Bronchopulmonary Aspergillosis In Cystic Fibrosis Research Articles

The Clinical Association between Aspergillus fumigatus and Respiratory Outcomes in Adolescents and Adults with Cystic Fibrosis.

Rationale: The clinical significance of Aspergillus fumigatus (Af) detection in the absence of allergic bronchopulmonary aspergillosis in cystic fibrosis (CF) airways remains unclear. Yet, some clinicians initiate antifungal therapy for Af-positive respiratory cultures out of concern for infection in people with CF. Objectives: To determine the association between the presence of Af and respiratory outcomes in individuals with CF. Methods: We conducted a prospective longitudinal cohort study of 206 adults and adolescents (age 14 yr and older) with CF and collected sputum for selective fungus culture. We assessed clinical outcome measurements, including patient-reported outcomes (measured by the Cystic Fibrosis Questionnaire-Revised), spirometry, and number of pulmonary exacerbations (PEx) for a 1-year period. We used mixed-effects linear models to determine the association between positive Af culture results, defined as Af detection in sputum culture at the study visit, with both respiratory domain score and forced expiratory volume in 1 second (FEV1) percent predicted, adjusted for confounders. Mixed-effects Poisson regression models were employed to examine the association between positive Af culture results and PEx events. We explored the association between Af history, defined as Af detection at baseline or within 2 years of enrollment, and respiratory outcomes. Results: Af prevalence was 10.3% (95% confidence interval [CI], 6.8, 15.7) at baseline. Forty-eight (23.3%; 95% CI, 17.7, 29.7) participants had at least one Af-positive culture result during the study period. Positive Af culture result was not associated with lower respiratory domain score. However, Af history was associated with a 6.48-point lower respiratory domain score, reflective of worse respiratory quality of life (95% CI, -11.96, -0.99; P = 0.02). Positive Af culture result was associated with a 2.54% lower FEV1 percent predicted (95% CI, -4.64, -0.44; P = 0.02) and a 1.71-fold increase in severe PEx incidence (95% CI, 1.05, 2.76; P = 0.03). Conclusions: Positive Af culture result was not associated with lower patient-reported, respiratory-related quality of life. Yet, positive Af culture result was associated with both lower FEV1 percent predicted and increased frequency of severe PEx warranting intravenous antibiotics in adolescents and adults with CF. Future studies are required to better understand the direct role of Af in lung disease progression in CF.

Beyond the Guidelines: Treatment of Allergic Bronchopulmonary Aspergillosis in Cystic Fibrosis.

To review the available literature addressing alternative allergic bronchopulmonary aspergillosis (ABPA) treatment options for patients with cystic fibrosis (CF). A literature search of PubMed was performed (January 2002 to April 2021) using the following search terms: allergic bronchopulmonary aspergillosis, aspergillus-related lung disease, cystic fibrosis. Manufacturer prescribing information, clinical practice guidelines, and data from ClinicalTrials.gov were incorporated in the reviewed data. Relevant English-language studies or those conducted in humans were considered for inclusion. Available literature for alternative ABPA treatments in CF is lacking randomized controlled trials, but there is considerable support in case reports and case series describing the benefits in pediatric and adult patients. Recent literature has begun to explore the place in therapy for novel, corticosteroid-sparing treatment approaches. The alternative therapies summarized in this review all resulted in clinical improvement and subsequent discontinuation or dose reductions of oral corticosteroids, with minimal reported adverse drug effects. Although corticosteroids are the cornerstone of ABPA management, the toxicities can be significant limitations in an already high-risk patient population. Patients may fail or become intolerant to guideline-recommended therapies and require alternative treatment approaches. Alternative treatment modalities for ABPA in patients with CF, including azole antifungals, pulsed intravenous glucocorticoids, omalizumab, mepolizumab, and inhaled amphotericin, appear to be efficacious and well tolerated. Pharmacological properties including route of administration, storage and stability, beyond use dating, and adverse effects of the various treatment modalities must be considered when selecting a practical care plan for patients.

