RESUMO
Allergic bronchopulmonary aspergillosis (ABPA) is an immunological pulmonary disorder caused by hypersensitivity to Aspergillus which colonizes the airways of patients with asthma and cystic fibrosis. Its diagnosis could be difficult in some cases due to atypical presentations especially when there is no medical history of asthma. Treatment of ABPA is frequently associated to side effects but cumulated drug toxicity due to different molecules is rarely reported. An accurate choice among the different available molecules and effective on ABPA is crucial. We report a case of ABPA in a woman without a known history of asthma. She presented an acute bronchitis with wheezing dyspnea leading to an acute respiratory failure. She was hospitalized in the intensive care unit. The bronchoscopy revealed a complete obstruction of the left primary bronchus by a sticky greenish material. The culture of this material isolated Aspergillus fumigatus and that of bronchial aspiration fluid isolated Pseudomonas aeruginosa. The diagnosis of ABPA was based on elevated eosinophil count, the presence of specific IgE and IgG against Aspergillus fumigatus and left segmental collapse on chest computed tomography. The patient received an inhaled treatment for her asthma and a high dose of oral corticosteroids for ABPA. Her symptoms improved but during the decrease of corticosteroids, the patient presented a relapse. She received itraconazole in addition to corticosteroids. Four months later, she presented a drug-induced hepatitis due to itraconazole which was immediately stopped. During the monitoring of her asthma which was partially controlled, the patient presented an aseptic osteonecrosis of both femoral heads that required surgery. Nine months after itraconazole discontinuation, she presented a second relapse of her ABPA. She received voriconazole for nine months associated with a low dose of systemic corticosteroid therapy with an improvement of her symptoms. After discontinuation of antifungal treatment, there was no relapse for one year follow-up.
Assuntos
Aspergilose Broncopulmonar Alérgica/diagnóstico , Aspergillus fumigatus/isolamento & purificação , Asma/diagnóstico , Pulmão/microbiologia , Corticosteroides/uso terapêutico , Idoso , Antiasmáticos/uso terapêutico , Antifúngicos/uso terapêutico , Aspergilose Broncopulmonar Alérgica/tratamento farmacológico , Aspergilose Broncopulmonar Alérgica/microbiologia , Aspergilose Broncopulmonar Alérgica/fisiopatologia , Asma/tratamento farmacológico , Asma/fisiopatologia , Feminino , Humanos , Pulmão/efeitos dos fármacos , Pulmão/fisiopatologia , Reinfecção , Resultado do TratamentoRESUMO
OBJECTIVE: To present an interesting case of left opaque hemithorax in an adult female and discuss its assessment and management. METHODS: Design: Case Report. SETTING: Tertiary care hospital. PATIENT: One. RESULTS: 44yrs retropositive female admitted with complaints of acute onset dry cough since 15-20 days, sudden breathlesness since 5 days which was progressive in nature, left sided heaviness in chest since 5 days. CECT Thorax showed complete collapse of left lung with cut off of left main bronchus while video bronchoscopy showed left main bronchus completely blocked with very thick necrotic mass and was difficult to dislodge. Debulking with cryo probe was done and left main bronchus was completely cleared off. Allergen panel showed very high serum IgE, high S.IgE against aspergillus and high specific S.IgG against aspergillus. Patient and her Chest X-ray showed significant improvement post cryo debulking and was discharged satisfactorily on oral voriconazole therapy. CONCLUSION: Endobronchial aspergillosis is characterized by massive intrabronchial overgrowth of the aspergillus species, mainly aspergillus fumigatus. Most patients with chronic pulmonary aspergillosis, including those with simple aspergillomas and Aspergillus nodules, have positive Aspergillus IgG antibodies in the blood. We hereby present a case of 44 yrs female presenting with complaints of dry cough and dyspnea and was diagnosed with endobronchial aspergillosis with complete obliteration of left main bronchus by fungal debris in which cryo debulking was done which relieved the symptoms significantly and was discharged in satisfactory condition on oral voriconazole therapy.
