Outcomes of patients admitted to intensive care units for acute manifestation of small-vessel vasculitis: a multicenter, retrospective study
Kimmoun et al. Critical Care
Outcomes of patients admitted to intensive care units for acute manifestation of small-vessel vasculitis: a multicenter, retrospective study
Antoine Kimmoun 0 1 2
Elisabeth Baux 0 1 2
Vincent Das 12
Nicolas Terzi 18
Patrice Talec 17
Pierre Asfar 17
Stephan Ehrmann 16
Guillaume Geri 15
Steven Grange 20
Nadia Anguel 19
Alexandre Demoule 14
Anne Sophie Moreau 10
Elie Azoulay 11
Jean-Pierre Quenot 8
Julie Boisramé-Helms 9
Guillaume Louis 6
Romain Sonneville 7
Nicolas Girerd 4
Nicolas Ducrocq 1 2
Nelly Agrinier 5
Denis Wahl 3
Xavier Puéchal 13
Bruno Levy 1 2
0 Equal contributors
1 INSERM U1116, Vandoeuvre-les-Nancy , Nancy , France
2 Brabois Medical Intensive Care Unit, Nancy University Hospital, Vandoeuvre-les-Nancy , Nancy 54000 , France
3 Vascular Medicine Division and Regional Competence Center for Rare Vascular and Systemic Autoimmune Diseases, Nancy University Hospital, Vandoeuvre-les Nancy , Nancy 54511 , France
4 INSERM CIC1433, Nancy University Hospital , Nancy 54000 , France
5 INSERM CIC-EC, CIE6, Nancy University Hospital , Nancy 54000 , France
6 Medical Intensive Care Unit, Mercy Regional Hospital , Ars-Laquenexy 57530 , France
7 Medical Intensive Care Unit, Bichat - Claude-Bernard University Hospital , Paris 75018 , France
8 Medical Intensive Care Unit, Dijon University Hospital , Dijon F-21079 , France
9 Medical Intensive Care Unit, NHC University Hospital , Strasbourg F-67091 , France
10 Medical-Surgical Intensive Care Unit, Lille University Hospital , Lille F-59000 , France
11 Medical Intensive Care Unit, Saint-Louis University Hospital , Paris 75010 , France
12 Medical-Surgical Intensive Care Unit, Andre Gregoire District Hospital Center , Montreuil F-93105 , France
13 National Referral Center for Necrotizing Vasculitides and Systemic Sclerosis, Cochin Hospital, University Paris Descartes , Paris F-75014 , France
14 Medical Intensive Care Unit and Respiratory Division, Pitié-Salpêtrière University Hospital , Paris 75013 , France
15 Medical Intensive Care Unit, Cochin University Hospital , Paris F-75014 , France
16 Medical Intensive Care Unit, Bretonneau University Hospital , Tours F-37044 , France
17 Medical Intensive Care Unit, Angers University Hospital , Angers F-49933 , France
18 Medical Intensive Care Unit, Caen University Hospital , Avenue de la Côte de Nacre, 14000 Caen , France
19 Medical Intensive Care Unit, Kremlin-Bicêtre University Hospital , Paris F-94275 , France
20 Medical Intensive Care Unit, Rouen University Hospital , Rouen 76031 , France
Background: The outcomes of patients admitted to the intensive care unit (ICU) for acute manifestation of small-vessel vasculitis are poorly reported. The aim of the present study was to determine the mortality rate and prognostic factors of patients admitted to the ICU for acute small-vessel vasculitis. Methods: This retrospective, multicenter study was conducted from January 2001 to December 2014 in 20 ICUs in France. Patients were identified from computerized registers of each hospital using the International Classification of Diseases, Ninth Revision (ICD-9). Inclusion criteria were (1) known or highly suspected granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis (respectively, ICD-9 codes M31.3, M30.1, and M31.7), or anti-glomerular basement membrane antibody disease (ICD-9 codes N08.5X-005 or M31.0+); (2) admission to the ICU for the management of an acute manifestation of vasculitis; and (3) administration of a cyclophosphamide pulse in the ICU or within 48 h before admission to the ICU. The primary endpoint was assessment of mortality rate 90 days after admission to the ICU. Results: Eighty-two patients at 20 centers were included, 94 % of whom had a recent (<6 months) diagnosis of small-vessel vasculitis. Forty-four