Biomarkers for the Diagnosis of Allergic Bronchopulmonary Aspergillosis in Cystic Fibrosis: A Systematic Review and Meta-Analysis

Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity reaction to Aspergillus fumigatus and impacts 10% of individuals with cystic fibrosis (CF). A diagnosis of ABPA is challenging to establish in CF owing to overlapping clinical and radiologic features with CF lung disease. Recent studies have identified blood tests, imaging, and other biomarkers that may be useful for diagnosis. To summarize biomarkers that can aid in the diagnosis of ABPA in CF patients and to quantify their diagnostic accuracy through meta-analysis. We searched MEDLINE, EMBASE, and the Cochrane Controlled Register of Trials and included studies that used a laboratory technique or imaging modality in CF patients diagnosed with ABPA. Pooled sensitivity and specificity were calculated using a hierarchical summary receiver operating characteristic model. We identified 791 articles, of which 29 met our eligibility criteria and 9 were included in the meta-analysis. Hyperattenuating mucus on computed tomography (CT) scan (n= 3 studies; pooled sensitivity 62% and specificity 92%) and serum specific immunoglobulin E against recombinant Aspergillus funigatus antigens f4 (n= 6; 69%, 89%) and f6 (n= 6; 39%, 97%) demonstrated high specificity. Based on single studies, serum thymus and activation regulated chemokine (92%, 94%), stimulated basophil expression of CD203c (94%, 74%), the inverted mucoid impaction signal on magnetic resonance imaging (94%, 100%), and skin prick test with recombinant Aspergillus fumigatus f4 and/or f6 (100%, 100%) showed high sensitivity and specificity. Recent studies have found promising biomarkers for diagnosing ABPA in CF. Further research is needed to improve our understanding of their utility in diagnosis and disease monitoring.

Frequent Pet Contact as Risk Factor for Allergic Bronchopulmonary Aspergillosis in Cystic Fibrosis.

Aspergillus fumigatus (Af) frequently colonizes the respiratory tract of patients with cystic fibrosis (CF). Af is associated with loss of pulmonary function and allergic bronchopulmonary aspergillosis (ABPA), a hypersensitivity fungal lung disease. Environmental factors have impact on CF patients’ lung function variation. The aim of this nationwide questionnaire survey was to investigate the amount of CF patients with frequent pet contact including pet species and to examine the potential impact of frequent pet contact on the occurrence of Af colonization and ABPA diagnosis in these patients. The survey was carried out in 31 German CF centers in 2018. A total of 1232 who completed the surveys were included, and statistical analysis was performed by chi-squared test. Within the study cohort 49.8% of subjects (n = 614; CF patients < 18years: 49.4%, n = 234; ≥ 18years: 50.1%, n = 380) reported frequent contact to pets, of which 60.7% reported frequent contact to dogs, 42.3% to cats and other animals. Of those with frequent pet contact, 71.8% (n = 441) had contact to one pet or more pets from the same family. Af colonization was not significantly associated with frequent pet contact. ABPA diagnosis was documented in 16.7% (n = 206) of all included CF patients and was significantly associated with frequent pet contact (18.9%, n = 116, p = 0.042), confirming previous single center examinations. Particularly, patients with frequent contact to dogs showed an increased ABPA prevalence of 21.3%. Frequent pet contact might be a risk factor for ABPA. CF patients who are sensitized to Af should be informed about the increased risk to develop an ABPA by frequent pet contact. Patients with recurrent onset of ABPA should be evaluated in terms of frequent pet contact.

P127 Pulsed intravenous methylprednisolone for allergic bronchopulmonary aspergillosis in cystic fibrosis

P127 Pulsed intravenous methylprednisolone for allergic bronchopulmonary aspergillosis in cystic fibrosis

Nonsystemic allergic bronchopulmonary aspergillosis in cystic fibrosis: A suggested paradigm for the evolution from topical to systemic disease.

No abstract available Keywords: ABPA; allergic bronchopulmonary aspergillosis; cystic fibrosis.

Allergic Bronchopulmonary Aspergillosis in Cystic Fibrosis: MR Imaging of Airway Mucus Contrasts as a Tool for Diagnosis.

