The severity of bronchiectasis could predict subsequent mortality in primary antibody deficiency (PAD) syndromes, suggests research led by King's College London hospitals.
The researchers conducted a retrospective analysis of all patients with common variable immunodeficiency (CVID) and PAD with respiratory disease initially seen by immunology services between 2002 and 2017 in two tertiary care centres in London.
Bronchiectasis severity index (BSI) and FACED scores at presentation were obtained from 153 patients with CVID and PAD. All patients with CVID and 33 (79%) patients with specific antibody deficiency (SAD) were on immunoglobulin replacement. Sixteen patients with CVID also had granulomatous-lymphocytic interstitial lung disease.
The results, presented in Thorax, show mean FACED and BSI scores were 3.1 and 7.8, respectively, for CVID and 3.5 and 9.9, respectively, for SAD. Proportions of patients categorised as severe bronchiectasis (FACED >4 or BSI >8) were 16 per cent (FACED) and 37 per cent (BSI) for CVID and 26 (FACED) and 62 (BSI) per cent for SAD.
BSI scores were significantly higher in those who subsequently died (HR, 1.230; 95% CI, 1.080-1.404; P=.0002). There was a similar pattern for FACED scores, however, this did not reach statistical significance.
Pseudomonas aeruginosa in sputum cultures was associated with significant reduction in forced expiratory volume in the first second (FEV1) at presentation. Lower FEV1 at the time of diagnosis was associated with shorter survival (HR, 0.070; 95% CI, 0.007-0.666; P=.021). P. aeruginosa was also associated with shorter survival than the presence of Staphylococcus aureus (HR, 8.06; 95% CI, 1.55-41.79; P=.021), however, not when compared with isolation of bacteria or no growth.
The authors say the findings suggest bronchiectasis is a major driver of long-term mortality in patients with PAD on immunoglobulin replacement therapy.