patients (54 %) had granulomatosis with polyangiitis. The main reasons for admission were respiratory failure (34 %) and pulmonary-renal syndrome (33 %). Mechanical ventilation was required in 51 % of patients, catecholamines in 31 %, and renal replacement therapy in 71 %. Overall mortality at 90 days was 18 % and the mortality in ICU was 16 %. The main causes of death in the ICU were disease flare in 69 % and infection in 31 %. In univariable analysis, relevant factors associated with death in nonsurvivors compared with survivors were Simplified Acute Physiology Score II (median [interquartile range] 51 [38-82] vs. 36 [27-42], p = 0.005), age (67 years [62-74] vs. 58 years [40-68], p < 0.003), Sequential Organ Failure Assessment score on the day of cyclophosphamide administration (11 [6-12] vs. 6 [3-7], p = 0.0004), and delayed administration of cyclophosphamide (5 days [3-14] vs. 2 days [1-5], p = 0.0053). (Continued on next page)
(Continued from previous page)
Conclusions: Patients admitted to the ICU for management of acute small-vessel vasculitis benefit from early,
aggressive intensive care treatment, associated with an 18 % death rate at 90 days.
The revised International Chapel Hill Consensus
Conference Nomenclature of Vasculitides [
vasculitis as a function of the size of the vessel involved.
According to this nomenclature, small-vessel vasculitides
(SVV) are a group of diseases that includes
antineutrophil cytoplasmic antibody–associated vasculitis (AAV)
and immune complex SVV. Epidemiological data on
SVV remain scarce, although they could be considered
as orphan diseases [
]. With the development of
therapeutic strategies that include corticosteroids,
immunosuppressants, and (plasma exchange [PLEX]), SVV
survival rates have considerably improved, from 30 to
75 % at 5 years [
]. The leading causes of death are
related mainly to a life-threatening disease at the time of
diagnosis or long-term complications of
immunosuppressive therapies, all of which may require intensive
care unit (ICU) admission [
]. To date, there are only
few studies, all retrospective, in which researchers have
reported the outcomes of patients with vasculitis
admitted to the ICU. The reported ICU mortality rates of the
three most recent studies ranged from 11 to 52 % [
AAV, and particularly granulomatosis with polyangiitis
(GPA; Wegener’s granulomatosis), was the most
frequent form of vasculitis. Unfortunately, all three were
single-center studies and heterogeneous in nature, as
they included several types of vasculitides with different
prognoses. Patients were admitted either in the initial
phase of the disease or after a long-term evolution.
Finally, therapeutic vasculitis management was poorly
described and mostly inhomogeneous between studies.
Nonspecific ICU scores at admission, such as the
Simplified Acute Physiology Score II (SAPS II) or Sequential
Organ Failure Assessment (SOFA) score, have been
reported to be associated with outcome; however, specific
vasculitis scores are not adapted to the ICU setting. In
light of these circumstances, we carried out a
retrospective, multicenter study to describe the clinical course,
outcomes, and prognostic factors of patients admitted to
the ICU for acute manifestation of new-onset SVV.
In this retrospective, observational, multicenter study, 22
ICUs in northern France were contacted individually by
e-mail on three occasions to analyze the outcomes of
patients admitted to the ICU for acute manifestations of
SVV. Two of the centers did not partake in the study. In
the 20 participating centers, patients were identified by
1. We used the computerized registers of each hospital
to identify patients with the International
Classification of Diseases, Ninth Revision (ICD-9),
codes M31.3 for GPA, M30.1 for eosinophilic GPA,
M31.7 for microscopic polyangiitis, and N08.5X-005
or M31.0+ for anti–glomerular basement membrane
(GBM) antibody disease.