Purpose To assess the diagnostic accuracy of mucus contrast characterization by using magnetic resonance (MR) imaging to discriminate allergic bronchopulmonary aspergillosis (ABPA) in cystic fibrosis (CF). Materials and Methods The study was approved by the local Ethics Committee, and all patients or their parents gave written informed consent. One hundred ten consecutive patients with CF were screened between January 2014 and July 2015. All patients underwent a non-contrast material-enhanced MR protocol that included routine T1-weighted and T2-weighted sequences. The presence of mucus with both high T1 and low T2 signal intensities and the so-called inverted mucoid impaction signal (IMIS) sign was qualitatively and quantitatively assessed by two physicians who were blinded to all other data. The reference standard for a diagnosis of ABPA was the criteria of the Cystic Fibrosis Foundation Consensus Conference. ABPA status was followed up for 1 year. Reproducibility was assessed by using the κ test, correlation was assessed by using the Spearman coefficient, and diagnostic accuracy was assessed by calculating the sensitivity and specificity of IMIS. Results One hundred eight patients with CF were included (mean age, 20 years ± 11 [standard deviation]; range, 6-53 years): 18 patients with ABPA and 90 patients without ABPA. At the lobar level, inter- and intrareader reproducibility were very good (κ > 0.90). IMIS had 94% sensitivity (95% confidence interval [CI]: 73%, 99%) and 100% specificity (95% CI: 96%, 100%) for the diagnosis of ABPA. A complete resolution of IMIS was observed in patients with ABPA after 3 months of specific treatment that was significantly correlated with decrease in total immunoglobulin E level (ρ = 0.47; P = .04). Conclusion The IMIS sign was both specific and sensitive for the diagnosis of ABPA in CF. Allergic fungal inflammation appears to induce characteristic modifications of mucus contrasts that are assessable by using a noninvasive, contrast material-free, and radiation-free method. © RSNA, 2017 Online supplemental material is available for this article.

"Blood basophil activation is a reliable biomarker of allergic bronchopulmonary aspergillosis in cystic fibrosis." Yael Gernez, Jeffrey Waters, Bojana Mirković, Gillian M. Lavelle, Colleen E. Dunn, Zoe A. Davies, Cassie Everson, Rabindra Tirouvanziam, Elana Silver, Sylvan Wallenstein, Sanjay H. Chotirmall, Noel G. McElvaney, Leonore A. Herzenberg and Richard B. Moss. Eur Respir J 2016; 47: 177-185.

"Blood basophil activation is a reliable biomarker of allergic bronchopulmonary aspergillosis in cystic fibrosis." Yael Gernez, Jeffrey Waters, Bojana Mirković, Gillian M. Lavelle, Colleen E. Dunn, Zoe A. Davies, Cassie Everson, Rabindra Tirouvanziam, Elana Silver, Sylvan Wallenstein, Sanjay H. Chotirmall, Noel G. McElvaney, Leonore A. Herzenberg and Richard B. Moss. Eur Respir J 2016; 47: 177-185.

Basophil activation test determination of CD63 combined with CD203c is not superior to CD203c alone in identifying allergic bronchopulmonary aspergillosis in cystic fibrosis

Basophil activation test determination of CD63 combined with CD203c is not superior to CD203c alone in identifying allergic bronchopulmonary aspergillosis in cystic fibrosis

Omalizumab Treatment for Allergic Bronchopulmonary Aspergillosis in Cystic Fibrosis.

