorax, or death ≤90 days were defined as clinically significant complications. A multivariable model was created to assess clinically significant complications. RESULTS: The extent of traction bronchiectasis (Odds ratio [OR] 1.30, Confidence interval [CI] 1.03-1.65, p = 0.027) and young age (OR 7.96, CI 2.32-27.3, p = 0.001) were associated with the risk of clinically significant complications whereas the use of oral corticosteroids ≤30 days before the TBLC (OR 3.65, CI 0.911-14.6, p = 0.068) did not quite reach statistical significance. A history of serious cough was associated with the risk of pneumothorax (OR 4.18, CI 1.10-16.0, p = 0.036). Procedure associated mortality ≤90 days was 1%. CONCLUSION: The extent of traction bronchiectasis on HRCT and young age were associated with the risk of clinically significant complications whereas oral corticosteroid use did not quite reach statistical significance. A history of serious cough was associated with the risk of clinically significant pneumothorax.
Asunto(s)
Bronquiectasia , Enfermedades Pulmonares Intersticiales , Neumotórax , Biopsia/métodos , Bronquiectasia/etiología , Broncoscopía/efectos adversos , Broncoscopía/métodos , Tos/etiología , Humanos , Pulmón/patología , Enfermedades Pulmonares Intersticiales/diagnóstico , Neumotórax/epidemiología , Neumotórax/etiología , Neumotórax/patología , Estudios Prospectivos , Factores de RiesgoRESUMEN
BACKGROUND: Accumulation of mucus into the lungs in bronchiectasis lowers the patients' quality of life. Hypertonic aerosols stimulate the removal of mucus. MATERIAL AND METHODS: 37 bronchiectasis patients without cystic fibrosis inhaled 4 ml of a 6 per cent saline solution twice a day for three months. Amount of coughs, PEF values and oxygen saturation associated with the first inhalation were measured. Quality of life was assessed by the SGRQ (St George's Respiratory Questionnaire). RESULTS: Inhalation did not decrease oxygen saturation but did lower slightly the PEF value. SGRQ score decreased by 9.2 points on the average (p = 0.002) indicating clinically significant improvement in quality of life. CONCLUSIONS: Hypertonic saline inhalations in patients with brochiectasis are safe and may also improve quality of life.