Severity assessment of non-cystic fibrosis bronchiectasis by the FACED score

INTRODUCTION

Bronchiectasis (BE) is a major health problem associated with a high morbidity and mortality.^[1,2] It is a chronic respiratory disease. characterized by abnormal and irreversible dilatation of the airways and symptoms such as chronic cough, purulent sputum production, hemoptysis, dyspnea and other distressing symptoms.^[1] It is related to a vicious cycle of compromised host defenses, inflammation, impaired mucociliary clearance, chronic colonization with bacteria, and recurrent infection leading to destruction and remodeling of the bronchial wall.^[2] Radiologically, there is abnormal and permanent dilatation of the bronchi.^[2]

In recent years, BE has become a major health concern for several reasons. In fact, the clinical course of the disease is characterized by the occurrence of acute exacerbations (AE) which progressively worsens dyspnea, lung function and deterioration in quality of life.^[2,3] Thus, BE is associated with repeated hospitalizations and high morbidity and mortality, causing a significant economic burden.^[4]

BE is classified as cystic fibrosis related BE and noncystic fibrosis bronchiectasis (NCFB).^[5] NCFB affects a heterogeneous population of patients with diverse etiologies. It is a multidimensional disease. It may result from various hereditary or acquired, local, or systemic diseases.^[6]

Despite the limited number of effective treatment approaches and evidence-based management guidelines, it is necessary to evaluate disease severity meticulously to ensure better therapeutic approach.^[7]

Several single variables such as the decline in lung function, different clinical parameters, radiological findings, and the presence of chronic colonization have been used to assess the severity of NCFB and to predict key outcomes of the disease.^[8] However, the severity and prognosis of NCFB cannot be adequately assessed through a single variable analysis.^[9]

Accordingly, few multidimensional scales have been published in recent years, using several different variables that are easily obtained and have a proven capacity for an accurate appreciation of the severity and the prognosis of the disease.^[10,11] In this regard, two multidimensional validated scores are currently used to assess the severity of NCFB: The FACED score and the Bronchiectasis Severity Index (BSI).^[10,11]

Martinez-Garcia et al. recently developed the FACED score, which comprises five variables: Functional (percentage of predicted forced expiratory volume in 1 s [FEV1% predicted]), physiological (age), microbiological (chronic colonization by Pseudomonas aeruginosa), radiological (number of lobes affected), and clinical parameter (degree of dyspnea, evaluated by the Medical Research Council [MRC] scale).^[10] The FACED score is a scale contributing to stratify patients into risk categories.

This scale has been validated in several countries.^[12,13] Nonetheless, there is little information regarding NCFB in African countries. Furthermore, to the best of our knowledge, there are no published data related to the study of the FACED score in the latter countries.

The aim of this study is to determine the relation of the FACED score with the severity of NCFB among our North-African based population.

MATERIALS AND METHODS

RESULTS

Baseline characteristics of patients with NCFB

A total of 105 patients fulfilled the eligibility criteria and were included in the study. The baseline characteristics of the patients included are described in [Table 2]. The mean age of the total sample was 56 ± 18 years, with 53% males, and 7% current or former smokers. The mean FEV1 was 1.44 ± 1 (51 ± 24% of predicted). The mean number of BE AEs per year was 1.6 ± 1.3. The mean FACED score was 2.7 ± 2. Patients were followed for a mean period of 56 months. Forty-two patients (40%) were treated by long-acting beta-2 agonist. No patient had a long-term antibiotic treatment.

Table 2: Baseline characteristics of NCFB patients.

	Number/mean	Frequency (%)
N patients	105
Age	56±18
Gender (male)	55	53
Smoking (current/former)	8	7
Pack years	39±26
N of attained lobes	3.3±1.7
N of comorbidities ≥1	67	64
mMRC ≥2	75	71
FEV1(L)	1.44±1
FEV1(%)	51±24
FVC (L)	1.94±1
FVC (%)	57±21
FEV1/FVC (%)	71±15
FEV1<50%	64	61
PaO2 (mmHg)	76±17
PaCO2 (mmHg)	40±6
N acute exacerbations/year	1.6±1.3
Frequent exacerbators (N AE≥2/y)	37	35
N H pulmonary department/year	0.8±0.5
H in ICU	14	13
Chronic respiratory failure	36	34
Home Oxygen	22	21
Follow-up (month)	56

NCFB: Non cystic fibrosis bronchiectasis, N: Number, mMRC: Modified Medical Research Council, FEV1: Forced expiratory volume in one second, FVC: Forced vital capacity, PaO2: Oxygen arterial tension, PaCO2: Carbon dioxide arterial tension, AE: Acute exacerbation, y: Year, H: Hospitalization, ICU: Intensive care unit, L: Liter

According to the FACED score, 44% of the patients had mild BE (46 cases): Group 1; 33% moderate BE (35 cases) and 23% severe BE (24 cases): Group 2 [Figure 1].