Allergic bronchopulmonary aspergillosis (ABPA) in cystic fibrosis (CF) is characterized by destructive changes in the airways. Long-term treatment with oral corticosteroids is often required for repeated exacerbations. Because elevated total IgE is a cardinal abnormality of ABPA, omalizumab has been used sporadically to decrease corticosteroid dose or totally replace corticosteroids. The aim of this report is to describe our experience with omalizumab treatment in patients with CF and ABPA. We conducted a review of 6 CF patients with ABPA receiving omalizumab. All patients were treated with oral prednisolone and itraconazole. Omalizumab was started if the patient was not responding to steroid treatment, which was determined according to serum IgE levels and/or clinical findings or depending on if there were side effects caused by steroid treatment. The mean age of patients at the beginning of omalizumab treatment was 16.1 years. One patient had a new diagnosis of ABPA; however, the others had the first to third exacerbation when treated with omalizumab. The mean duration of ABPA by the time that treatment with omalizumab started was 13 ± 12.4 months (range = 2-29 months). With omalizumab treatment, IgE levels were decreased in all patients, and Aspergillus-specific IgE levels were decreased in 4 patients; however, FEV1(% predicted) improved only in 2 patients who had mild disease. Corticosteroids were reduced in the first, second, and third months of omalizumab treatment in 2, 1, and 3 patients, respectively. In 2 patients, steroid treatment was stopped. None of the patients suffered from side effects of omalizumab. The mean duration of omalizumab treatment was 12.5 months (range = 6-18 months). This study showed steroid-sparing effect, decreasing IgE levels, and improvement in respiratory symptoms in 6 CF patients with omalizumab treatment. Although this is a small sample of the population, omalizumab may be an alternative therapy for ABPA in CF patients who fail to respond to systemic corticosteroids or have serious adverse effects.

Prevalence of Aspergillus sensitization and allergic bronchopulmonary aspergillosis in cystic fibrosis: systematic review and meta-analysis.

The prevalence of Aspergillus sensitization (AS) and allergic bronchopulmonary aspergillosis (ABPA) in cystic fibrosis (CF) has been varyingly reported. The aim of this systematic review was to estimate the overall prevalence of AS/ABPA in CF. We searched the PubMed and EmBase databases for studies reporting the prevalence of AS/ABPA in CF. We calculated the proportion with 95% confidence interval (CI) to assess the prevalence of AS and ABPA in the individual studies and then pooled the results using a random effects model. Statistical heterogeneity was assessed using the I2 test while publication bias was assessed using both graphical and statistical methods. Our search yielded 64 eligible studies. The pooled prevalence of AS was 39.1% (95% CI: 33.3-45.1) and was higher with skin test compared to specific IgE (43.8% vs. 32.8%, P = 0.002); however, the prevalence did not vary with the type of skin test used (intradermal or percutaneous). The prevalence of ABPA was 8.9% (95% CI: 7.4-10.7) and was higher in adults as compared to children (10.1% vs. 8.9%, P < 0.0001). There was a wide variation in the criteria used for diagnosing ABPA. Almost 50% (12/23) of the publications after 2004 used criteria other than the CF foundation criteria for diagnosing ABPA. There was significant statistical heterogeneity and evidence of publication bias. There is a high prevalence of AS and ABPA in patients with CF. Despite six decades of research, there is still a need to adopt uniform methodology and criteria for the diagnosis of AS/ABPA.

Blood basophil activation is a reliable biomarker of allergic bronchopulmonary aspergillosis in cystic fibrosis.

The diagnosis of cystic fibrosis (CF) patients with allergic bronchopulmonary aspergillosis (ABPA) is clinically challenging, due to the absence of an objective biological test. Since blood basophils play a major role in allergic responses, we hypothesised that changes in their surface activation pattern discriminate between CF patients with and without ABPA.We conducted a prospective longitudinal study (Stanford cohort) comparing basophil activation test CD203c levels by flow cytometry before and after activation with Aspergillus fumigatus allergen extract or recombinant Asp f1 in 20 CF patients with ABPA (CF-ABPA) and in two comparison groups: CF patients with A. fumigatus colonisation (AC) but without ABPA (CF-AC; n=13) and CF patients without either AC or ABPA (CF; n=12). Patients were tested every 6 months and when ill with pulmonary exacerbation. We also conducted cross-sectional validation in a separate patient set (Dublin cohort).Basophil CD203c surface expression reliably discriminated CF-ABPA from CF-AC and CF over time. Ex vivo stimulation with A. fumigatus extract or recombinant Asp f1 produced similar results within the Stanford (p<0.0001) and the Dublin cohorts. CF-ABPA patients were likelier to have elevated specific IgE to A. fumigatus and were less frequently co-infected with Staphylococcus aureus.Basophil CD203c upregulation is a suitable diagnostic and stable monitoring biomarker of ABPA in CF.