Assuntos
Aspergilose Broncopulmonar Alérgica , Aspergillus fumigatus/isolamento & purificação , Broncoscopia/métodos , Criocirurgia/métodos , Procedimentos Cirúrgicos de Citorredução/métodos , Voriconazol/administração & dosagem , Adulto , Antifúngicos/administração & dosagem , Aspergilose Broncopulmonar Alérgica/diagnóstico , Aspergilose Broncopulmonar Alérgica/tratamento farmacológico , Aspergilose Broncopulmonar Alérgica/fisiopatologia , Aspergilose Broncopulmonar Alérgica/cirurgia , Aspergillus fumigatus/imunologia , Técnicas Bacteriológicas/métodos , Feminino , Humanos , Imunoglobulina E/sangue , Radiografia Torácica/métodos , Tomografia Computadorizada por Raios X/métodos , Resultado do TratamentoRESUMO
Allergic bronchopulmonary aspergillosis (ABPA) is a condition characterized by an exaggerated response of the immune system to the fungus Aspergillus. This study aimed to assess the relationship between carcinoembryonic antigen (CEA) and eosinophils in ABPA patients. We describes a case of a 50-year-old patient who was diagnosed with ABPA presenting with high level of CEA and eosinophils. Besides,we used immunohistochemistry and immunofluorescence to identify eosinophils and CEA in sections which were obtained by Endobronchial ultrasound-guided transbronchial lung biopsy aspiration (EBUS-TBLB). The sections were then visualized using confocal microscopy. We also retrospectively analyzed a cohort of 37 ABPA patients between January 2013 and December 2019 in our hospital. We found the patient whose serum CEA levels were consistent with eosinophils during the follow-up (r = 0.929, P = 0.022). The positive expression of CEA and abnormal expression of eosinophils was higher in the ABPA tissue compared to the normal lung tissue. The co-localization was represented as pixels containing both red and green color in the image (with various shades of orange and yellow) which signified that eosinophils were immunohistochemically positive for CEA. Patients with higher levels of eosinophils had higher levels of CEA in the serum (P < 0.001). The results of Pearson correlation analysis showed that the levels of eosinophils were positively correlated with serum CEA levels (r = 0.459 and r = 0.506, P = 0.004 and P = 0.001). Serum CEA level is elevated in ABPA patients. The elevated serum CEA level was shown to be normalized after treatment. Increased CEA levels in ABPA patients may be positively correlated with eosinophil levels, and eosinophils may be served as CEA-secreting cells in patients with ABPA.
Assuntos
Aspergilose Broncopulmonar Alérgica/imunologia , Antígeno Carcinoembrionário/imunologia , Eosinófilos/metabolismo , Adulto , Idoso , Aspergilose Broncopulmonar Alérgica/fisiopatologia , Aspergillus/imunologia , Aspergillus fumigatus/imunologia , Asma/patologia , China , Estudos de Coortes , Eosinófilos/imunologia , Feminino , Humanos , Contagem de Leucócitos , Pulmão/patologia , Masculino , Pessoa de Meia-Idade , Estudos RetrospectivosRESUMO
Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity reaction (HR) mediated by antigens to Aspergillus fumigatus. It is estimated that 2-15% of patients with cystic fibrosis (CF) and between 1% and 5% of asthmatics develop ABPA, affecting approximately 4.8 million people worldwide. The goals of treatment are controlling inflammation, reducing the number of exacerbations and limiting the progression of lung damage. Systemic steroids are therefore used as the mainstay therapy, along with antifungal medications. However, many patients do not respond or develop side effects to treatment. In this scenario, biological drugs such as Omalizumab, Mepolizumab, Benralizumab and Dupilumab have been implemented in clinical practice, even though there is a lack of scientific evidence to support their use. We performed a literature review of the studies carried out which analyzed biologics for the management of ABPA in adult populations with asthma and CF. To our knowledge this is the first literature review that included all biologics. We included a total of 32 studies, all but one were descriptive studies, and the vast majority evaluated the use of Omalizumab. Biologics appeared to have more benefit for patients with ABPA and asthma than CF, specifically at decreasing the frequency of acute exacerbations and by having a steroid-sparing effect. Although a decrease in serum IgE level is considered a measure of therapy success, values may not decline as expected in the context of a significant clinical improvement, highlighting the importance of measuring patient-oriented outcomes. As evidence comes mainly from case series and case reports, randomized controlled trials are needed to evaluate further the safety and efficacy of biologics in ABPA.The reviews of this paper are available via the supplemental material section.
Assuntos
Anticorpos Monoclonais/uso terapêutico , Aspergilose Broncopulmonar Alérgica/tratamento farmacológico , Asma/tratamento farmacológico , Produtos Biológicos/uso terapêutico , Fibrose Cística/tratamento farmacológico , Pulmão/efeitos dos fármacos , Adulto , Idoso , Anticorpos Monoclonais/efeitos adversos , Antifúngicos/uso terapêutico , Aspergilose Broncopulmonar Alérgica/diagnóstico , Aspergilose Broncopulmonar Alérgica/microbiologia , Aspergilose Broncopulmonar Alérgica/fisiopatologia , Asma/diagnóstico , Asma/fisiopatologia , Produtos Biológicos/efeitos adversos , Fibrose Cística/diagnóstico , Fibrose Cística/fisiopatologia , Progressão da Doença , Feminino , Humanos , Pulmão/microbiologia , Pulmão/fisiopatologia , Masculino , Pessoa de Meia-Idade , Medição de Risco , Fatores de Risco , Esteroides/uso terapêutico , Resultado do Tratamento , Adulto JovemRESUMO
BACKGROUND: Cystic Fibrosis (CF) is a hereditary pulmonary and extra-pulmonary disease that occurs equally in men and women. However, a difference in morbidity and mortality rates between the sexes has been long documented. Similarly, a sex-disparity in disease severity has been reported in asthma as well. Studies done to date point to estrogen as a possible cause of this sex disparity in pulmonary outcomes in both conditions. CASE PRESENTATION: Here, we describe a case of a patient with CF and asthma/allergic bronchopulmonary aspergillosis (ABPA) undergoing sex reassignment therapy (male-to-female) and the negative impact it had on her lung function and frequency of pulmonary exacerbations in the context of increasing doses of exogenous estrogen. CONCLUSIONS: This case raises the possibility of a link between estrogen and worsening pulmonary outcomes and the need for further studies into transgender individuals with CF and/or asthma/ABPA as well as those undergoing high dose estrogen therapy for other indications.
