Introduction CPA is a chronic progressive respiratory infectious disease results in significant lung tissue destruction with a 50%+ 5 year mortality. Response to antifungal therapy is slow, with ∼80% of patients who respond doing so by 6 months. We recently demonstrated the reliability and validity of SGRQ in examining HS in CPA (Chest, in press), and now present longitudinal data on the efficacy of LTAFT in improving HS in CPA patients.
Method HS of 98 CPA patients were assessed 3 times over 6 months using the well-established standardised SGRQ. CPA severity was assessed using our published CPA banding system. FEV1, BMI, dyspnoea (using MRC dyspnoea scale) were measured.
Results Mean age was 58 years and 48% were female; and 25, 58 and 15 had band 1, 2 and 3 CPA respectively. At visit 2 and 3 (V2 and V3), we found that overall total and domain SGRQ scores were either lower (improved health status) or similar compared to V1 (table 1).
Categorizing the cohort by those who reported improvement or deterioration by a total SGRQ score of ≥4 at V3 comparing to V1, we found that 43% improved, 22% remained stable and 35% deteriorated. The median (IQR) of total SGRQ score of the improved group at V3 was 58 (42–66) compared to 71 (60–79) at V1; and for the deteriorated group was 67.5 (57–76) at V3 compared to 62 (41–67)) at V1. The deteriorated were older (62 (9.8) years versus 56.1 (9.3) (p=0.008); and tended to have lower BMI, more dyspnoea and worse lung function. Moreover, binary regression multivariate analysis showed that age maintained its association with deterioration in HS (OR 1.13, 95% CI 1.01–1.26, p=0.03) after correcting for gender, BMI and FEV1%.
Of the 37 patients started on an antifungal agent at V1 who took it for 3+ months (including a 3 week IV course of amphotericin B), 22 (59%) improved, 11 (30%) were stable and 4 (11%) deteriorated at V3.
Conclusion LTAFT prevented/reduced the progression of CPA and patients preserved overall good HS. More therapeutic approaches for this progressive disease are urgently needed.