Letter to the Editor regarding the article by R. Casciaro et al. titled: ‘Role of nebulized amphotericin B in the management of allergic bronchopulmonary aspergillosis in cystic fibrosis: case report and review of literature’

To the Editors:We have read with interest the article by R. Casciaro et al. titled ‘Role of nebulized amphotericin B in the management of allergic bronchopulmonary aspergillosis in cystic fibrosis:...

Comment to Letter to the Editor regarding the article by R. Casciaro et al. titled ‘Role of nebulized amphotericin B in the management of allergic bronchopulmonary aspergillosis in cystic fibrosis: case report and review of literature’

Comment to Letter to the Editor regarding the article by R. Casciaro et al. titled ‘Role of nebulized amphotericin B in the management of allergic bronchopulmonary aspergillosis in cystic fibrosis: case report and review of literature’ Rosaria Casciaro, Federico Cresta, Aldo Naselli, Elio Castagnola Cystic Fibrosis Center, IRCCS Istituto Giannina Gaslini, Genova, Italy, Infectious Diseases Unit, IRCCS Istituto Giannina Gaslini, Genova, Italy

Role of nebulized amphotericin B in the management of allergic bronchopulmonary aspergillosis in cystic fibrosis: Case report and review of literature

Objectives:To review the data available in literature about nebulized amphotericin B (AMB) in the treatment of allergic bronchopulmonary aspergillosis (ABPA) in cystic fibrosis (CF) and to report our experience in the use of this drug, with a particular therapeutic scheme.Case Report:We used nebulized liposomal amphotericin B (L-AMB) in a patient affected by CF, complicated by ABPA. The previous combined treatment with oral steroids and azoles had no respiratory benefit and caused relevant side effects. Amphotericin B has always been well tolerated and permitted a slight steroid tapering. We also observed benefits in pulmonary function and laboratory tests.Conclusions:Few data are available in literature about the use of nebulized AMB in CF and there are no RCTs evaluating antifungals in CF-ABPA. In our opinion, the reported case suggests that nebulized L-AMB could represent a possible strategy in ABPA management in CF patients.

Serum markers for allergic bronchopulmonary aspergillosis in cystic fibrosis: State of the art and further challenges

Allergic bronchopulmonary aspergillosis (ABPA), which results from hypersensitivity, primarily to Aspergillus, represents a severe complication in patients suffering from asthma or cystic fibrosis (CF). Since early treatment of ABPA is supposed to prevent long-term damages, ABPA has to be diagnosed promptly. However, this diagnosis is not straightforward due to clinical and radiological features of ABPA overlapping with those of CF. Despite ABPA specific diagnosis criteria proposed by the Cystic Fibrosis Foundation in 2003, making a definitive ABPA diagnosis in CF patients remains a challenge. Recent advances in the immunopathogenesis of ABPA have initiated the development of new serological tests, such as the recently reported detection of specific IgE to recombinant A. fumigatus allergens, or Thymus- and activation-regulated chemokine (TARC / CCL17), both of which are of value in the diagnosis of APBA. We review in this paper the serum markers that can advance ABPA diagnosis in CF patients, ranging from the well known criteria (anti-A. fumigatus IgE, IgG, and precipitins) to the recent biomarkers (IgE towards recombinant A. fumigatus allergens or TARC detection). Taking into account the up-dated physiopathology of ABPA, we discuss their place and their usefulness, especially TARC, to improve early ABPA detection and monitoring in CF patients.

Use of Nebulized Amphotericin B in the Treatment of Allergic Bronchopulmonary Aspergillosis in Cystic Fibrosis

Background. Systemic steroids and adjunctive antifungal therapy are the cornerstone in treating allergic bronchopulmonary aspergillosis (ABPA) in the context of CF. Aim. Evaluate the use of inhaled amphotericin B (iAMB) as antifungal agent in this context. Methods. Report of 7 CF patients with recurrent or difficult to treat ABPA and failure to taper systemic corticosteroids treated with AMB deoxycholate (AMB-d) (Fungizone 25 mg 3× a week) or AMB lipid complex (ABLC) (Abelcet 50 mg twice weekly). Successful therapy was defined as steroid withdrawal without ABPA relapse within 12 months. Results. Therapy was successful in 6 of 7 patients treated with iAMB. In 5/6, lung function improved. The patient with treatment failure has concomitant MAC lung infection. Conclusion. Inhaled AMB may be an alternative to commonly used adjunctive antifungal therapy in the treatment of ABPA. More data are needed on safety and efficacy.

