RESUMO
We present a case of cardiac sarcoidosis of insidious onset mimicking arrhythmogenic right ventricular cardiomyopathy. Our patient initially presented with systemic sarcoidosis but later developed palpitations. The similarity in clinical presentation and cardiac magnetic resonance findings in both conditions posed a challenge in differentiating between the two in the absence of histological diagnosis. We highlighted the role of positron emission tomography in aiding a diagnosis.
Assuntos
Displasia Arritmogênica Ventricular Direita/diagnóstico por imagem , Cardiomiopatias/diagnóstico por imagem , Tomografia por Emissão de Pósitrons/métodos , Sarcoidose/diagnóstico por imagem , Diagnóstico Diferencial , Fluordesoxiglucose F18 , Humanos , Imageamento por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-IdadeRESUMO
We describe the case of a 70-year-old never smoker with chronic lymphocytic leukaemia, treated with single agent ibrutinib therapy. Chest imaging noted nodular change and mediastinal lymphadenopathy, which showed avid uptake on positron emission tomography and guided subsequent biopsies (bronchoscopy using endobronchial ultrasound, mediastinoscopy). Despite negative aspergillus blood immunology tests, he was found to have invasive aspergillosis, which is a known risk with ibrutinib therapy. He has since been successfully treated with antifungal therapy.
Assuntos
Neoplasias Pulmonares , Adenina/análogos & derivados , Idoso , Antifúngicos , Broncoscopia/métodos , Humanos , Neoplasias Pulmonares/patologia , Macrófagos/patologia , Masculino , Mediastinoscopia/métodos , Mediastino/patologia , Estadiamento de Neoplasias , PiperidinasRESUMO
BACKGROUND AND OBJECTIVE: The aim of the present study was to report the features of five patients with concurrent histopathological features of pulmonary alveolar proteinosis (PAP) and hypersensitivity pneumonitis (HP) and their high-resolution CT (HRCT) appearances. METHODS: Patients with histopathological features of both HP and PAP on surgical lung biopsy referred for tertiary review were retrospectively identified. The pathology and HRCT images were semi-quantitatively scored to evaluate the relative contribution to HP and PAP. RESULTS: Five patients had histopathological features of HP and PAP but had varied HRCT appearances. All had imaging features of PAP to a varying degree with two patients also showing characteristics of HP but three patients had ill-defined thickened interlobular septa, not typical of either disease. CONCLUSIONS: We describe the coexistence of PAP and HP in five patients and discuss possible linkages between these two distinct pathologies.
Assuntos
Alveolite Alérgica Extrínseca/patologia , Proteinose Alveolar Pulmonar/patologia , Adulto , Idoso , Alveolite Alérgica Extrínseca/diagnóstico por imagem , Feminino , Humanos , Pulmão/diagnóstico por imagem , Pulmão/patologia , Masculino , Pessoa de Meia-Idade , Proteinose Alveolar Pulmonar/diagnóstico por imagem , Estudos Retrospectivos , Tomografia Computadorizada por Raios XRESUMO
Sarcoidosis remains difficult to diagnose, assess and treat. The last decade has brought significant diagnostic and therapeutic advances in the field of sarcoidosis including endobronchial ultrasound, F-fluorodeoxyglucose positron emission tomography and biologics. In this article we use clinical vignettes to discuss commonly encountered cases to illustrate and explain the application of these, and other advances.
Assuntos
Sarcoidose Pulmonar , Sarcoidose , Fluordesoxiglucose F18 , Humanos , Tomografia por Emissão de Pósitrons , Compostos Radiofarmacêuticos , Sarcoidose/diagnóstico , Sarcoidose/terapia , Sarcoidose Pulmonar/diagnóstico por imagem , Sarcoidose Pulmonar/terapiaAssuntos
COVID-19 , Fibrose Pulmonar , COVID-19/epidemiologia , Humanos , Pandemias , Fibrose Pulmonar/terapia , SARS-CoV-2RESUMO
Idiopathic pulmonary fibrosis (IPF) is an important, and devastating, interstitial lung disease. It has a median mortality of only 3 years, worse than many cancers, and its incidence continues to rise. In this article, an overview of key developments in our understanding and clinical management of IPF will be provided.
