Introduction Up to 70% of patients with Primary Immunodeficiency syndromes such as Common Variable Immunodeficiency (CVID) have bronchiectasis. Within this population it is a major driver of morbidity.¹

The Bronchiectasis Severity Index (BSI) is capable of accurately categorising non-cystic fibrosis bronchiectasis patients into three severity groups that predict risk of hospitalisation and mortality at one and four years.² It consists of nine clinical parameters, and was derived and validated in a diverse international bronchiectasis population. Mild disease is defined as a BSI score of <4, moderate 5–8 and severe >9.

This study aims to assess the relative severity of bronchiectasis associated with primary immunodeficiency.

Methods 24 Patients from the Royal Free Hospital, London and 22 patients from the Freeman Hospital Newcastle were recruited. Age, body mass index,% predicted FEV1, number of hospitalisations in the last 2 years, number of exacerbations in the last year, medical research council dyspnoea (MRCD) score, Pseudomonas and other pathogen colonisation status and number or lobes involved on CT chest were obtained to calculate the BSI. Statistical analysis was carried out using SPSS V11.

Results The 46 patients were 67.4% female with a mean age of 55.9. There were no significant differences in age, gender or disease severity between the two centres. The median BSI was 4 (i.e. mild disease).

56% of patients had mild disease, 21.7% were moderate and 21.7% severe bronchiectasis. These patients had markedly less severe disease than the mixed aetiology population of 603 patients used to derive the scoring tool.

Conclusion Patients with primary Immunodeficiency associated bronchiectasis were younger with less severe disease compared to the BSI cohort population previously reported. This suggests good multidisciplinary care in Primary Immunodeficiency with earlier referral to respiratory specialists. It also correlates with our prior longitudinal data that FEV1 decline in immunodeficiency-related bronchiectasis is less rapid than other aetiologies.¹

Reference

1. Hurst JR et al. Activity, severity and impact of respiratory disease in primary antibody deficiency syndromes. J C Immunol 2014;34:68–75

2. Chalmers JD et al. The bronchiectasis severity index. An international derivation and validation study. Am J RCCM 2014;189:576–585

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Abstract P79 Figure 1

Severity Distribution in all aetiologies vs primary immunodeficiency bronchiectasis