Tracheomalacia in Adults with Cystic Fibrosis: Determination of Prevalence and Severity with Dynamic Cine CT

HomeRadiologyVol. 275, No. 3 PreviousNext CommunicationsFree AccessErrataTracheomalacia in Adults with Cystic Fibrosis: Determination of Prevalence and Severity with Dynamic Cine CTShaunagh McDermott, Sinead C. Barry, Eoin E. Judge, Susan Collins, Pim A. de Jong, Harm A. W. M. Tiddens, Edward F. McKone, Charles G. Gallagher, Jonathan D. DoddShaunagh McDermott, Sinead C. Barry, Eoin E. Judge, Susan Collins, Pim A. de Jong, Harm A. W. M. Tiddens, Edward F. McKone, Charles G. Gallagher, Jonathan D. DoddShaunagh McDermottSinead C. BarryEoin E. JudgeSusan CollinsPim A. de JongHarm A. W. M. TiddensEdward F. McKoneCharles G. GallagherJonathan D. DoddPublished Online:May 21 2015https://doi.org/10.1148/radiol.2015154012MoreSectionsPDF ToolsImage ViewerAdd to favoritesCiteTrack CitationsPermissionsReprints ShareShare onFacebookTwitterLinked In Originally published in:Radiology 2009;252(2):577–586 DOI: 10.1148/radiol.2522081956Erratum in:Radiology 2015;275(3):934 DOI:10.1148/radiol.2015154012In the author list, the third author should be listed as Eoin P. Judge.Article HistoryPublished online: May 21 2015Published in print: June 2015 FiguresReferencesRelatedDetailsCited ByThat antibiotic prophylaxis should be used in children with repaired oesophageal atresia and or trachea-oesophageal fistula – The case in favourPeter J.Merkus2016 | Paediatric Respiratory Reviews, Vol. 18Accompanying This ArticleTracheomalacia in Adults with Cystic Fibrosis: Determination of Prevalence and Severity with Dynamic Cine CTAug 1 2009RadiologyRecommended Articles Allergic Bronchopulmonary Aspergillosis in Cystic Fibrosis: MR Imaging of Airway Mucus Contrasts as a Tool for DiagnosisRadiology2017Volume: 285Issue: 1pp. 261-269Use of Dynamic Chest Radiography to Assess Treatment of Pulmonary Exacerbations in Cystic FibrosisRadiology2022Volume: 303Issue: 3pp. 675-681Three-dimensional Isotropic Functional Imaging of Cystic Fibrosis Using Oxygen-enhanced MRI: Comparison with Hyperpolarized 3He MRIRadiology2018Volume: 290Issue: 1pp. 229-237Self-gated Non–Contrast-enhanced Functional Lung MR Imaging for Quantitative Ventilation Assessment in Patients with Cystic FibrosisRadiology2016Volume: 283Issue: 1pp. 242-251Quantification of Cystic Fibrosis Lung Disease with Radiomics-based CT ScoresRadiology: Cardiothoracic Imaging2020Volume: 2Issue: 6See More RSNA Education Exhibits CF-SCRIbeR Study: Cystic Fibrosis Structured Radiology Report, Standardisation of CT Reporting in Patients with Pulmonary Cystic Fibrosis (CF)Digital Posters2018Imaging in Cystic Fibrosis Follow-up: What is the News?Digital Posters2019Cystic Fibrosis in Adult Patients: A Pictorial Review of Common and Uncommon Abdominal Manifestations Digital Posters2018 RSNA Case Collection Pulmonary manifestations of cystic fibrosisRSNA Case Collection2020Allergic Bronchopulmonary AspergillosisRSNA Case Collection2021Mounier-Kuhn syndromeRSNA Case Collection2020 Vol. 275, No. 3 Metrics Altmetric Score PDF download