Assuntos
Aspergilose Broncopulmonar Alérgica/complicações , Asma/complicações , Fibrose Cística/complicações , Estrogênios/efeitos adversos , Pulmão/fisiopatologia , Aspergilose Broncopulmonar Alérgica/fisiopatologia , Asma/fisiopatologia , Fibrose Cística/fisiopatologia , Estrogênios/administração & dosagem , Feminino , Humanos , Masculino , Testes de Função Respiratória , Cirurgia de Readequação Sexual , Pessoas Transgênero , Adulto JovemRESUMO
BACKGROUND: Allergic bronchopulmonary aspergillosis (ABPA) is an allergic pulmonary disease caused by a hypersensitivity reaction to Aspergillus colony in the airways. Due to the diversity of disease manifestations and physicians' incomplete knowledge of ABPA, patients with ABPA are often misdiagnosed and given poor prognosis. OBJECTIVES: To investigate the clinical characteristics, treatment and prognosis status among patients with ABPA. METHODS: We retrospectively analyzed 75 patients diagnosed as ABPA in Zhongshan Hospital, Fudan University between October 2008 and June 2018. RESULTS: Among 75 patients, 28% had asthma as the predisposing condition and 69% suffered from misdiagnosis before ABPA diagnosis was made, among which 21% were misdiagnosed as tuberculosis. About 34% of patients had eosinophil count below 500 cells/µL and 25% had total serum IgE level <1000 IU/mL. Nearly half of patients did not meet the diagnostic criteria of ABPA and all the patients who overlapped with invasive pulmonary aspergillosis (IPA) did not meet the diagnostic criteria of standard ABPA. Patients who did not fully meet the diagnostic criteria were more likely treated with inhaled corticosteroids (ICS) and antibiotics, but more difficult to attain response and remission and easier to develop advanced ABPA. CONCLUSIONS: This study demonstrates an unsatisfactory situation of diagnosis, treatment and prognosis of ABPA, including a high misdiagnosis rate, uncertainty in the diagnosis of ABPA, lack of standardization in treatment and relatively poor prognosis.
Assuntos
Aspergilose Broncopulmonar Alérgica/diagnóstico , Aspergilose Broncopulmonar Alérgica/microbiologia , Aspergillus/isolamento & purificação , Asma/complicações , Erros de Diagnóstico/estatística & dados numéricos , Administração por Inalação , Corticosteroides/administração & dosagem , Corticosteroides/uso terapêutico , Adulto , Idoso , Antibacterianos/uso terapêutico , Antifúngicos/uso terapêutico , Aspergilose Broncopulmonar Alérgica/tratamento farmacológico , Aspergilose Broncopulmonar Alérgica/fisiopatologia , Asma/epidemiologia , China/epidemiologia , Erros de Diagnóstico/prevenção & controle , Eosinófilos/citologia , Feminino , Humanos , Imunoglobulina E/sangue , Contagem de Leucócitos , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Tuberculose Pulmonar/diagnósticoRESUMO
Allergic bronchopulmonary aspergillosis (ABPA) is a complex immunological disorder complicating asthma. Uncommonly, ABPA presents without underlying asthma. Herein, we describe the outcomes of ABPA with and without asthma. Of the 530 subjects (median follow-up, 39 months), 37 (7%) were ABPA sans asthma. Bronchiectasis was more frequent (97.3% vs. 83.2%, P = .02), and the lung function was significantly better in ABPA sans asthma. The incidence-rate of ABPA exacerbation was higher in those with asthma than without (112 vs. 242 per 1000 person-years, P = .0001). ABPA sans asthma appears to be a distinct subset of ABPA, with a better lung function and fewer exacerbations.
