Adult Patients With Bronchiectasis: A First Look at the US Bronchiectasis Research Registry.
Mayo Clinic1, Georgetown University2, Oregon Health & Science University3, University of North Carolina at Chapel Hill4, Mount Sinai St. Luke's and Mount Sinai Roosevelt5, University of Texas Health Science Center at San Antonio6, University of Connecticut7, University of Miami8, Columbia University Medical Center9, University of Pennsylvania10
TL;DR: Adult patients with bronchiectasis enrolled in the US BRR are described, with differences noted in demographic, radiographic, microbiological, and treatment variables based on stratification of the presence of NTM.
About: This article is published in Chest.The article was published on 2017-05-01 and is currently open access. It has received 243 citations till now. The article focuses on the topics: Bronchiectasis.
Citations
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University of Colorado Denver1, Boston University2, Paris Diderot University3, University of Texas System4, University of Zurich5, McMaster University6, University of Texas Health Science Center at San Antonio7, Pierre-and-Marie-Curie University8, University Health Network9, Bellvitge University Hospital10, Duke University11, Radboud University Nijmegen12, University of Freiburg13, Oregon Health & Science University14
TL;DR: This guideline focuses on pulmonary disease in adults (without cystic fibrosis or human immunodeficiency virus infection) caused by the most common NTM pathogens such as Mycobacterium avium complex, MycOBacterium kansasii, and Myc Cobacterium xenopi among the slowly growing NTM and MyCobacterius abscessus among the rapidly growing N TM.
Abstract: Nontuberculous mycobacteria (NTM) represent over 190 species and subspecies, some of which can produce disease in humans of all ages and can affect both pulmonary and extrapulmonary sites. This guideline focuses on pulmonary disease in adults (without cystic fibrosis or human immunodeficiency virus infection) caused by the most common NTM pathogens such as Mycobacterium avium complex, Mycobacterium kansasii, and Mycobacterium xenopi among the slowly growing NTM and Mycobacterium abscessus among the rapidly growing NTM. A panel of experts was carefully selected by leading international respiratory medicine and infectious diseases societies (ATS, ERS, ESCMID, IDSA) and included specialists in pulmonary medicine, infectious diseases and clinical microbiology, laboratory medicine, and patient advocacy. Systematic reviews were conducted around each of 22 PICO (Population, Intervention, Comparator, Outcome) questions and the recommendations were formulated, written, and graded using the GRADE (Grading of Recommendations Assessment, Development, and Evaluation) approach. Thirty-one evidence-based recommendations about treatment of NTM pulmonary disease are provided. This guideline is intended for use by healthcare professionals who care for patients with NTM pulmonary disease, including specialists in infectious diseases and pulmonary diseases.
329 citations
University of Colorado Denver1, Boston University2, Paris Diderot University3, University of Texas System4, University of Zurich5, McMaster University6, University of Texas Health Science Center at San Antonio7, Pierre-and-Marie-Curie University8, University Health Network9, Bellvitge University Hospital10, Duke University11, Radboud University Nijmegen12, University of Freiburg13, Oregon Health & Science University14
TL;DR: This guideline focuses on pulmonary disease in adults (without cystic fibrosis or human immunodeficiency virus infection) caused by the most common NTM pathogens such as Mycobacterium avium complex, MycOBacterium kansasii, and Myc Cobacterium xenopi among the slowly growing NTM and MyCobacteria abscessus among the rapidly growing N TM.
Abstract: Nontuberculous mycobacteria (NTM) represent over 190 species and subspecies, some of which can produce disease in humans of all ages and can affect both pulmonary and extrapulmonary sites. This guideline focuses on pulmonary disease in adults (without cystic fibrosis or human immunodeficiency virus infection) caused by the most common NTM pathogens such as Mycobacterium avium complex, Mycobacterium kansasii, and Mycobacterium xenopi among the slowly growing NTM and Mycobacterium abscessus among the rapidly growing NTM. A panel of experts was carefully selected by leading international respiratory medicine and infectious diseases societies (ATS, ERS, ESCMID, IDSA) and included specialists in pulmonary medicine, infectious diseases and clinical microbiology, laboratory medicine, and patient advocacy. Systematic reviews were conducted around each of 22 PICO (Population, Intervention, Comparator, Outcome) questions and the recommendations were formulated, written, and graded using the GRADE (Grading of Recommendations Assessment, Development, and Evaluation) approach. Thirty-one evidence-based recommendations about treatment of NTM pulmonary disease are provided. This guideline is intended for use by healthcare professionals who care for patients with NTM pulmonary disease, including specialists in infectious diseases and pulmonary diseases.
297 citations
University of Colorado Denver1, Boston University2, Paris Diderot University3, University of Texas System4, University of Zurich5, McMaster University6, University of Texas Health Science Center at San Antonio7, Pierre-and-Marie-Curie University8, University Health Network9, Bellvitge University Hospital10, Duke University11, Radboud University Nijmegen12, University of Freiburg13, Oregon Health & Science University14
TL;DR: This guideline focuses on pulmonary disease in adults (without cystic fibrosis or human immunodeficiency virus infection) caused by the most common NTM pathogens such as Mycobacterium avium complex, MycOBacterium kansasii, and Myc Cobacterium xenopi among the slowly growing NTM and MyCobacterius abscessus among the rapidly growing N TM.
