C-Reactive Protein in Stable Cystic Fibrosis: An Additional Indicator of Clinical Disease Activity and Risk of Future Pulmonary Exacerbations

Intravenous; Prior-year PExs: IV-treated Pulmonary Exacerbations 1-year Prior to Baseline; PExs 1-year post-baseline: IV-treated Pulmonary Exacerbations 1-year Post Baseline; CFRD: Cystic Fibrosis Related Diabetes; ABPA: Allergic Bronchopulmonary Aspergillosis; CFTR: Cystic Fibrosis Transmembrane Regulator Protein; ∆F508: F508del-CFTR.Wojewodka et al. [7] studied a cohort of 52 adult patients with CF.They demonstrated that stable patients who developed PExs over a 1-year follow-up had worse baseline disease severity (based on spirometry), worse baseline Clinical and Complications subscores Abstract Introduction: In stable adult cystic fibrosis (CF) patients, we assessed the role of baseline high sensitivity C-reactive protein (hs-CRP) on CF clinical variables and frequency of intravenous (IV) treated pulmonary exacerbations (PExs) 1-year post-baseline.Methods: We recruited 51 clinically stable CF patients from our Adult CF Center.We incorporated collected parameters into Matouk CF clinical score and CF questionnaire-revised quality of life score (QOL).We used the clinical minus complications subscores as a clinical disease activity score (CDAS).We dichotomized our patients according to the cohort median baseline hs-CRP of 5.2 mg/L.Results: Patients in the high hs-CRP group (≥ 5.2 mg/L) demonstrated worse CDAS (r=0.67,p=0.0001) and QOL scores (r=0.57,p=0.0017) at a given FEV 1 % predicted.In both hs-CRP groups, prior-year IV-treated PExs and baseline CDASs were significant predictors of future IV-treated PExs.Interestingly, the association between baseline CDAS and future PExs frequency was more robust in the high compared to the low hs-CRP group (r=-0.88,p<0.0001, r=-0.48,p=0.017, respectively) with a steeper regression slope (p=0.001).In addition, a significant interaction was demonstrated between elevated baseline hs-CRP levels and CDASs for the prediction of increased risk of future PExs (p=0.02).This interaction provided an additional indicator of clinical disease activity and added another dimension to the prior year PExs frequency phenotype to identify patients at increased risk for future PExs.Conclusion: Stable CF patients with elevated baseline hs-CRP (≥ 5.2 mg/L) demonstrated worse clinical disease activity and QOL scores at a given level of disease severity (FEV 1 % predicted).Elevated baseline hs-CRP values combined with clinical disease activity scores are associated with increased risk for future IV-treated PExs even in those with mild clinical disease activity scores.

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