Assuntos
Aspergilose Broncopulmonar Alérgica/classificação , Aspergilose Broncopulmonar Alérgica/fisiopatologia , Bronquiectasia/etiologia , Adulto , Aspergilose Broncopulmonar Alérgica/etiologia , Asma/complicações , Bases de Dados Factuais , Progressão da Doença , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Testes de Função Respiratória , Estudos Retrospectivos , Adulto JovemRESUMO
BACKGROUND: The clinical effects of Aspergillus fumigatus in the cystic fibrosis (CF) airway, with the exception of allergic bronchopulmonary aspergillosis, is unclear. METHODS: CF adolescents and adults (age 14â¯years and older) underwent bacterial and semi-selective fungal culture testing to determine the prevalence of fungi in the CF respiratory tract and the independent association between the presence of Aspergillus fumigatus and clinical characteristics. RESULTS: Aspergillus fumigatus (10.3%) and Candida species (57.8%) were the most common filamentous fungi and yeast seen respectively in the sputa of 206 individuals with CF. Inhaled corticosteroid (ICS) use was more common in Aspergillus fumigatus-positive than Aspergillus fumigatusnegative (100% versus 75.8%, pâ¯=â¯.01). Aspergillus fumigatus was significantly associated with lower respiratory domain score (ß -8.74, 95% CI -16.6, -0.88, pâ¯=â¯.03), representing worse respiratory-related quality of life, accounting for demographics, disease characteristics, and the presence of a pulmonary exacerbation. CONCLUSION: The presence of Aspergillus fumigatus in CF sputum was associated with worse respiratory quality of life in CF in a crosssectional, single center study. Longitudinal analysis examining the clinical implications of Aspergillus fumigatus on respiratory health over time is needed.
Assuntos
Aspergilose Broncopulmonar Alérgica , Aspergillus fumigatus/isolamento & purificação , Fibrose Cística , Qualidade de Vida , Escarro/microbiologia , Adolescente , Adulto , Antifúngicos/farmacologia , Aspergilose Broncopulmonar Alérgica/epidemiologia , Aspergilose Broncopulmonar Alérgica/fisiopatologia , Aspergilose Broncopulmonar Alérgica/terapia , Estudos Transversais , Fibrose Cística/epidemiologia , Fibrose Cística/fisiopatologia , Fibrose Cística/psicologia , Fibrose Cística/terapia , Feminino , Humanos , Masculino , Prevalência , Testes de Função Respiratória/métodos , Testes de Função Respiratória/estatística & dados numéricos , Estados Unidos/epidemiologiaRESUMO
BACKGROUND: Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity lung disease due to Aspergillus fumigatus (Af) which occurs in 10% of patients with cystic fibrosis (CF). ABPA is associated with increased morbidity and accelerated lung function decline; however, existing diagnostic criteria are nonspecific and diagnosis remains challenging. As ABPA is driven by Th2 inflammation, the aim of this study was to evaluate exhaled nitric oxide (FE NO ), eosinophilic cationic protein (ECP), peripheral eosinophil count, and bronchodilator response (BDR) in patients with CF. METHODS: A prospective observational cohort study of pediatric CF patients in a tertiary center. Patients had a clinical and serologic ABPA assessment, FENO , serum ECP, peripheral eosinophil count, and assessment of BDR. Patients were stratified into three groups; ABPA, Af sensitized (AFS), and non-ABPA non-Af-sensitized (non-AFS). RESULTS: A total of 62 patients were included in the study: 13% ABPA, 19% AFS, and 68% non-AFS. Mean FENO was higher in the ABPA group at 37.8 ppb compared to AFS 15.1 ppb (P = .05) and non-AFS 13.7 ppb (P = .04). Mean peripheral eosinophil count in ABPA group was also higher at 1000 cells/uL, compared to AFS 221 cells/uL (P = .03) and non-AFS 220 cells/uL (P = .03). Mean BDR in ABPA group was 13% compared to 5.5% in non-AFS (P = .01). Serum ECP was higher in patients with ABPA positive compared to the other groups, although this was not statistically significant. CONCLUSION: In children with cystic fibrosis, FENO and peripheral eosinophil counts are elevated in ABPA compared to those that are just sensitized to Aspergillus and may serve as useful diagnostic tests.