Abstract: Nontuberculous mycobacteria (NTM) represent over 190 species and subspecies, some of which can produce disease in humans of all ages and can affect both pulmonary and extrapulmonary sites. This guideline focuses on pulmonary disease in adults (without cystic fibrosis or human immunodeficiency virus infection) caused by the most common NTM pathogens such as Mycobacterium avium complex, Mycobacterium kansasii, and Mycobacterium xenopi among the slowly growing NTM and Mycobacterium abscessus among the rapidly growing NTM. A panel of experts was carefully selected by leading international respiratory medicine and infectious diseases societies (ATS, ERS, ESCMID, IDSA) and included specialists in pulmonary medicine, infectious diseases and clinical microbiology, laboratory medicine, and patient advocacy. Systematic reviews were conducted around each of 22 PICO (Population, Intervention, Comparator, Outcome) questions and the recommendations were formulated, written, and graded using the GRADE (Grading of Recommendations Assessment, Development, and Evaluation) approach. Thirty-one evidence-based recommendations about treatment of NTM pulmonary disease are provided. This guideline is intended for use by healthcare professionals who care for patients with NTM pulmonary disease, including specialists in infectious diseases and pulmonary diseases.
217 citations
TL;DR: Some of the recent advances in endotyping, genetics, and disease heterogeneity of bronchiectasis are described including observations related to the microbiome.
195 citations
TL;DR: Muco-Obstructive Lung Diseases This review covers the normal mechanisms of mucus formation and how they are abnormal in common conditions — such as COPD, cystic fibrosis, and non-cystic Fibrosis — and the abnormal mechanisms in these conditions.
Abstract: Muco-Obstructive Lung Diseases This review covers the normal mechanisms of mucus formation and how they are abnormal in common conditions — such as COPD, cystic fibrosis, and non–cystic fibrosis br...
183 citations
References
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TL;DR: These guidelines were designed to identify relevant studies in non-cystic fibrosis (CF) bronchiectasis, to provide guidelines on management based on published studies where possible or a consensus view, and to identify gaps in knowledge and identify areas for future study.
Abstract: The diagnosis, investigation and particularly management of bronchiectasis has been largely empirical and the subject of relatively few controlled clinical trials. There are no clear guidelines, although an Australian position statement has been published concerning bronchiectasis in children. The purposes of these guidelines were therefore threefold: (1) to identify relevant studies in non-cystic fibrosis (CF) bronchiectasis; (2) to provide guidelines on management based on published studies where possible or a consensus view; and (3) to identify gaps in our knowledge and identify areas for future study.
848 citations
TL;DR: Intensive investigation of this population of patients with bronchiectasis led to identification of one or more causative factor in 47% of cases, and the cause identified had implications for prognosis and treatment.
Abstract: Bronchiectasis is a pathologic description of lung damage characterized by inflamed and dilated thick-walled bronchi. These findings may result from a number of possible causes and these may influence treatment and prognosis. The aim of this study was to determine causative factors in 150 adults with bronchiectasis (56 male, 94 female) identified using high-resolution computerized tomography. Relevant factors were identified in the clinical history; cystic fibrosis gene mutation analysis was performed; humoral immune defects were determined by measuring immunoglobulins, IgG subclasses and functional response to Pneumovax II vaccine; assessment was made of neutrophil function (respiratory burst, adhesion molecule expression, and chemotaxis); ciliary function was observed and those likely to have allergic bronchopulmonary aspergillosis (ABPA) were identified. Causes identified were: immune defects (12 cases), cystic fibrosis (4), Young's syndrome (5), ciliary dysfunction (3), aspiration (6), panbronchiolitis (1), congenital defect (1), ABPA (11), rheumatoid arthritis (4), and early childhood pneumonia, pertussis, or measles (44). Intensive investigation of this population of patients with bronchiectasis led to identification of one or more causative factor in 47% of cases. In 22 patients (15%), the cause identified had implications for prognosis and treatment.
613 citations
Additional excerpts
...(%) 1,645 No growth in any culture 136 (8) 85 (8) 51 (9) Multiple pathogens isolated 1,050 (64) 736 (67) 314 (58)...
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...(%) No growth in any culture 1,406 93 (7) 68 (8) 25 (5) ....
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...57 Haemophilus influenzae 1,406 116 (8) 72 (8) 44 (9) ....
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...01 Rheumatologic disease 1,775 142 (8) 87 (8) 55 (8) ....
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...01 Only 1 of the above sites 1,491 119 (8) 78 (8) 41 (7) < ....
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TL;DR: Patients with PNTM infection are taller and leaner than control subjects, with high rates of scoliosis, pectus excavatum, mitral valve prolapse, and cystic fibrosis transmembrane conductance regulator mutations, but without recognized immune defects.
Abstract: Rationale: Pulmonary nontuberculous mycobacterial (PNTM) disease is increasing, but predisposing features have been elusive.