2. If no patient was found in the computerized
database of the medical informatics department,
then the following keywords were searched in the
hospital report database of each ICU department:
“microscopic polyarteritis,” “granulomatosis with
polyangiitis (or Wegener’s),” “eosinophilic
granulomatosis with polyangiitis (or Churg-Strauss),”
“anti-glomerular basement membrane disease (or
All patients admitted to the ICU for SVV management
were screened. When a patient was hospitalized in the
ICU on more than one occasion, only the first ICU
admission was considered.
To be included, patients had to fulfill the following criteria:
1. Patients had to be admitted to the ICU for acute
manifestations of known or highly suspected SVV
(new diagnosis or relapse). On the basis of the
results of previously published studies, acute
manifestations of known or highly suspected SVV
requiring admission in ICU include respiratory
failure, acute renal failure, cardiac failure, coma due
to central nervous system involvement, and severe
gastrointestinal involvement (e.g., peritonitis due to
small intestine perforation) [
6, 9, 10
2. Patients had to receive cyclophosphamide pulse
therapy according to French recommendations
] within 48 h before admission or during their
3. Primary SVV patients were included if they
presented with a diagnosis of AAV: microscopic
polyarteritis, GPA (formerly known as Wegener’s
granulomatosis), and eosinophilic GPA (formerly
known as Churg–Strauss syndrome). Due to similar
clinical presentation and initial treatment in the ICU,
patients with anti–GBM antibody disease (an immune
complex vasculitis formerly known as Goodpasture
syndrome) were also included in this study.
Considering their heterogeneous clinical presentation
and management, other immune complex SVV
(cryoglobulinemic vasculitis, immunoglobulin A vasculitis,
hypocomplementemic urticarial vasculitis [anti-C1q vasculitis])
were excluded from this analysis. Details on SVV not
included in the present study are provided in Additional
file 1. Patients admitted for an infectious complication
secondary to SVV immunosuppressive treatments were
excluded from the study.
Each clinical record, in either paper or electronic form,
was reviewed at each site by the principal investigator.
All scores were calculated by the same principal
investigator to ensure interscore reliability. At ICU admission,
the following data were collected for each patient:
demographic data; reason for admission; medical history; SVV
diagnosis type; and disease assessment scores, including
SAPS II score, SOFA score, Birmingham Vasculitis
Activity Score (BVAS) (version 3), and revised Five-Factor
SAPS II and SOFA scores were used to assess disease
severity. The SAPS II score is calculated using the worst
12 physiological variables during the first 24 h in the
ICU and also includes three disease-related variables
]. The SOFA score is based on six physiological
variables and can be calculated on a daily basis [
Vasculitis disease activity was assessed on the basis of
the BVAS [
]. This score is based on clinical and
biological items in nine separate organ systems: general;
cutaneous; mucous membrane and eyes; ear, nose, and
throat; cardiovascular; gastrointestinal; pulmonary; renal;
and nervous system. The revised FFS was calculated at
admission for patients with microscopic polyangiitis,
GPA, eosinophilic granulomatosis with polyangiitis, and
anti–GBM antibody disease. This score is used to assess
prognosis at the time of diagnosis and includes the
following items: serum creatinine level (>150 μmol or
<150 μmol); presence of severe gastrointestinal tract
involvement; cardiomyopathy; age; and ear, nose, and
throat involvement [
The primary endpoint was assessment of mortality rate
90 days after ICU admission. Outcome was also
recorded (survivors and nonsurvivors) in the ICU and at
day 90. For each patient, three specific adverse events
reflecting global consequences of immunosuppression
were recorded during the ICU stay: sepsis, hemorrhagic
syndrome, and hematological disorders such as aplasia
and thrombopenia. The incidence of these adverse
events was collected only if they occurred at least 48 h
after the cyclophosphamide pulse. The duration between
the cyclophosphamide pulse and each adverse event was
also recorded. Details are provided in Additional file 1.