Assuntos
Aspergilose Broncopulmonar Alérgica/diagnóstico , Fibrose Cística , Adolescente , Aspergilose Broncopulmonar Alérgica/imunologia , Aspergilose Broncopulmonar Alérgica/metabolismo , Aspergilose Broncopulmonar Alérgica/fisiopatologia , Aspergillus fumigatus , Biomarcadores/metabolismo , Criança , Estudos de Coortes , Fibrose Cística/imunologia , Fibrose Cística/metabolismo , Fibrose Cística/fisiopatologia , Eosinófilos/imunologia , Feminino , Volume Expiratório Forçado , Humanos , Contagem de Leucócitos , Masculino , Óxido Nítrico/metabolismoAssuntos
Antialérgicos/uso terapêutico , Aspergilose Broncopulmonar Alérgica/tratamento farmacológico , Fibrose Cística/fisiopatologia , Omalizumab/uso terapêutico , Adolescente , Corticosteroides/uso terapêutico , Adulto , Antifúngicos/uso terapêutico , Aspergilose Broncopulmonar Alérgica/complicações , Aspergilose Broncopulmonar Alérgica/fisiopatologia , Criança , Fibrose Cística/complicações , Volume Expiratório Forçado , Humanos , Masculino , Falha de Tratamento , Resultado do TratamentoRESUMO
BACKGROUND: Coexistence of aspergilloma and allergic bronchopulmonary aspergillosis (ABPA) has rarely been reported. Although the treatment for ABPA includes administration of corticosteroids and antifungal agents, little is known about the treatment for coexisting aspergilloma and ABPA. Furthermore, the impact of surgical resection for aspergilloma on ABPA is not fully understood. Here, we present an interesting case of recurrent ABPA with long-term follow-up after surgical resection of aspergilloma. CASE PRESENTATION: A 53-year-old man with a medical history of tuberculosis was referred to our hospital with cough and dyspnea. Imaging revealed multiple cavitary lesions in the right upper lobe of the lung, with a fungus ball and mucoid impaction. The eosinophil count, total serum immunoglobulin E (IgE), and Aspergillus-specific IgE levels were elevated. Specimens collected on bronchoscopy revealed fungal filaments compatible with Aspergillus species. Based on these findings, a diagnosis of ABPA with concomitant aspergilloma was made. Although treatment with corticosteroids and antifungal agents was administered, the patient's respiratory symptoms persisted. Therefore, he underwent lobectomy of the right upper lobe, which resulted in a stable condition without the need for medication. Twenty-three months after discontinuation of medical treatment, his respiratory symptoms gradually worsened with a recurrence of elevated eosinophil count and total serum IgE. Imaging revealed recurrent bronchiectasis and cavities with mucoid impaction in the right lower lobe, suggesting relapse of aspergilloma and ABPA. Corticosteroids and antifungal agents were re-administered; aspergilloma improved slightly over a 5-year period, and ABPA remained well controlled with low-dose prednisolone (5 mg/day). CONCLUSIONS: We describe the long-term follow-up outcomes of a patient with concomitant ABPA and aspergilloma, who underwent surgical resection for aspergilloma. Physicians should carefully monitor patients with coexisting ABPA and aspergilloma, as the condition may relapse after remission, even despite surgical resection for aspergilloma. Additionally, surgical resection for aspergilloma could result in resolution of ABPA.
Assuntos
Antifúngicos/administração & dosagem , Aspergilose Broncopulmonar Alérgica , Aspergillus , Eosinófilos , Glucocorticoides/administração & dosagem , Imunoglobulina E/sangue , Pulmão , Pneumonectomia , Complicações Pós-Operatórias , Aspergilose Broncopulmonar Alérgica/diagnóstico , Aspergilose Broncopulmonar Alérgica/etiologia , Aspergilose Broncopulmonar Alérgica/fisiopatologia , Aspergillus/efeitos dos fármacos , Aspergillus/isolamento & purificação , Broncoscopia/métodos , Humanos , Contagem de Leucócitos/métodos , Pulmão/diagnóstico por imagem , Pulmão/microbiologia , Masculino , Pessoa de Meia-Idade , Pneumonectomia/efeitos adversos , Pneumonectomia/métodos , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/fisiopatologia , Aspergilose Pulmonar/diagnóstico , Aspergilose Pulmonar/fisiopatologia , Aspergilose Pulmonar/cirurgia , Radiografia Torácica/métodos , Recidiva , Resultado do TratamentoRESUMO
Objective To summarize the clinical features of eosinophilic lung diseases(ELD). Methods We retrospectively analyzed the clinical manifestations,laboratory findings,accessory examination results,and pathology of 40 patients who were diagnosed with ELD and hospitalized in Peking Union Medical College Hospital from January 2013 to December 2016.Results There were 19 males and 21 females,and the average age was(48.58±18.25) years.The diagnoses included allergic bronchopulmonary aspergillosis(n=20),eosinophilic granulomatosis with polyangiitis(also known as churg-strauss syndrome)(n=10),chronic eosinophilic pneumonia(n=8),parasitic infection(n=1),and drug-induced eosinophilic pneumonia(n=1).Eosinophils counts in peripheral blood were increased in 35 patients(87.5%),and eosinophils counts in bronchoalveolar lavage fluids increased in 17 of 18 patients(94.4%).Arterial blood gas analysis showed varying degrees of hypoxemia in 23 patients(57.5%),and pulmonary function test showed ventilatory dysfunction in 27 patients(67.5%) and defect in diffusion capacity in 12 patients(30.0%).Chest CT revealed bilateral flaky,streaky,or diffuse ground-glass infiltrates and consolidations;in addition,central cylindrical bronchiectasis and mucous plugging with "finger-in-glove" pattern were seen in patients with allergic bronchopulmonary aspergillosis.Diffuse eosinophil infiltration was revealed in lung or other tissue biopsy.Glucocorticoids alone or combined with other therapies were effective in most patients.Conclusions ELD has a wide range of clinical presentations and can easily be misdiagnosed.Increased eosinophils count in peripheral blood and bronchoalveolar lavage fluids combined with infiltration manifestations in chest imaging are helpful for the diagnosis of ELD.Oral administration of glucocorticoids is the primary therapy for ELD.