Objectives: To prospectively determine the morphotype, immunophenotype, and cystic fibrosis transmembrane conductance regulator genotype in a large cohort with PNTM.
Methods: We prospectively enrolled 63 patients with PNTM infection, each of whom had computerized tomography, echocardiogram, pulmonary function, and flow cytometry of peripheral blood. In vitro cytokine production in response to mitogen, LPS, and cytokines was performed. Anthropometric measurements were compared with National Health and Nutrition Examination Survey (NHANES) age- and ethnicity-matched female control subjects extracted from the NHANES 2001–2002 dataset.
Measurements and Main Results: Patients were 59.9 (±9.8 yr [SD]) old, and 5.4 (±7.9 yr) from diagnosis to enrollment. Patients were 95% female, 91% white, and 68% lifetime nonsmokers. A total of 46 were infected with Mycobacterium avium complex, M. xenopi, or M. kansasii; 17 were infected with rapidly growing mycobacteria. Female patients were significantly taller (164.7 vs. 161.0 cm; P < 0.001) and thinner (body mass index, 21.1 vs. 28.2; P < 0.001) than matched NHANES control subjects, and thinner (body mass index, 21.1 vs. 26.8; P = 0.002) than patients with disseminated nontuberculous mycobacterial infection. A total of 51% of patients had scoliosis, 11% pectus excavatum, and 9% mitral valve prolapse, all significantly more than reference populations. Stimulated cytokine production was similar to that of healthy control subjects, including the IFN-γ/IL-12 pathway. CD4+, CD8+, B, and natural killer cell numbers were normal. A total of 36% of patients had mutations in the cystic fibrosis transmembrane conductance regulator gene.
Conclusions: Patients with PNTM infection are taller and leaner than control subjects, with high rates of scoliosis, pectus excavatum, mitral valve prolapse, and cystic fibrosis transmembrane conductance regulator mutations, but without recognized immune defects.
389 citations
Additional excerpts
...06 Restriction 317 (20) 200 (20) 117 (21) ....
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...10 Aspergillus species 1,087 211 (19) 159 (21) 52 (16) ....
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TL;DR: It is suggested that over 110,000 persons in the United States may be receiving treatment for bronchiectasis, resulting in additional medical-care expenditures of $630 million annually.
Abstract: :We employed a retrospective cohort design to estimate the prevalence and economic burden of bronchiectasis Data were obtained from the health-care claims processing systems of more than 30 US health plans (with a combined total of 56 million covered lives) and spanned the period January 1
292 citations
Additional excerpts
...10 Staphylococcus aureus 1,406 170 (12) 92 (10) 78 (15) < ....
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...01 Mycobacterium avium complex 1,314 484 (37) 484 (52) 0 Mycobacterium abscessus/chelonae 1,314 130 (10) 130 (14) 0 Mycobacterium kansasii 1,314 8 (1) 8 (1) 0 Mycobacterium gordonae 1,314 37 (3) 37 (4) 0 Other mycobacterial species 1,314 36 (3) 36 (4) 0 Nocardia 1,314 9 (1) 8 (1) 1 (0) Fungal culture findings, No....
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...01 Inhaled suppressive antibiotics 1,759 178 (10) 113 (10) 65 (10) ....
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...01 Any oral steroid 1,789 237 (13) 112 (10) 125 (19) < ....
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TL;DR: A concise clinical review focuses on the major etiologies, diagnostic testing, microbiology, and management of patients with adult non-cystic fibrosis bronchiectasis, and outline current therapies and review the data that support their use.
Abstract: There is renewed interest in non–cystic fibrosis bronchiectasis, which is a cause of significant morbidity in adults and can be diagnosed by high-resolution chest computed tomography scan. No longer mainly a complication after pulmonary infection with Mycobacterium tuberculosis, diverse disease processes and mechanisms have been demonstrated to result in the chronic cough, purulent sputum production, and airway dilation that characterize this disease. Improved understanding of the role of mucus stasis in causing bacterial colonization has led to increased emphasis on the use of therapies that enhance airway clearance. Inhalational antibiotics reduce the bacterial burden associated with a worse outcome. Low-dose, chronic macrolide therapy has been shown to decrease exacerbation frequency and airway inflammation. For the first time, a number of therapies for non–cystic fibrosis bronchiectasis are undergoing testing in clinical research trials designed specifically for this population. This concise clinical ...
281 citations
"Adult Patients With Bronchiectasis:..." refers background in this paper
...74 Former 693 (38) 447 (39) 246 (37) Current 28 (2) 18 (2) 10 (2) Chest wall deformity, No....
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...02 Pectus excavatum 56 (3) 39 (4) 17 (3) Other 18 (1) 6 (1) 12 (2) Otitis or rhinosinusitis, No....
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...01 Medicaid and other state programs 49 (3) 24 (2) 25 (4)...
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...60 Chronic ulcerative colitis or Crohn’s disease 1,795 47 (3) 26 (2) 21 (3) ....
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...01 Primary ciliary dyskinesia 1,791 52 (3) 20 (2) 32 (5) < ....
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