Clinical Significance of High-attenuation Mucus in Patients with Allergic Bronchopulmonary Aspergillosis

HomeRadioGraphicsVol. 28, No. 5 PreviousNext Letters to the EditorClinical Significance of High-attenuation Mucus in Patients with Allergic Bronchopulmonary AspergillosisRam Prakash Galwa, Pushpender Gupta, Hina Arif MumtazRam Prakash Galwa, Pushpender Gupta, Hina Arif MumtazAuthor AffiliationsDepartment of Radiodiagnosis and Imaging, Postgraduate Institute of Medical Education and Research Chandigarh 160012, India, e-mail: [email protected]Ram Prakash GalwaPushpender GuptaHina Arif MumtazPublished Online:Sep 1 2008https://doi.org/10.1148/radiographics.28.5.0281460MoreSectionsFull textPDF ToolsImage ViewerAdd to favoritesCiteTrack CitationsPermissionsReprints ShareShare onFacebookTwitterLinked In References1 JeongYJ, Kim KI, Seo IJ, et al. Eosinophilic lung diseases: a clinical, radiologic, and pathologic overview. RadioGraphics2007; 27: 617–639. Link, Google Scholar2 AgarwalR, Gupta D, Agarwal AN, Saxena AK, Chakrabarti A, Jindal SK. Clinical significance of hyperattenuating mucoid impaction in allergic bronchopulmonary aspergillosis. Chest2007; 132: 1183–1190. Crossref, Medline, Google ScholarArticle HistoryPublished in print: Sept 2008 FiguresReferencesRelatedDetailsRecommended Articles Allergic Bronchopulmonary Aspergillosis in Cystic Fibrosis: MR Imaging of Airway Mucus Contrasts as a Tool for DiagnosisRadiology2017Volume: 285Issue: 1pp. 261-269Cardiopulmonary and Gastrointestinal Manifestations of Eosinophil- associated Diseases and Idiopathic Hypereosinophilic Syndromes: Multimodality Imaging ApproachRadioGraphics2016Volume: 36Issue: 2pp. 433-451Bronchiectasis in Low-Dose CT Screening for Lung CancerRadiology2022Volume: 304Issue: 2pp. 437-447Inverted Mucoid Impaction Signal in Allergic Bronchopulmonary Aspergillosis: Are We Calling a Spade a Big Spoon?Radiology2017Volume: 285Issue: 2pp. 691-692CT Features of Lymphatic Plastic Bronchitis in Adults: Correlation with Multimodality Lymphatic ImagingRadiology: Cardiothoracic Imaging2022Volume: 4Issue: 2See More RSNA Education Exhibits Mucus Plugs Provide Clues to Respiratory Disease Diagnosis and ManagementDigital Posters2020The Role of Thoracic Imaging in Cystic Fibrosis: Correlation with Clinical and Physiological FindingsDigital Posters2019Wheezy-breezy? Not So Simple: Imaging of Asthma Complications and SimulatorsDigital Posters2020 RSNA Case Collection Allergic Bronchopulmonary AspergillosisRSNA Case Collection2021Pulmonary aspergillomaRSNA Case Collection2020Pulmonary manifestations of cystic fibrosisRSNA Case Collection2020 Vol. 28, No. 5 Metrics Altmetric Score PDF download

Pathogenesis of allergic bronchopulmonary aspergillosis in cystic fibrosis: current understanding and future directions

Allergic bronchopulmonary aspergillosis (ABPA) is an allergic disease characterized clinically by wheezing, pulmonary infiltrates, bronchiectasis, and fibrosis that affects patients with asthma and cystic fibrosis (CF). Although this disease has been characterized by a Th2 immune response to Aspergillus, the disease has some features such as central bronchiectasis which is not seen in other Th2 driven lung diseases such as atopic asthma. Here we will review the current pathophysiology of ABPA in CF and highlight new molecules that may affect immune responses against Aspergillus and ABPA disease pathogenesis.