Assuntos
Pneumopatias/diagnóstico , Pneumopatias/fisiopatologia , Pulmão/patologia , Adulto , Idoso , Aspergilose Broncopulmonar Alérgica/diagnóstico , Aspergilose Broncopulmonar Alérgica/fisiopatologia , Líquido da Lavagem Broncoalveolar/citologia , Eosinófilos/citologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Eosinofilia Pulmonar/diagnóstico , Eosinofilia Pulmonar/fisiopatologia , Estudos RetrospectivosRESUMO
BACKGROUND: Allergic bronchopulmonary aspergillosis (ABPA) is an allergic pulmonary disease comprising a complex hypersensitivity reaction to Aspergillus fumigatus. Clinical features of ABPA are wheezing, mucoid impaction, and pulmonary infiltrates. Oral corticosteroids and anti-fungal agents are standard therapy for ABPA, but long-term use of systemic corticosteroids often causes serious side effects. CASE PRESENTATION: A 64-year-old woman was diagnosed with ABPA based on a history of bronchial asthma (from 40 years of age), elevated total IgE, the presence of serum precipitating antibodies and elevated specific IgE antibody to A. fumigatus, and pulmonary infiltration. Bronchoscopy showed eosinophilic mucoid impaction. Systemic corticosteroid therapy was initiated, and her symptoms disappeared. Peripheral eosinophilia and pulmonary infiltration recurred five months after cessation of corticosteroid treatment. Systemic corticosteroids were re-initiated and itraconazole was added as an anti-fungal agent. The patient was free of corticosteroids, aside from treatment with a short course of systemic corticosteroids for asthma exacerbation, and clinically stable with itraconazole and asthma treatments for 3 years. In 2017, she experienced significant deterioration. Laboratory examination revealed marked eosinophilia (3017/µL) and a chest computed tomography (CT) scan demonstrated pulmonary infiltration in the left upper lobe and mucoid impaction in both lower lobes. The patient was treated with high-dose inhaled corticosteroid/long-acting beta-agonist, a long-acting muscarinic antagonist, a leukotriene receptor antagonist, and theophylline; spirometry revealed a forced expiratory volume in 1 s (FEV1) of 1.01 L. An uncontrolled asthma state was indicated by an Asthma Control Test (ACT) score of 18. Mepolizumab, 100 mg every 4 weeks, was initiated for the treatment of severe bronchial asthma with ABPA exacerbation. Bronchial asthma symptoms dramatically improved, and ACT score increased to 24, by 4 weeks after mepolizumab treatment. Peripheral eosinophil count decreased to 174/µL. Spirometry revealed improvement of lung function (FEV1: 1.28 L). A chest CT scan demonstrated the disappearance of pulmonary infiltration and mucoid impaction. CONCLUSIONS: To our knowledge, this is the first case of ABPA to be treated with mepolizumab. Dramatic improvements were observed in symptoms, lung function, peripheral eosinophil counts, and chest images. Mepolizumab could serve as an alternative treatment with the potential to provide a systemic corticosteroid-sparing effect.
Assuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Aspergilose Broncopulmonar Alérgica/tratamento farmacológico , Asma/tratamento farmacológico , Corticosteroides/uso terapêutico , Agonistas Adrenérgicos beta/uso terapêutico , Antiasmáticos/uso terapêutico , Antifúngicos/uso terapêutico , Aspergilose Broncopulmonar Alérgica/complicações , Aspergilose Broncopulmonar Alérgica/fisiopatologia , Asma/complicações , Asma/fisiopatologia , Progressão da Doença , Feminino , Volume Expiratório Forçado , Humanos , Itraconazol/uso terapêutico , Antagonistas de Leucotrienos/uso terapêutico , Pessoa de Meia-Idade , Antagonistas Muscarínicos/uso terapêutico , Teofilina/uso terapêuticoRESUMO
Allergic bronchopulmonary mycosis (ABPM) is a pulmonary hypersensitivity disease mainly caused by Aspergillus fumigatus. The mainstay treatment for ABPM is systemic corticosteroid therapy. A 25-year-old man presented with pulmonary infiltrates. His peripheral eosinophil, total serum IgE, and serum Aspergillus-specific IgE levels were elevated. The patient tested positive in a skin test for Aspergillus. However, sputum cultures revealed a Curvularia lunata infection. We therefore diagnosed ABPM possibly caused by C. lunata, which is rare in Japan. The clinical state of the patient improved under observation. Identification of the causative fungus is an important aspect of the ABPM diagnosis.
