Abstract

BackgroundThe fibrosis-4 index (FIB-4) has been reported to be associated with all-cause mortality in several chronic diseases. In this study, we investigated whether at diagnosis could be associated with all-cause mortality in patients with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA).MethodsWe retrospectively reviewed the medical records of 132 MPA and GPA patients without chronic liver diseases. Conventional risk factors included old age (≥ 65 years), male gender, diabetes mellitus (DM) and hypertension (HTN) at diagnosis, and disease-related risk factor included GPA, antineutrophil cytoplasmic antibody, Birmingham vasculitis activity score (BVAS) and five factor score (FFS (2009)). The cut-off of FIB-4 for significant liver fibrosis (S2–4) was set at 1.45.ResultsThe mean age was 57.2 years and 27 patients (20.5%) had significant liver fibrosis (FIB-4 ≥ 1.45). Fifteen patients (11.4%) died during follow-up. In the univariable Cox Hazards model, age ≥ 65 years (Hazard ratio (HR) 5.055), DM (HR 3.446), HTN (HR 4.611), FFS (2009) ≥ 2 (HR 4.849) and FIB-4 ≥ 1.45 (HR 9.958) at diagnosis were significantly associated with all-cause mortality. In the multivariable Cox Hazards model, only FIB-4 at diagnosis ≥1.45 (HR 6.253, 95% confidence interval 1.398, 27.963) was associated with all-cause mortality during the follow-up in patients with MPA and GPA.ConclusionsFIB-4 at diagnosis ≥1.45 is an independent predictor of all-cause mortality during follow-up in patients with MPA and GPA, and furthermore its predictive potential is higher than those of conventional and AAV-related risk factors for all-cause mortality.

Highlights

  • The fibrosis-4 index (FIB-4) has been reported to be associated with all-cause mortality in several chronic diseases

  • Patients We retrospectively reviewed the medical records of 138 immunosuppressive drug-naïve patients with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) based on the following inclusion criteria: i) patients who had been first classified as associated vasculitis (AAV) at the Department of Internal Medicine, Yonsei University College of Medicine, Severance Hospital, from October 2000 to December 2017; ii) patients who fulfilled the American

  • Because Birmingham vasculitis activity score (BVAS) for GPA has a different weightsystem compared to BVAS, we evenly applied BVAS to GPA to unify the scoring system; iv) patients who had results on perinuclear (P)-Antineutrophil cytoplasmic antibody (ANCA) and cytoplasmic (C)ANCA or myeloperoxidase (MPO)-ANCA and proteinase 3 (PR3)-ANCA levels at diagnosis. v) patients who had been followed up for 12 weeks or greater; vi) patients who had no medical history to affect either BVAS, ANCA positivity or FIB-4 prior to or at diagnosis, chronic liver diseases including viral hepatitis, coexisting malignancies, serious comorbidities and serious infection, which were identified in the 10th revised International Classification Diseases; vii) patients who had never received immunosuppressive drugs for AAV prior to diagnosis, which were searched by the Korean Drug Utilisation Review system

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Summary

Introduction

The fibrosis-4 index (FIB-4) has been reported to be associated with all-cause mortality in several chronic diseases. We investigated whether at diagnosis could be associated with all-cause mortality in patients with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA). Park et al BMC Gastroenterology (2019) 19:90 mortality in patients with AAV from different ethnic and geographic backgrounds: one year-cumulative patient survival rates were from 82 to 95% in Western countries [4], and that was reported to be 79.1% in Japan [5]. Infectious or immunosuppressive drug-related causes are major aetiologies of death in AAV patients, identifying predictors of all-cause mortality at diagnosis in immunosuppressive drug-naïve patients may have clinical implications in the real settings. In this study, we investigated whether FIB-4 at diagnosis could be associated with all-cause mortality in 132 immunosuppressive drug-naïve patients with MPA and GPA

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