According to French law (L.1121-1 paragraph 1 and
R1121-2, Public Health Code), neither informed consent
nor approval of an ethics committee was necessary for
anonymous data extraction from and analysis of patients’
Continuous variables are presented as median and
interquartile range, and categorical variables are reported as
frequency (percent). Two groups were defined according
to 90-day mortality: survivors and nonsurvivors.
Comparison between the two groups was performed on
continuous variables using Mann–Whitney U tests due to a
nonnormal distribution of all variables. For qualitative
variables, a χ2 test or Fisher’s exact test was used as
appropriate. Correlations were assessed using the Pearson
correlation test. Association between baseline ICU
characteristics with mortality was assessed in univariable and
multivariable logistic regression. Given the low number
of events, only two explanatory variables could be
entered in the multivariable models; that is, several models
were constructed, each containing two explanatory
variables. These candidate variables entered in multivariable
analysis were chosen on the basis of the preceding
univariable analysis (entry criteria p < 0.05 in univariable
analysis). Models adjusted for various possible
confounders (age, SOFA score at admission, SAPS II at
admission) were ultimately presented. Because of the
absence of a universally accepted threshold, continuous
variables were categorized according to the thresholds
identified using the Youden index from receiver
operating characteristic curve analyses. Mortality was
described using Kaplan–Meier survival estimates and
compared between the group baseline characteristics
by log-rank tests. All analyses were performed using
Prism software (GraphPad Software, La Jolla, CA, USA)
and IBM SPSS Statistics 20.0 software (IBM, Armonk,
NY, USA). The two-tailed significance level was set at
p < 0.05.
The study population characteristics are provided in
Table 1. In the 20 participating centers, 82 patients (36
women, 46 men) with a median age of 67.0 years (63.0–
74.5) were included from January 2001 to December 2014
(Fig. 1). The delay between traditional hospitalization
wards and ICU admission was 6.5 days (1–14). Among
the study population, 52 patients (63 %) had no prior
medical history and 78 patients (95 %) had a performance
status score of 0 or 1. Of the included patients, 77 (94 %)
were admitted for a new or recent diagnosis of SVV, with
GPA (Wegener’s) being the main diagnosis (44 patients,
54 %). Thirteen patients (16 %) were admitted to the ICU
for an anti-GBM antibody disease. The predominant
clinical patterns at admission were pulmonary-renal syndrome
(27 patients, 33 %), isolated respiratory failure (28 patients,
34 %), and isolated renal failure (24 patients, 29 %).
Reasons for admission for all patients with acute renal failure
were indications of renal replacement therapy with the
need to pursue PLEX. SAPS II and BVAS at admission
were 37.5 (28.0–46.5) and 16.0 (12.0–20.0), respectively.
Small-vessel vasculitis and ICU management
Data for small-vessel vasculitis and ICU management
are provided in Table 2. All patients received
cyclophosphamide with a median dose of 1000 mg (800–1000).
Glucocorticoid pulses were administered in 74 patients
(90 %), and 79 patients (96 %) received daily high-dose
glucocorticoids. PLEX was performed in 63 patients
(77 %). In the ICU, 42 patients (51 %) required
mechanical ventilation during 11.5 days (8.0–22.5) and 25
patients (31 %) received vasopressor therapy during
7.0 days (3.0–18.5). Renal replacement therapy was
performed in 58 patients (71 %) for 13.0 days (8.0–20.75)
and was maintained after ICU stay in 28 patients (34 %).
Adverse events in the ICU
Data for adverse events in the ICU are given in Table 3.
Nine patients (11 %) presented with neutropenia <1500/
mm3 after the cyclophosphamide pulse, three (4 %) of
whom had a nadir <500/mm3. Infection was reported in
25 patients (30 %), with the lung being the most
frequently infected site (15 patients, 60 %), predominantly
by Gram-negative microorganisms (16 patients, 64 %).
Unfavorable evolution toward septic shock was observed
in 13 patients (16 %). Venovenous extracorporeal
membrane oxygenation was initiated for refractory respiratory
failure in six patients (7 %), four of whom survived.
