Abstract
BackgroundScleroderma interstitial lung disease (SSc-ILD) has a variable and poorly predictable course. Data on independent role of bronchoalveolar lavage fluid (BALF) analysis in prognostic stratification of SSc-ILD patients are conflicting. We wanted to retrospectively evaluate the role of BALF analysis to predict severe course of disease in our cohort of SSc-ILD.ObjectivesWe wanted to retrospectively evaluate the role of BALF analysis to predict severe course of disease in our cohort of SSc-ILD.MethodsForty SSc-ILD patients naive to immunosuppressants underwent comprehensive clinical evaluation, bronchoalveolar fluid analysis with total and differential leukocyte count, pulmonary function tests (PFTs), high-resolution computed tomography (HRCT). All the patients had fibrosing lung disease affecting more than 10% of lung volume on HRCT. Baseline alveolar (AS) and interstitial score (IS) on HRCT were computed. The 15-year crude mortality rate was retrospectively assessed.ResultsThe enrolled patients (pts) (male 20.0%, aged 53.9±13.0 years) had a disease duration of 4.3±3.4 years. Diffuse LeRoy variant was reported in 47.5% of patients and anti-Scl70 and anti-centromere antibodies were detected in 57.5% and 12.5% of pts, respectively. The average AS and IS were 6.5±4.8 and 6.2±2.7, respectively, while 25.0% had FVC≤80% and 35.0% had DLco≤50%. During follow-up 18 pts received immunosuppressants. BALF neutrophilia (≥3.0%), eosinophilia (≥1.0%) and lymphocytosis (≥15.0%) were reported in 40.0%, 16.0% and 5.0% of patients, respectively. Twenty-five pts (62.5%) died within 15 years after bronchoscopy with a mean survival of 12.7 years (95% IC 11.5-13.9) overall. Fifteen-year mortality was predicted by BALF alveolitis (HR 2.87, 95% IC 1.26-6.56), and neutrophilia (HR 3.74, 95% IC 1.64-8.55), log-transformed absolute count of total cells (HR 2.52, 95% IC 1.12-5.58), neutrophils (HR 1.80, 95% IC 1.28-2.54), and eosinophils (HR 1.31, 95% IC 1.05-1.62), and percentages of neutrophils (HR 1.8, 95% IC 1.26-2.73) and eosinophils (HR 1.32, 95% IC 1.01-1.73). Only absolute and relative neutrophil counts were independently associated with mortality also in all models adjusted for demographics (age, gender), main disease traits (diffuse LeRoy variant, anti-Scl70 positivity), baseline pulmonary function tests (FVC, DLco), baseline HRCT involvement (AS, IS) and presence of vascular involvement (pulmonary hypertension, digital ulcers).ConclusionHigh BALF neutrophils were associated with high 15-year mortality independently from other established clinical risk factors. Given the negative prognosis of SSc-ILD, its poorly predictable clinical course, and the availability of new promising biomarkers, BALF analysis may be considered in assessing these pts to improve prognosis prediction.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of InterestsNone Declared.
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