Assuntos
Fibrose Pulmonar/terapia , Administração Oral , Corticosteroides/administração & dosagem , Anti-Inflamatórios/administração & dosagem , Terapia Combinada , Cortisona/administração & dosagem , Humanos , Prognóstico , Fibrose Pulmonar/diagnóstico , Fibrose Pulmonar/epidemiologiaRESUMO
STUDY OBJECTIVES: To evaluate the effect of adding zafirlukast or low-dose theophylline to a beclomethasone dipropionate (BDP) extra-fine hydrofluoroalkane aerosol on bronchial hyperresponsiveness as the primary outcome variable. METHODS: Twenty-four patients with mild-to-moderate asthma were studied using a randomized crossover design with the following three treatment blocks: (1) beclomethasone, 100 microg/d, alone for the first 2 weeks followed by 400 microg/d alone for the next 2 weeks; (2) beclomethasone, 100 microg/d, followed by 400 microg/d, with the addition of zafirlukast, 20 mg bid; (3) beclomethasone, 100 microg/d, followed by 400 microg/d, with the addition of theophylline, 200 to 300 mg bid. Measurements were made after 2 and 4 weeks of each treatment and at pretreatment baseline. RESULTS: The mean trough plasma theophylline concentration was 6.7 mg/L, coinciding with the anti-inflammatory target range (ie, 5 to 10 mg/L). The provocative dose of methacholine causing a 20% fall in FEV(1) (as doubling dose difference from baseline) was significantly (p < 0.05) greater with beclomethasone, 100 microg, plus zafirlukast (1.1 doubling dose) but not with beclomethasone, 100 microg, plus theophylline (0.7 doubling dose) compared to beclomethasone, 100 microg alone (0.4 doubling dose), but not compared to beclomethasone, 400 microg alone (1.1 doubling dose). There were also significant (p < 0.05) differences between beclomethasone, 100 microg, plus zafirlukast (but not BDP, 100 microg, plus theophylline) vs beclomethasone, 100 microg, alone in terms of nitric oxide level, midexpiratory phase of forced expiratory flow, and peak expiratory flow. There were no further significant improvements observed with the addition of zafirlukast or theophylline to beclomethasone, 400 microg. CONCLUSIONS: A leukotriene receptor antagonist, but not low-dose theophylline, conferred significant additive anti-inflammatory effects to therapy with a low-dose inhaled corticosteroid but not to that with a medium dose of an inhaled corticosteroid. Thus, optimizing the dose of inhaled corticosteroid as monotherapy would seem to be the logical first step, which is in keeping with current guidelines.
Assuntos
Antiasmáticos/uso terapêutico , Asma/tratamento farmacológico , Beclometasona/uso terapêutico , Hiper-Reatividade Brônquica/tratamento farmacológico , Antagonistas de Leucotrienos/uso terapêutico , Teofilina/uso terapêutico , Compostos de Tosil/uso terapêutico , Administração por Inalação , Administração Oral , Adulto , Antiasmáticos/administração & dosagem , Asma/fisiopatologia , Beclometasona/administração & dosagem , Criança , Estudos Cross-Over , Esquema de Medicação , Feminino , Humanos , Indóis , Antagonistas de Leucotrienos/administração & dosagem , Masculino , Pessoa de Meia-Idade , Fenilcarbamatos , Testes de Função Respiratória , Sulfonamidas , Teofilina/administração & dosagem , Compostos de Tosil/administração & dosagemRESUMO
BACKGROUND: The frequencies of various causes of pulmonary granulomas in pathological material are unknown, as is the influence of geographical location on aetiology. The aim of this study was to identify the causes of pulmonary granulomas in pathological specimens, to define their frequencies, and to determine whether these causes vary by geographical location. METHODS: 500 lung biopsies and resections containing granulomas were reviewed retrospectively by expert pulmonary pathologists from 10 institutions in seven countries. Fifty consecutive cases from each location were assigned a diagnosis based on histological features and available clinical/microbiological data. RESULTS: A specific cause was identified in 58% of cases (290/500), most commonly sarcoidosis (136, 27%) and mycobacterial or fungal infections (125, 25%). Mycobacteria were identified in 19% of cases outside the USA versus 8% within the USA. In contrast, fungi accounted for 19% cases in the USA versus 4% in other locations. Fungi were mostly detected by histology, whereas most mycobacteria were identified in cultures. In 42% of cases (210/500) an aetiology could not be determined. CONCLUSIONS: Across several geographical settings, sarcoidosis and infections are the most common causes of pulmonary granulomas diagnosed in pathological specimens. Fungi are more commonly identified than mycobacteria in the USA, whereas the reverse is true in other countries. A definite aetiology cannot be demonstrated in more than a third of all cases of pulmonary granulomas, even after histological examination. These findings highlight the need to submit material for histology as well as cultures in all cases in which granulomatous disease enters the differential diagnosis.
