Dataset Information

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

ABSTRACT:

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.

SUBMITTER: Park HJ

PROVIDER: S-EPMC6567497 | biostudies-literature | 2019 Jun

REPOSITORIES: biostudies-literature

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Publications

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

Park Hee Jin HJ Park Jun Yong JY Jung Seung Min SM Song Jason Jungsik JJ Park Yong-Beom YB Lee Sang-Won SW

BMC gastroenterology 20190613 1

<h4>Background</h4>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).<h4>Methods</h4>We retrospectively reviewed the medical records of 132 MPA and GPA patients without chronic liver diseases. Conventional risk factors included old age (≥ 65 ye ...[more]

PMID: 31196158

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Project description:BackgroundUtilizing the traditional centers of excellence approach to conduct clinical trials involving rare diseases remains challenging. Patient-based registries have been shown to be both feasible and valid in several other diseases.ObjectiveThis report outlines the clinical characteristics of a large internet registry cohort of participants with a self-reported diagnosis of granulomatosis with polyangiitis or microscopic polyangiitis.MethodsPatients with a self-reported diagnosis of granulomatosis with polyangiitis or microscopic polyangiitis in an internet-based prospective longitudinal cohort (from the Vasculitis Patient-Powered Research Network) were included. Data on symptoms, diagnostic testing, and treatment were collected using standardized questionnaires.ResultsThe study compared patients with granulomatosis with polyangiitis (n=762) and patients with microscopic polyangiitis (n=164). Of the cohort, 97.7% (904/925) reported the diagnosis had been confirmed by a physician. Compared to microscopic polyangiitis, patients with granulomatosis with polyangiitis reported significantly more ear, nose, and throat manifestations (granulomatosis with polyangiitis: 641/723, 88.7%; microscopic polyangiitis: 89/164, 54.3%; z=10.42, P<.001), fevers (granulomatosis with polyangiitis: 325/588, 55.3%; microscopic polyangiitis: 64/139, 46.0%; z=1.96, P=.05), joint involvement (granulomatosis with polyangiitis: 549/688, 79.8%; microscopic polyangiitis: 106/154, 68.8%; z=2.96, P=.003), and pulmonary involvement (granulomatosis with polyangiitis: 523/734, 71.3%; microscopic polyangiitis: 90/154, 58.4%; z=3.13, P=.002). Compared to microscopic polyangiitis, patients with granulomatosis with polyangiitis reported significantly less renal involvement (granulomatosis with polyangiitis: 457/743, 61.5%; microscopic polyangiitis: 135/163, 82.8%; z=-5.18, P<.001) and renal transplantation (granulomatosis with polyangiitis: 10/721, 1.4%; microscopic polyangiitis: 7/164, 4.3%; z=-2.43, P=.02). Antineutrophil cytoplasmic antibody positivity was reported in 94.2% (652/692) of patients with granulomatosis with polyangiitis and 96.1% (147/153) of patients with microscopic polyangiitis. A biopsy showing vasculitis was reported in 77.0% (562/730) of patients with granulomatosis with polyangiitis and 81.9% (131/160) of patients with microscopic polyangiitis.ConclusionsIn this large, internet-based cohort of patients with a self-reported diagnosis of granulomatosis with polyangiitis or microscopic polyangiitis, disease manifestations were consistent with expectations for each type of vasculitis. Given the rarity of these and other vasculitides, conducting some types of research through internet-based registries may provide an efficient alternative to inperson, center-of-excellence clinical trials.

Dataset Information

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

Background

Methods

Results

Conclusions

Publications

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

Similar Datasets

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