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Figure 1:

Patients grouping and risk stratification according to the FACED score.

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Concerning the etiology of NCFB, data analysis revealed that 58 patients (55%) had idiopathic NCFB. The most common cause being post-infectious (non-tuberculosis) and accounting for 22 (21%). Different other causes were identified: Post-tuberculosis (13 patients – 12%), primary ciliary dyskinesia (5 patients – 5%), primary immunodeficiencies (4 patients – 4%), and foreign body airway obstruction (3 patients – 3%).

During the period of follow-up, five patients from the G2 died, whereas we did not note any death in G1.

We compared different severity parameters of BE concerning the baseline status and AE between the two groups of patients: G1: FACED score ≤2 (46 patients, 44%) and G2: FACED Score ≥ 3 (59 patients, 56%) [Figure 1].

DISCUSSION

NCFB is associated with a high morbidity and mortality.^[1,2] Stratifying patients into severity classes is a major concern related to the disease to ensure the best care. In line with this target, the FACED score was conceived.^[10] This study aimed to determine the relation of the FACED score with the severity of NCFB among our North-African based patients. The results suggest that a high FACED score is associated with more symptoms, an altered respiratory function, a higher number and more severe AE, and more health-care utilization with worse outcomes.

Some variables were used to assess and predict NCFB outcomes. In fact, FEV1 has been used to define the severity of BE in different reports.^[3,10,19] The decline in lung function also correlates with clinical parameters, the risk of AE occurrence, and the presence of chronic colonization by P. aeruginosa.^[3,8] Furthermore, CT findings provide important information about structural abnormalities in airway diseases and the extent of the lesions.^[20] CT scores, such as the modified Reiff et al. score and Bhalla et al. score, have also been proposed as indicators of disease severity.^[21,22] Other variables, such as age, airway chronic colonization, and quality of life, have proved usefulness in this respect by showing a correlation with mortality in BE.^[8] However, these separate parameters did not correlate adequately with the severity of NCFB.^[7-9] Indeed, just one of these factors on its own is certainly inadequate for predicting outcomes or helping in clinical decision-making.^[7-9]A scoring system including different variables was then essential to a better assessment of BE severity. There are mainly two scales that can be used to assess the severity and the prognosis of the NCFB: The FACED and BSI scores. The FACED score is a multidimensional scale. It was conceived and validated for the 1^st time by Martinez-Garcia et al.^[10] The latter conducted an observational multicenter study including 819 patients diagnosed with NCFB using HRCT. The outcome was 5-year all-cause mortality after radiological diagnosis. Different parameters were studied to establish the score. The final seven-point score incorporated five variables. It stratifies patients into three severity risk categories to predict the likelihood of mortality.

The FACED score is an easy-to-use multidimensional tool that has demonstrated an excellent prognostic value for mortality in patients with BE.^[10] One of the important advantages of the FACED score is its simplicity. The constituent variables of the score are very easy to memorize and the total score is easy to obtain, calculate, and interpret. In our research, it was not possible to study the prognosis value of the FACED score due to the low number of death in our population.

This score was conceptualized basically to predict the likelihood of 5-year mortality of NCFB of any etiology.^[9] However, it was used to assess other severity parameters of BE in other studies. In fact, the FACED score was studied and validated in different populations.^[9,12,13] In this context, a Latin America validation of the FACED score was performed. Athanazio et al. conducted a retrospective and multicenter study including 651 patients with BE. Even if the clinical features and etiologies were different from the original cohort of Martinez-Garcia et al., the FACED score maintained an excellent power to predict all-cause and respiratory mortality. The score maintained also an excellent discriminatory power by identifying a profile of increased severity in patients with BE that was similar to that seen in the original Spanish study. Furthermore, the authors demonstrated that the score has a good capacity to predict AE and hospitalizations.^[12] These results are in agreement with our findings. In a recent study, an increasing disease severity as measured by FACED score was correlated with a lower quality of life.^[5]