Assuntos
Granuloma/etiologia , Pneumopatias/etiologia , Características de Residência , Infecções Respiratórias/complicações , Sarcoidose Pulmonar/complicações , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Ásia/epidemiologia , Biópsia , Brasil/epidemiologia , Criança , Pré-Escolar , Europa (Continente)/epidemiologia , Feminino , Granuloma/epidemiologia , Granuloma/patologia , Humanos , Incidência , Pulmão/patologia , Pneumopatias/epidemiologia , Pneumopatias/patologia , Masculino , Pessoa de Meia-Idade , Infecções Respiratórias/epidemiologia , Infecções Respiratórias/patologia , Estudos Retrospectivos , Fatores de Risco , Sarcoidose Pulmonar/epidemiologia , Sarcoidose Pulmonar/patologia , Estados Unidos/epidemiologia , Adulto JovemAssuntos
Fibrose Pulmonar Idiopática , Guias de Prática Clínica como Assunto , Anti-Inflamatórios não Esteroides/economia , Anti-Inflamatórios não Esteroides/uso terapêutico , Ensaios Clínicos como Assunto , Diagnóstico Diferencial , Humanos , Fibrose Pulmonar Idiopática/diagnóstico , Fibrose Pulmonar Idiopática/terapia , Piridonas/economia , Piridonas/uso terapêutico , Resultado do TratamentoRESUMO
Chronic obstructive pulmonary disease (COPD) accounts for a major workload in both primary and secondary care. It is characterised by progressive airflow obstruction which does not fully reverse to inhaled or oral pharmacotherapy. The diagnosis should be considered in any current or former smoker who has symptoms of breathlessness, wheeze, cough, sputum production and impaired exercise tolerance. From a pharmacological perspective, short-acting bronchodilators (anti-cholinergics and beta(2)-agonists) play a vital role in immediate relief of symptoms. However, in patients with persistent symptoms and exacerbations, long-acting bronchodilator therapy is advocated for regular use. Tiotropium is a newly introduced long-acting anti-cholinergic which facilitates once daily administration. This evidence based review article discusses the use of long acting bronchodilators in COPD with a particular emphasis on the putative benefits of tiotropium.
Assuntos
Broncodilatadores/uso terapêutico , Doença Pulmonar Obstrutiva Crônica/tratamento farmacológico , Derivados da Escopolamina/uso terapêutico , Administração por Inalação , Corticosteroides/uso terapêutico , Agonistas de Receptores Adrenérgicos beta 2 , Agonistas Adrenérgicos beta/uso terapêutico , Antagonistas Colinérgicos/uso terapêutico , Quimioterapia Combinada , Humanos , Ensaios Clínicos Controlados Aleatórios como Assunto , Brometo de TiotrópioAssuntos
Doenças Pulmonares Intersticiais/diagnóstico , Doenças do Tecido Conjuntivo/complicações , Eletrocardiografia , Exposição Ambiental/efeitos adversos , Refluxo Gastroesofágico/complicações , Humanos , Doenças Pulmonares Intersticiais/etiologia , Anamnese , Oximetria , Linhagem , Exame Físico/métodos , Encaminhamento e Consulta , Testes de Função Respiratória , Fumar/efeitos adversos , Tomografia Computadorizada por Raios X , Urinálise/métodosRESUMO
BACKGROUND: Current guidelines advocate the use of preventative anti-inflammatory therapy for mild persistent asthma. OBJECTIVE: We compared the efficacy and anti-inflammatory profiles of a leukotriene receptor antagonist and a low dose of inhaled corticosteroid in patients with mild persistent asthma. METHODS: Twenty-one adult patients with mild asthma received 4 weeks of either once-daily inhaled hydrofluoroalkane triamcinolone acetonide (450 microg/day ex-actuator dose) or oral montelukast (10 mg/day) in a randomized, placebo-controlled, single-blinded crossover study. Measurements were made before and after 2 and 4 weeks of each treatment. RESULTS: At the endpoint (after 4 weeks), triamcinolone and montelukast had improved the primary outcome (provocative dose of methacholine required to produce a 20% fall in FEV(1)) in comparison with placebo (P <.05), there being no difference between the treatments (1.09-fold; 95% CI 0.73 to 1.63). Triamcinolone was better than placebo or montelukast for effects on all other surrogate inflammatory markers (P <.05), including exhaled nitric oxide, blood eosinophils, serum eosinophil cationic protein, plasma intracellular circulating adhesion molecule 1, and plasma E-selectin. Both treatments improved (P <.05) morning and evening peak flow, nighttime beta2-agonist use, and symptoms in comparison with placebo, though triamcinolone was better than montelukast (P <.05) with regard to peak flow. Triamcinolone produced suppression (P <.05) of overnight urinary cortisol/creatinine and serum osteocalcin. CONCLUSION: Once-daily inhaled corticosteroid and leukotriene antagonist improved the primary outcome variable of bronchial hyperresponsiveness to a similar degree.
