Immunodeficiency in Bronchiectasis

Coulter, Tanya; Devlin, Lisa; Downey, D.G.; Elborn, J. Stuart; Edgar, J. D.

doi:10.1007/978-3-319-61452-6_7

Cited by 2 publications

(2 citation statements)

References 125 publications

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“…Notably, secondary immunosuppression through necessary steroid use in ABPA treatment confers the risk of transitioning to other Aspergillus-associated disease states including CPA. 41,44,45 Chronic Pulmonary Aspergillosis CPA, also referred to as semi-invasive or subacute aspergillosis, is most commonly observed as a complication of established and severe bronchiectasis. 13,34,46 Unlike IA, that remains characterized by vascular invasion, CPA is limited to slowly progressing cavitary lesions of the lung parenchyma that follows Aspergillus infection (►Table 1).…”

Section: Allergic Bronchopulmonary Aspergillosismentioning

confidence: 99%

Aspergillus-Associated Endophenotypes in Bronchiectasis

Jaggi

Ter

Aogáin

et al. 2021

Semin Respir Crit Care Med

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Bronchiectasis is a chronic condition of global relevance resulting in permanent and irreversible structural airway damage. Bacterial infection in bronchiectasis is well studied; however, recent molecular studies identify fungi as important pathogens, either independently or in association with bacteria. Aspergillus species are established fungal pathogens in cystic fibrosis and their role is now increasingly being recognized in noncystic fibrosis bronchiectasis. While the healthy airway is constantly exposed to ubiquitously present Aspergillus conidia in the environment, anatomically damaged airways appear more prone to colonization and subsequent infection by this fungal group. Aspergilli possess diverse immunopathological mechanistic capabilities and when coupled with innate immune defects in a susceptible host, such as that observed in bronchiectasis, it may promote a range of clinical manifestations including sensitization, allergic bronchopulmonary aspergillosis, Aspergillus bronchitis, and/or invasive aspergillosis. How such clinical states influence “endophenotypes” in bronchiectasis is therefore of importance, as each Aspergillus-associated disease state has overlapping features with bronchiectasis itself, and can evolve, depending on underlying host immunity from one type into another. Concurrent Aspergillus infection complicates the clinical course and exacerbations in bronchiectasis and therefore dedicated research to better understand the Aspergillus-host interaction in the bronchiectasis airway is now warranted.

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Section: Allergic Bronchopulmonary Aspergillosismentioning

confidence: 99%

Aspergillus-Associated Endophenotypes in Bronchiectasis

Jaggi

Ter

Aogáin

et al. 2021

Semin Respir Crit Care Med

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“…Individuals with APDS and bronchiectasis should be treated as in other patients with bronchiectasis, including airway clearance, physiotherapy, influenza immunization, patient education antibiotic treatment for infective exacerbations, the consideration of interval intensive physiotherapy and antibiotics, nebulized hypertonic saline and bronchodilators, and prophylactic antibiotics where these therapies may benefit. It is recommended that the management and monitoring of patients with bronchiectasis and immune deficiency should be provided through a joint clinical immunology and respiratory (±pediatricians) care model with access to specialist respiratory nursing and physiotherapy services with an expertise in bronchiectasis ( 16 – 18 ).…”

Section: Immunoglobulin Replacement Therapymentioning

confidence: 99%

The Treatment of Activated PI3Kδ Syndrome

Coulter

Cant

2018

Front. Immunol.

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Activated phosphoinositide 3-kinase δ syndrome (APDS), also known as PASLI disease (p110d-activating mutation causing senescent T cells, lymphadenopathy, and immunodeficiency) are combined immunodeficiencies resulting from gain-of-function mutations in the genes (PIK3CD and PIK3R1) encoding the subunits of phosphoinositide 3-kinase δ (PI3Kδ) and were first described in 2013. These mutations result in the hyperactivation of the PI3K/AKT/mTOR/S6K signally pathways. In this mini-review we have detailed the current treatment options for APDS. These treatments including conventional immunodeficiency therapies such as immunoglobulin replacement, antibiotic prophylaxis, and hematopoietic stem cell transplant. We also discuss the more targeted therapies of mTOR inhibition with sirolimus and selective PI3Kδ inhibitors.

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Immunodeficiency in Bronchiectasis

Cited by 2 publications

References 125 publications

Aspergillus-Associated Endophenotypes in Bronchiectasis

Aspergillus-Associated Endophenotypes in Bronchiectasis

The Treatment of Activated PI3Kδ Syndrome

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