Abstract
PurposeData for ANCA-associated vasculitis (AAV) patients requiring intensive care are scarce.MethodsWe included 97 consecutive patients with acute AAV manifestations (new onset or relapsing disease), admitted to 18 intensive care units (ICUs) over a 10-year period (2002–2012). A group of 95 consecutive AAV patients with new onset or relapsing disease, admitted to two nephrology departments with acute vasculitis manifestations, constituted the control group.Results In the ICU group, patients predominantly showed granulomatosis with polyangiitis and proteinase-3 ANCAs. Compared with the non-ICU group, the ICU group showed comparable Birmingham vasculitis activity score and a higher frequency of heart, central nervous system and lungs involvements. Respiratory assistance, renal replacement therapy and vasopressors were required in 68.0, 56.7 and 26.8% of ICU patients, respectively. All but one patient (99%) received glucocorticoids, 85.6% received cyclophosphamide, and 49.5% had plasma exchanges as remission induction regimens. Fifteen (15.5%) patients died during the ICU stay. The following were significantly associated with ICU mortality in the univariate analysis: the need for respiratory assistance, the use of vasopressors, the occurrence of at least one infection event in ICU, cyclophosphamide treatment, sequential organ failure assessment at admission and simplified acute physiology score II. After adjustment on sequential organ failure assessment or infection, cyclophosphamide was no longer a risk factor for mortality. Despite a higher initial mortality rate of ICU patients within the first hospital stay (p < 0.0001), the long-term mortality of hospital survivors did not differ between ICU and non-ICU groups (18.6 and 20.4%, respectively, p = 0.36). Moreover, we observed no renal survival difference between groups after a 1-year follow-up (82.1 and 80.5%, p = 0.94).ConclusionThis study supports the idea that experiencing an ICU challenge does not impact the long-term prognosis of AAV patients.
Highlights
Anti-neutrophil cytoplasmic antibodies (ANCAs)-associated vasculitis (AAV) are life-threatening multisystem autoimmune diseases characterized by necrotizing inflammation of small- to medium-sized vessels [1, 2]
Baseline characteristics of the intensive care unit (ICU) and non‐ICU‐AAV populations Ninety-seven and ninety-five AAV patients with a median follow-up of 2.28 [IQR 0.2–4.7] and 4.18 [IQR 1.7–7.0] years were included in the ICU group and the non-ICU control group, respectively
A notably high rate of alveolar hemorrhage was observed in ICU patients (64 vs. 10% in non-ICU patients), but the renal involvement rate was comparable
Summary
Anti-neutrophil cytoplasmic antibodies (ANCAs)-associated vasculitis (AAV) are life-threatening multisystem autoimmune diseases characterized by necrotizing inflammation of small- to medium-sized vessels [1, 2]. There are three differentiated entities based on clinical and pathological criteria: microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic granulomatosis with polyangiitis (EGPA) [3]. Even though ANCA negativity does not exclude AAV diagnosis, diffuse forms of AAV are usually associated with serum positivity for ANCAs [1, 7] Given their high level of specificity, ANCA detection is critical for AAV diagnosis, and ANCA positivity with a compatible clinical diagnosis usually allows the initiation of immunosuppressive treatments [8, 9]
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