Assuntos
Acetatos/administração & dosagem , Asma/tratamento farmacológico , Antagonistas de Leucotrienos/administração & dosagem , Quinolinas/administração & dosagem , Triancinolona/administração & dosagem , Administração por Inalação , Adolescente , Adulto , Asma/fisiopatologia , Hiper-Reatividade Brônquica/tratamento farmacológico , Estudos Cross-Over , Ciclopropanos , Esquema de Medicação , Feminino , Humanos , Antagonistas de Leucotrienos/efeitos adversos , Masculino , Pessoa de Meia-Idade , Método Simples-Cego , SulfetosRESUMO
The authors report the case of a 60-year-old man with acromegaly, who developed narcolepsy 2 weeks after completing radiotherapy for a pituitary adenoma. Cataplexy and sleepiness were predominant symptoms. Onset of narcolepsy is unusual at this age and the temporal relationship following radiotherapy suggests this treatment was implicated. His CSF hypocretin levels were normal, indicating other factors may be important in his narcolepsy.
Assuntos
Acromegalia/radioterapia , Peptídeos e Proteínas de Sinalização Intracelular , Narcolepsia/etiologia , Irradiação Hipofisária/efeitos adversos , Lesões por Radiação/etiologia , Proteínas de Transporte/líquido cefalorraquidiano , Estimulantes do Sistema Nervoso Central/uso terapêutico , Humanos , Masculino , Mazindol/uso terapêutico , Pessoa de Meia-Idade , Narcolepsia/líquido cefalorraquidiano , Narcolepsia/tratamento farmacológico , Neuropeptídeos/líquido cefalorraquidiano , Orexinas , Lesões por Radiação/líquido cefalorraquidiano , Lesões por Radiação/tratamento farmacológico , Paralisia do Sono/etiologiaAssuntos
Sarcoidose/terapia , Cardiomiopatias/diagnóstico , Cardiomiopatias/etiologia , Cardiomiopatias/terapia , Diagnóstico por Imagem , Doenças do Sistema Digestório/diagnóstico , Doenças do Sistema Digestório/etiologia , Doenças do Sistema Digestório/terapia , Oftalmopatias/diagnóstico , Oftalmopatias/etiologia , Oftalmopatias/terapia , Fadiga/etiologia , Humanos , Nefropatias/diagnóstico , Nefropatias/etiologia , Nefropatias/terapia , Doenças Linfáticas/etiologia , Anamnese , Doenças Musculoesqueléticas/diagnóstico , Doenças Musculoesqueléticas/etiologia , Doenças Musculoesqueléticas/terapia , Exame Físico , Guias de Prática Clínica como Assunto , Encaminhamento e Consulta , Sarcoidose/diagnóstico , Sarcoidose/etiologia , Sarcoidose Pulmonar/diagnóstico , Sarcoidose Pulmonar/etiologia , Sarcoidose Pulmonar/terapia , Dermatopatias/diagnóstico , Dermatopatias/etiologia , Dermatopatias/terapiaRESUMO
AIMS: Whether chronic dosing with montelukast confers benefit in patients with moderate to severe asthma remains to be fully established. A proof of concept study was performed evaluating putative benefits with montelukast in moderate persistent asthmatics who were taken off inhaled corticosteroids (ICS) and switched to salmeterol. The latter was done to dissociate the effects of montelukast from ICS. METHODS: Twenty moderate to severe persistent asthmatics completed a randomized double-blind crossover study. Subjects received montelukast 10 mg daily or placebo for 2 weeks each. This was preceded by a 2-week run-in when ICS were discontinued and salmeterol started, and used on a regular basis throughout the study. Measurements were made after run-in and after both randomized treatments. RESULTS: There were no significant sequence effects for responses as to whether placebo or montelukast were given first or second. Methacholine PD20 values after run-in, first and second placebo were 63 micro g, 60 micro g and 64 micro g, respectively (corresponding to 2, 4 and 6 weeks of ICS washout, respectively). Lung function deteriorated pre vs post run-in, which was significant (P < 0.05) for FEF25-75 % predicted. Montelukast conferred significant (P < 0.05) improvements as change from post run-in compared with placebo in methacholine PD20, FEV1 % predicted, FEF25-75 % predicted, diurnal peak expiratory flow, symptoms and salbutamol use. For the primary outcome of methacholine PD20, this amounted to a 1.6-fold difference (95% CI 1.1, 2.5). CONCLUSIONS: In moderate persistent asthmatics switched from taking ICS to salmeterol alone, adding montelukast conferred significant benefits on all parameters of asthma control. Further studies are indicated to evaluate whether montelukast exhibits additive effects to ICS/long-acting beta2-adrenoceptor agonist combination inhalers upon clinically important outcomes.