Diffuse panbronchiolitis

Poletti, Venerino

doi:10.1183/09031936.06.00131805

Cited by 126 publications

(88 citation statements)

References 67 publications

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“…In these patients, mucoid P. aeruginosa infection, where it occurs in ∼15% of affected individuals, primarily emerges after 30 years of age [14], whereas among CF patients, over 80% are infected with this organism by late adolescence. Furthermore, in the disease diffuse panbronchiolitis, found commonly in Japanese patients with chronic obstructive pulmonary disease and thus impaired interactions of the airway epithelium with P. aeruginosa, mucoid P. aeruginosa infection is common, although CFTR function is intact [15]. Thus, when one views the pathogenesis of CF lung disease as involving multiple lung abnormalities derived from loss of functional CFTR and thickened mucus with the proposal that CFTR is a receptor for P. aeruginosa mediating critical aspects of innate immunity, a picture emerges that explains how different physiologic abnormalities resulting from loss of proper CFTR function all contribute to the pathogenesis of P. aeruginosa in the CF lung.…”

Section: Histopathologic Basis For Defining Relevant Interactions Betmentioning

confidence: 99%

Airway epithelial control of Pseudomonas aeruginosa infection in cystic fibrosis

Campodónico

Gadjeva

Paradis-Bleau

et al. 2008

Trends in Molecular Medicine

100

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Defective expression or function of the cystic fibrosis transmembrane conductance regulator (CFTR) underlies the hypersusceptibility of cystic fibrosis (CF) patients to chronic airway infections, particularly with Pseudomonas aeruginosa. CFTR is involved in the specific recognition of P. aeruginosa, thereby contributing to effective innate immunity and proper hydration of the airway surface layer (ASL). In CF, the airway epithelium fails to initiate an appropriate innate immune response, allowing the microbe to bind to mucus plugs that are then not properly cleared because of the dehydrated ASL. Recent studies have identified numerous CFTR-dependent factors that are recruited to the epithelial plasma membrane in response to infection and that are needed for bacterial clearance, a process that is defective in CF patients hypersusceptible to infection with this organism. Pseudomonas aeruginosa in cystic fibrosisCystic fibrosis (CF) is unique among human genetic disorders in that mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, which encodes a protein whose primary function described to date has been to regulate conductance of ions in and out of cells and intracellular vacuoles, lead to hypersusceptibility to chronic lung infection. Human diseases wherein individuals homozygous for mutant genes are predisposed to infections serve as a firm foundation for understanding the molecular, cellular, physiologic and immunologic aspects of normal and compromised resistance to infection. CF is not just a prime example of how we can learn about normal and pathologic states; it could, in fact, be the only known human genetically determined disease wherein infection with a single pathogen -Pseudomonas aeruginosa -drives the vast majority of morbidity, clinical deterioration and ultimately life-shortening mortality encountered in this disease. In humans with significant compromises to their immune system, such as advanced AIDS, we would normally expect to see a plethora of pathogens take advantage of this debilitated state, but this is not the case in CF.CF is characterized by the emergence and persistence of (and, ultimately, the inability to clear) chronic infection with a variant of P. aeruginosa (mucoid P. aeruginosa) that overproduces a surface polysaccharide known as alginate. The organism undergoes other significant genetic adaptations and selections within the CF lung, and it is the emergence of mucoid P. aeruginosa that is the primary determinant of the clinical course of this disease [1,2]. In those individuals with either an uncommon but fortuitous protective immune response to specific P. aeruginosa antigens [3,4] Histopathologic basis for defining relevant interactions between P.aeruginosa and the CF airway epitheliumAlthough CF is a multisystem disease characterized by GI and nutritional abnormalities, salt loss syndromes and male urogenital abnormalities, the major clinical problem for CF patients is chronic sinopulmonary disease. Therefore, relying on observations made fr...

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Section: Histopathologic Basis For Defining Relevant Interactions Betmentioning

confidence: 99%

Airway epithelial control of Pseudomonas aeruginosa infection in cystic fibrosis

Campodónico

Gadjeva

Paradis-Bleau

et al. 2008

Trends in Molecular Medicine

100

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“…DPB is a progressive inflammatory airway disease reported almost exclusively in East Asians and characterised by chronic airway infection, often complicated by Pseudomonas aeruginosa infection, chronic productive cough, dyspnoea, airflow limitation and chronic sinusitis [11,12]. The term ''diffuse'' refers to the distribution of the lesions throughout both lungs, while ''pan-'' refers to the inflammatory involvement of all layers of the respiratory bronchioles.…”

Section: Diffuse Panbronchiolitismentioning

confidence: 99%

Long-term macrolide treatment for chronic respiratory disease

2012

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Long-term macrolide treatment was first shown to alter the natural history of diffuse panbronchiolitis (DPB) in the late 1980s. Since then, macrolides have been demonstrated to exert antiinflammatory and immunomodulatory activity in addition to being antimicrobial. Indeed, their spectrum of action extends to the regulation of leukocyte function and production of inflammatory mediators, control of mucus hypersecretion, resolution of inflammation and modulation of host defence mechanisms. As such, the potential benefit of macrolide antibiotics has been evaluated in a variety of chronic respiratory diseases. The best studied condition is cystic fibrosis, of which there have been six randomised controlled trials showing evidence of benefit. However, most of the studies were limited by small numbers of patients and short follow-up. More recently, landmark studies have demonstrated the efficacy of azithromycin in reducing the risk of acute exacerbations in patients with chronic obstructive pulmonary disease, but the optimal duration and dosing of macrolide treatment remain uncertain.With the exception of patients with DPB and cystic fibrosis, until clear evidence of efficacy is available, the long-term use of macrolides should be limited to highly selected patients after careful evaluation of benefit and harm, or in the context of randomised controlled clinical trials. @ERSpublications Several studies evaluating macrolide antibiotics for the treatment of chronic respiratory diseases are underway

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“…Diffuse panbronchiolitis has been the topic of recent reviews [171,172], and will not be extensively discussed here.…”

Section: Diffuse Panbronchiolitismentioning

confidence: 99%

Small airways diseases, excluding asthma and COPD: an overview

Burgel¹,

Bergeron²,

Blic³

et al. 2013

European Respiratory Review

104

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This review is the summary of a workshop on small airways disease, which took place in Porquerolles, France in November 2011. The purpose of this workshop was to review the evidence on small airways (bronchiolar) involvement under various pathophysiological circumstances, excluding asthma and chronic obstructive pulmonary disease. Histopathological patterns associated with small airways disease were reviewed, including cellular and obliterative bronchiolitis. Many pathophysiological conditions have been associated with small airways disease including airway infections, connective tissue diseases and inflammatory bowel diseases, bone marrow and lung transplantation, common variable immunodeficiency disorders, diffuse panbronchiolitis, and diseases related to environmental exposures to pollutants, allergens and drugs. Pathogenesis, clinical presentation, a computed tomography scan and pulmonary function test findings are reviewed, and therapeutic options are described with the objective of providing an integrative approach to these disorders.

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Diffuse panbronchiolitis

Cited by 126 publications

References 67 publications

Airway epithelial control of Pseudomonas aeruginosa infection in cystic fibrosis

Airway epithelial control of Pseudomonas aeruginosa infection in cystic fibrosis

Long-term macrolide treatment for chronic respiratory disease

Small airways diseases, excluding asthma and COPD: an overview

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