The FACED score was also compared to the BSI in different populations. The BSI was introduced by Chalmers et al.^[11] This score included HRCT score, FEV1, MRC dyspnea score, bacterial colonization (P. aeruginosa or other pathogenic bacteria), prior hospital admission, and exacerbations. They found that the BSI was a sensitive tool in predicting future risks of hospitalization and mortality.^[11] McDonnell et al. evaluated both the FACED and BSI scores in a large population of 1612 patients including seven European cohorts. Both scores had a good discriminatory predictive value for mortality.^[13] However, the BSI was superior to FACED score in predicting multiple clinically useful outcomes including respiratory symptoms, AE, hospital admissions, quality of life, exercise capacity, and lung function decline.^[13] In other studies, the comparison between the two scores revealed that both scoring systems had similar predictive power for 5-year mortality with high specificity with a minor proportion of patients having discordant BSI and FACED scores.^[16,23] Whilst both scores were able to predict 5-year mortality, the FACED score was superior.^[16] Other conflicting results were found in a study including Aboriginal Australians. The latter were significantly younger and died at a significantly younger age than other groups. A milder assessed disease by FACED scores did not imply a better prognosis.^[24] Hence, extrapolating prognostic scores based on specific cohorts to other populations must be done with caution.^[24]

Patients with BE have frequent exacerbations that cause significant morbidity, mortality, and health-care costs.^[14] AE of BE is associated with increased airways and systemic inflammation,^[25] progressive lung damage,^[2,26] poor quality of life, daily symptoms,^[27] lung function decline,^[28] and mortality.^[11] The European registry data showed that approximately 50% of European BE patients have at least 2 AE per year and one-third require at least one hospitalization per year.^[29] Although the FACED score has demonstrated a great prognostic capacity in BE and predicts future events like AE, it does not include the number or severity of exacerbations as a separate variable.^[30] The E-FACED is a new scale including the number of severe exacerbations in 1 year. It was recently constructed and validated by Martinez-Garcia et al. to increase its capacity to predict future events (AE) and its prognostic value. The E-FACED score significantly increased the FACED capacity to predict future events (AE) while maintaining the score’s simplicity and prognostic capacity.^[30]

The economic burden of BE has been assessed by some studies. The costs are related to treatments, emergency visits, hospitalizations, and need for intensive care.^[2,4,31] They increase significantly with the severity. A higher FACED score was associated with increased costs.^[31]

To the best of our knowledge, this is the first study that has investigated the relation of the FACED score with the severity of NCFB in a North-African population. Among the strengths of the present study, is the availability of data to study several parameters related to the severity of the disease. Furthermore, the results represent the real-life situation in our hospital. Moreover, our NCFB population was not treated with long-term (≥3 months) anti-inflammatory or antibiotic medications and provides some insight on the natural history of the disease..

Nevertheless, the current study is not without limitations. First, given the retrospective character of this research, not all variables could be collected for every patient. For example, some AE may not be documented in the medical records leading to an under-appreciation of the real number of these events. On another hand, some investigations were not available for all patients and the impact of such missing data on selection bias should be considered. Second, it is a single-center study with a relatively small number of patients, which could have certain implications on the their interpretation. Furthermore, the mean period of follow-up was 56 months and they ere few death which precluded the predictive ability of the FACED score regarding mortality. In addition, the majority of the patients had either idiopathic or post-infective NCFB. Thus, the results may not be generalized to other NCFB populations having different etiology proportions. Finally, we considered HRCT reports in medical records to define cases of BE. The accurate assessment of BE may differ from one reporting radiologist to another.

CONCLUSION

Our results suggest that a high FACED score is associated with more symptoms, an altered respiratory function, a higher number and more severe AE, and more health-care utilization with worse outcomes. This scale provided a clinically relevant evaluation of disease severity. However, NCFB is characterized by its heterogeneity in terms of etiologies, clinical presentations, radiological findings, biological parameters, and bacterial features. The incorporation of other elements in the scoring system may be required. Further studies are needed to determine how such scales may have an impact on clinical practice, phenotyping process, and treatments choice in the scope of personalized medical care.