Fibrosis-4 index at diagnosis is associated with all-cause mortality in patients with microscopic polyangiitis and granulomatosis with polyangiitis

BACKGROUND: The 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). METHODS: We 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. RESULTS: The 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. CONCLUSIONS: FIB-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. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (10.1186/s12876-019-1007-z) contains supplementary material, which is available to authorized users.