Abstract

In patients presenting with anti-glomerular basement membrane (GBM) disease with advanced isolated kidney involvement, the benefit of intensive therapy remains controversial due to adverse events, particularly infection. We aim to describe the burden of severe infections (SI) (requiring hospitalization or intravenous antibiotics) and identify predictive factors of SI in a large cohort of patients with anti-GBM disease. Among the 201 patients (median [IQR] age, 53 [30–71] years) included, 74 had pulmonary involvement and 127 isolated glomerulonephritis. A total of 161 SI occurred in 116 patients during the first year after diagnosis. These infections occurred during the early stage of care (median [IQR] time, 13 [8–19] days after diagnosis) with mainly pulmonary (45%), catheter-associated bacteremia (22%) and urinary tract (21%) infections. In multivariable analysis, positive ANCA (HR [95% CI] 1.62 [1.07−2.44]; p = 0.02) and age at diagnosis (HR [95% CI] 1.10 [1.00–1.21]; p = 0.047) remained independently associated with SI. Age-adjusted severe infection during the first three months was associated with an increased three-year mortality rate (HR [95% CI] 3.13 [1.24–7.88]; p = 0.01). Thus, SI is a common early complication in anti-GBM disease, particularly in the elderly and those with positive anti-neutrophil cytoplasmic antibodies (ANCA). No significant association was observed between immunosuppressive strategy and occurrence of SI.

Highlights

  • With an estimated annual incidence of 0.5–1 per million in the population, anti-glomerular basement membrane disease is a rare form of immune complex small vessel vasculitis [1]

  • The aim of this study is to describe the burden of severe infections, identify predictive factors, and evaluate the impact on survival in a large French cohort of patients presenting with anti-glomerular basement membrane (GBM) disease between 1997 and April 2017

  • Patients with pulmonary involvement were significantly younger compared to patients with isolated glomerulonephritis

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Summary

Introduction

With an estimated annual incidence of 0.5–1 per million in the population, anti-glomerular basement membrane disease (anti-GBM disease) is a rare form of immune complex small vessel vasculitis [1]. This autoimmune disease is mediated by autoantibodies directed against the non-collagenous domain of the α3 chain of type IV collagen [2,3]. For older patients in whom isolated glomerulonephritis is most common, the benefit of intensive treatment remains controversial in the case of anuria or dialysis dependence, regarding a hypothetical increased infectious risk. Data on severe infections are lacking in patients with anti-GBM disease

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