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Acute Pulmonary Exacerbation Phenotypes in Patients with Cystic Fibrosis
Suzanne C Carter1,2, Alessandro N Franciosi3, Kate M O'Shea2
1National Referral Centre for Adult Cystic Fibrosis, St. Vincent's University Hospital, Dublin, Ireland.
Annals of the American Thoracic Society
|June 17, 2022
Summary
Cystic Fibrosis (CF) pulmonary exacerbations (PEx) can be classified into distinct subphenotypes based on viral status and C-reactive protein (CRP) levels. These CF PEx subphenotypes exhibit significant differences in clinical presentation, biomarker profiles, and patient outcomes.
Area of Science:
- Pulmonary Medicine
- Infectious Diseases
- Biomarkers
Background:
- Cystic Fibrosis (CF) pulmonary exacerbations (PEx) are complex, influenced by viral, bacterial, and non-infectious factors.
- Identifying distinct PEx subphenotypes is crucial for understanding disease heterogeneity and tailoring treatment.
- Viral infection and systemic inflammation, indicated by C-reactive protein (CRP), are key contributors to PEx.
Purpose of the Study:
- To investigate if viral infection status and CRP levels can classify CF PEx into distinct subphenotypes.
- To determine if these subphenotypes differ in clinical presentation, biomarker profiles, and patient outcomes.
- To validate these subphenotypes across different patient cohorts.
Main Methods:
- Patients admitted for PEx were recruited.
- Nasal swabs and sputum samples were analyzed for viral pathogens using multiplex PCR.
- Serum CRP levels were measured, and PEx were classified as 'pauci-inflammatory' (CRP < 5 mg/L), 'non-viral with systemic inflammation' (CRP ≥ 5 mg/L, no virus), or 'viral with systemic inflammation' (CRP ≥ 5 mg/L, virus detected).
Main Results:
- Subphenotype frequencies were: pauci-inflammatory (37%), non-viral with systemic inflammation (41%), and viral with systemic inflammation (22%).
- Significant differences in immunoglobulin levels, inflammatory cytokines, and serum calprotectin were observed across subphenotypes.
- The 'viral with systemic inflammation' group showed a greater reduction in lung function (FEV1pp) at exacerbation onset compared to the 'non-viral with systemic inflammation' group.
Conclusions:
- Distinct subphenotypes of CF PEx exist, characterized by differences in viral status and systemic inflammation.
- These subphenotypes are associated with variations in biomarker profiles, clinical presentation, and patient outcomes.
- Classification based on viral detection and CRP levels provides a valuable framework for understanding CF PEx heterogeneity.
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