Assuntos
Aspergilose Broncopulmonar Alérgica/diagnóstico , Aspergilose Broncopulmonar Alérgica/fisiopatologia , Aspergillus fumigatus/isolamento & purificação , Saccharomyces/isolamento & purificação , Corticosteroides/uso terapêutico , Adulto , Aspergilose Broncopulmonar Alérgica/tratamento farmacológico , Eosinófilos , Humanos , Hipersensibilidade , Japão , Masculino , Testes CutâneosRESUMO
BACKGROUND: Allergic bronchopulmonary aspergillosis (ABPA) is a severe lung disease complication caused by an Aspergillus fumigatus-induced hypersensitivity that affects 2-15% of patients with cystic fibrosis (CF). The mainstay treatment consists of a combination of corticosteroids and antifungals. However, repeated or long-term corticosteroid therapies can lead to serious side effects. The monoclonal anti-IgE antibody, omalizumab, has demonstrated its efficacy in allergic asthma. As ABPA results from a hypersensitivity to a specific allergen, omalizumab might benefit CF patients with ABPA. Therefore, we conducted a retrospective study to investigate the effects of omalizumab on ABPA in CF patients. METHODS: We retrospectively analyzed the clinical records of young patients with CF treated with omalizumab for an ABPA in several French CF centers. The clinical data were collected 3 months before the start of omalizumab treatment, at initiation, and every 3 months up to 12 following initiation. These data comprised clinical, biological, nutritional, and functional parameters. RESULTS: Eighteen patients were included (mean age: 17.1 ± 5.2 yrs). Under omalizumab was observed a stabilization of the lung function decline associated with a significant decrease in the corticosteroid daily dose (p = 0.0007) and an improvement in the nutritional status (p = 0.01). No serious side effect of omalizumab was reported. CONCLUSIONS: This study suggests that omalizumab might be an interesting therapeutic strategy in ABPA, associated with less side effects compared to long-term corticosteroids. Further randomized-controlled trials are needed to ascertain the efficacy of omalizumab in CF patients with ABPA.
Assuntos
Aspergilose Broncopulmonar Alérgica/tratamento farmacológico , Fibrose Cística/tratamento farmacológico , Omalizumab/uso terapêutico , Adolescente , Corticosteroides/uso terapêutico , Antialérgicos/uso terapêutico , Anticorpos Anti-Idiotípicos/uso terapêutico , Antifúngicos/uso terapêutico , Aspergilose Broncopulmonar Alérgica/complicações , Aspergilose Broncopulmonar Alérgica/fisiopatologia , Aspergillus fumigatus/efeitos dos fármacos , Criança , Fibrose Cística/complicações , Fibrose Cística/imunologia , Feminino , Humanos , Masculino , Omalizumab/administração & dosagem , Testes de Função Respiratória/métodos , Estudos Retrospectivos , Adulto JovemRESUMO
OBJECTIVE: To evaluate the effect of allergic bronchopulmonary aspergillosis (ABPA) on FEV1 percent predicted in children and adolescents with cystic fibrosis. DESIGN: Longitudinal data analysis (2008-2010). SETTING: Patients participating in the European Cystic Fibrosis Society Patient Registry. PARTICIPANTS: 3350 patients aged 6-17 years. MAIN OUTCOME MEASURE: FEV1 percent predicted was the main outcome measure (one measurement per year per child). To describe the effect of ABPA (main explanatory variable) on FEV1 while controlling for other prognostic factors, a linear mixed effects regression model was applied. RESULTS: In 2008, the mean (±SD) FEV1 percent predicted was 78.6 (±20.6) in patients with ABPA (n=346) and 88 (±19.8) in those without ABPA (n=2806). After considering other variables, FEV1 in subjects with ABPA on entry to the study was 1.47 percentage points lower than FEV1 in patients of similar age without ABPA (p=0.003). There was no FEV1 decline associated with ABPA over the subsequent study years as the interaction of ABPA with age was not significant (p>0.05). For patients aged 11.82 years (population mean age), poor body mass index had the greatest impact on FEV1 in 2008, followed by high-risk genotype (two severe mutations), female gender, diabetes mellitus, chronic Pseudomonas aeruginosa infection and ABPA in descending order of effect size. CONCLUSIONS: In contrast to the common clinical belief of ABPA having a serious impact on lung function, the difference in FEV1 between young patients with and without the complication was found to be modest when the effect of other prognostic factors was considered.
