PPFE IN BRONCHIECTASIS - 2.0

• Research type

  Research Study

• Full title

  PPFE PREVALENCE AND CLINICAL IMPACT IN BRONCHIECTASIS

• IRAS ID

  344201

• Contact name

  Ali Alshukry

• Contact email

  bb-sponsor@nottingham.ac.uk

• Sponsor organisation

  University of Nottingham

• Duration of Study in the UK

  0 years, 6 months, 31 days

• Research summary

  Bronchiectasis is a permanent and, usually, progressive bronchial dilation resulting from infection and chronic inflammation of the airway, leading to destruction of the bronchial wall.
  Bronchiectasis is associated with chronic and frequently purulent expectoration, multiple exacerbations and potentially disabling dyspnoea. These events gradually worsen the quality of life and lung function. In recent years, bronchiectasis has become a major health concern for several reasons: a significant increase in the number of diagnoses, the increase in the mortality rate and in hospital admissions, the considerable health costs involved, its negative impact on the quality of life and pulmonary function of patients.
  Moreover, bronchiectasis is a complex and heterogenous disease with multiple aetiologies and comorbidities.

  Pleuro-parenchymal fibroelastosis (PPFE) is a rare fibrosing lung disease, affecting the visceral pleura and the subpleural parenchyma with an upper lobe predilection, included as a distinct clinicopathologic entity in the latest international multidisciplinary classification of the idiopathic interstitial pneumonias (IIP) [3].
  The aetiology has yet to be clearly established, but a large proportion of reported PPFE cases has been described in association with lung, bone marrow, and hematopoietic cell transplantations, in association with a number of chemo- therapy drugs, as well as with occupational exposures such as aluminosilicate dust. In the case series by Reddy et al., approximately half of cases reported a history of recurrent lower respiratory tract infections, suggesting that repeated inflammatory damage caused by pulmonary infections may contribute to the development/progression of PPFE in a proportion of patients

  Observations from daily clinical practice suggest a higher prevalence of PPFE in non-cystic fibrosis (non-CF) bronchiectasis compared to general population. Therefore, the main aim of the present project is to estimate the prevalence of PPFE in non-CF bronchiectasis, and to explore potential functional/radiological/microbiological features associated with this morphological pattern.
  As secondary objective, the project aims to assess the impact of PPFE.

• REC name

  West of Scotland REC 1

• REC reference

  24/WS/0154

• Date of REC Opinion

  12 Dec 2024

• REC opinion

  Further Information Favourable Opinion