Immunological mechanisms behind the cystic fibrosis-ABPA link

Cystic fibrosis, atopy, asthma and ABPA.

The role of atopy on cystic fibrosis (CF) progression remains unclear but evidence suggests that it may influence the appearance of co-morbid conditions such as CF asthma or allergic bronchopulmonary aspergillosis (ABPA). Recognising asthma in patients with CF is not always easy but the identification of atopic markers favours the diagnosis. Physicians should be aware of this fact in order to a...

Steroid dependency despite omalizumab treatment of ABPA in cystic fibrosis.

macrocyclic structure as postulated by the authors; nevertheless this hypothesis must not be rejected and should always be checked. Thus, all mild to severe adverse hypersensitivity reactions should be accurately documented with most of the available gadolinium in order to determine cross reactivities. This is all the more important since challenge with another gadolinium agent giving negative ...

Immunological testing in non-cystic fibrosis bronchiectasis.

The review of non-cystic fibrosis bronchiectasis by Smith elegantly highlights the lack of evidence available in some areas. In particular, approach to immunological testing is varied. Table 2 on page 134 in this review describes the diagnostic features required for immunodeficiency (including immunoglobulin G [IgG] subclass 2 deficiency amongst other IgG subclass deficiencies). However, IgG su...

Immunological aspects on cystic fibrosis lung disease

Lumacaftor-ivacaftor (Orkambi) for cystic fibrosis: behind the 'breakthrough'.

Lumacaftor-ivacaftor (Orkambi) was recently approved by the Food and Drug Administration (FDA) in the USA to treat patients at least 12 years old who have cystic fibrosis due to two copies of the F508del (Phe508del) mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Lumacaftor-ivacaftor has been designated a ‘breakthrough’ and is slated to cost $259 000 per year pe...