Assuntos
Aspergilose Broncopulmonar Alérgica/fisiopatologia , Fibrose Cística/fisiopatologia , Adolescente , Distribuição por Idade , Aspergilose Broncopulmonar Alérgica/complicações , Índice de Massa Corporal , Criança , Fibrose Cística/complicações , Feminino , Volume Expiratório Forçado/fisiologia , Humanos , Estudos Longitudinais , Masculino , Sistema de Registros , Distribuição por SexoRESUMO
BACKGROUND: Allergic bronchopulmonary aspergillosis (ABPA) is an immunological lung disorder occurring due to hypersensitivity to fungus Aspergillus fumigatus. Early diagnosis of ABPA is desirable as the disease can cause irreversible damage and end stage lung disease. Diagnosis of ABPA is based upon a set of clinical, serological and radiological criteria. Radiology plays an important role in the diagnosis of ABPA. However a radiologist should also be familiar with various clinical and serological criteria, so as to have a complete understanding of the disease process and to give a confident diagnosis. OBJECTIVE: To describe various clinical, laboratory and radiological findings in ABPA with a comprehensive review of various diagnostic criteria. METHOD: We searched the literature for various criteria that have been used for diagnosis of ABPA. We also extensively reviewed the radiology literature to look for various imaging findings described in ABPA. We searched the role of various imaging modalities used in evaluation of ABPA starting with conventional radiography to computed tomography to magnetic resonance imaging. RESULTS: In this article, we have reviewed clinical, serological and radiological aspects of ABPA, with emphasis on the radiological findings. We have also described the various diagnostic criteria of ABPA. CONCLUSION: In recent years, role of radiology in ABPA has evolved. Radiology plays an important role not only in diagnosis but also classification and prognostication of the disease. A radiologist should also be familiar with the clinical spectrum and laboratory findings of the disease.
Assuntos
Aspergilose Broncopulmonar Alérgica/diagnóstico por imagem , Aspergillus fumigatus/isolamento & purificação , Fibrose Cística/complicações , Imunoglobulina E/sangue , Radiografia , Aspergilose Broncopulmonar Alérgica/imunologia , Aspergilose Broncopulmonar Alérgica/fisiopatologia , Biomarcadores/sangue , Quimiocina CCL17/sangue , Fibrose Cística/sangue , Fibrose Cística/fisiopatologia , Diagnóstico Diferencial , Diagnóstico Precoce , Humanos , Imunoglobulina E/imunologia , Guias de Prática Clínica como AssuntoRESUMO
Whether use of high-dose steroids in acute-stage allergic bronchopulmonary aspergillosis (ABPA) is associated with superior outcomes is not known. Herein, we compare the efficacy and safety of two glucocorticoid protocols in ABPA.Treatment-naive ABPA subjects randomly received either high-dose or medium-dose oral prednisolone. The primary outcomes were exacerbation rates and glucocorticoid-dependent ABPA after 1 and 2â years, respectively, of treatment. The secondary end-points were composite response rates after 6â weeks, improvement in lung function, time to first exacerbation, cumulative dose and adverse effects.92 subjects (high-dose n=44, medium-dose n=48) were included in the study. The numbers of subjects with exacerbation after 1â year (high-dose 40.9% versus medium-dose 50%, p=0.59) and glucocorticoid-dependent ABPA after 2â years (high-dose 11.4% versus medium-dose 14.6%, p=0.88) were similar in the two groups. Although composite response rates were significantly higher in the high-dose group, improvement in lung function and time to first exacerbation were similar in the two groups. Cumulative glucocorticoid dose and side-effects were significantly higher in the high-dose group.Medium-dose oral glucocorticoids are as effective and safer than high-dose in treatment of ABPA.
Assuntos
Aspergilose Broncopulmonar Alérgica/tratamento farmacológico , Asma/tratamento farmacológico , Bronquiectasia/tratamento farmacológico , Glucocorticoides/administração & dosagem , Prednisolona/administração & dosagem , Doença Aguda , Adulto , Aspergilose Broncopulmonar Alérgica/complicações , Aspergilose Broncopulmonar Alérgica/diagnóstico por imagem , Aspergilose Broncopulmonar Alérgica/fisiopatologia , Asma/complicações , Asma/diagnóstico por imagem , Asma/fisiopatologia , Bronquiectasia/complicações , Bronquiectasia/diagnóstico por imagem , Bronquiectasia/fisiopatologia , Progressão da Doença , Feminino , Volume Expiratório Forçado , Humanos , Masculino , Índice de Gravidade de Doença , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Capacidade Vital , Adulto JovemRESUMO
The role of total and specific IgE in monitoring treatment responses in allergic bronchopulmonary aspergillosis (ABPA) remains poorly studied. Here in, we evaluate the utility of total and Aspergillus fumigatus specific IgE in the follow-up of ABPA. Eighty-one consecutive treatment-naïve patients of ABPA (acute stage) with pulmonary infiltrates and bronchiectasis underwent measurement of total and A. fumigatus specific IgE at baseline, after 8 weeks of glucocorticoid therapy, and during exacerbations. There was clinical and radiological improvement after treatment with median decline of total IgE by 51.9%. The total IgE declined by at least 35%, 25% and 20% in 69 (85.2%), 76 (93.6%) and 78 (96.3%) patients, respectively. On the other hand, the A. fumigatus specific IgE increased in 42 (51.9%) subjects, and the mean increase was 1.4%, after 8 weeks. Among 13 patients with exacerbation, 12 (92.3%) had a rise of total IgE by >50%. The A. fumigatus specific IgE increased in only five (38.5%) subjects during exacerbation. Thus, the total IgE is a useful test in monitoring treatment responses in ABPA while A. fumigatus specific IgE has limited utility.