Lastly, 57 patients (69 %) presented with at least one
hemorrhagic syndrome during their ICU stay. The main
cause of death in the ICU was disease flare in 69 % of
cases, followed by infection in 31 % of cases.
Comparison between survivors and nonsurvivors at 90 days
Data derived from comparison of survivors and
nonsurvivors at 90 days are provided in Table 4, and the results
of univariable and multivariable analyses are given in
Overall mortality was 18 % (15 deaths) (Fig. 2). All
patients with an anti-GBM disease survived at 90 days.
Anti-GBM disease is known to have a better prognosis,
which may have lowered the mortality rate. After
removing patients with anti-GBM disease and considering only
patients with AAV, we found that the mortality rate in
the ICU and at 90 days remained less than 20 % and less
than 25 %, respectively.
Sex, medical history, performance status before ICU
admission, vasculitis type, delay between hospitalization
ward and ICU admission, reason for admission,
induction treatment for SVV, revised FFS, BVAS, and SOFA
score at admission were not significantly different
between survivors and nonsurvivors.
Nonsurvivors were older than survivors (67 years [62.0–
74] vs. 58.0 years [40–68], p = 0.003). SAPS II score was
also significantly higher at ICU admission in nonsurvivors
than in survivors (51 [38–82] vs. 36 [27–42], p = 0.005). A
higher SOFA score on the day of cyclophosphamide
administration (survivors 6 [
] vs. nonsurvivors 11 [
], p = 0.0004), with a threshold value of 8 (sensitivity
73 %, specificity 88 %), was associated with death (Fig. 3).
A delayed administration of cyclophosphamide after ICU
admission (survivors 2.0 days [1.0–5.0] vs. nonsurvivors
5.0 days [3.0–14.0], p = 0.0053), with a threshold value of
3.5 days (sensitivity 73 %, specificity 61 %), was also
associated with unfavorable evolution.
In univariable logistic regression, SOFA score on the
day of cyclophosphamide administration and timing
between admission and administration of
cyclophosphamide were significantly associated with outcome
(respectively, odds ratio [OR] with 95 % confidence
interval [CI] for a 1-point increase in SOFA score 1.32
[1.13–1.55], p < 0.001; and for a 1-day increase in delay
1.15 [1.04–1.28]; p = 0.007) (Table 5).
In multivariable analysis (Table 5), both SOFA score
on the day of cyclophosphamide administration and
timing between admission and administration of
cyclophosphamide were significantly associated with outcome (OR
for a 1-day increase in delay 1.16 [95 % CI 1.05–1.29], p
= 0.005; and OR for a 1-point increase in SOFA 1.35
[1.14–1.60]; p < 0.001). All other models identified a
significant association for delay between admission and
either administration of cyclophosphamide or SOFA score
on the day of cyclophosphamide administration, except
when adjusted for SAPS II (OR 1.20 [95 % CI 0.96–
1.48], p = 0.11).
All nonsurvivors received mechanical ventilation and
vasopressor therapy (Additional file 2: Table 6).
The main results of the present multicenter study of
patients admitted to the ICU with SVV are as follows: (1)
Sequential Organ Failure Assessment score at cyclophosphamide administration
Delay between ICU admission and cyclophosphamide administration, days
ICU intensive care unit
Data are presented as number (%) or median (interquartile range)
aMissing data: 3
bTwo patients with encephalitis and one with myocarditis
mortality represented about one-fifth of the included
population, despite life-threatening manifestations at
admission requiring aggressive immunosuppressive
therapy; (2) subject to other undetected confounding factors
that we were not able to include in the multivariable
analysis, ICU severity score, such as SOFA score on the
day of cyclophosphamide administration in the ICU, also
seemed to be associated with unfavorable outcome; and
(3) delayed administration of cyclophosphamide was also
likely associated with death.
Causes of ICU admission
Due to the noninclusion criteria, only one patient
presented with septic shock at admission and was diagnosed
thereafter with AAV. Consequently, all patients were
admitted for acute manifestations of the disease, which
CI confidence interval, ICU intensive care unit, OR odds ratio, SAPS Simplified Acute Physiology Score, SOFA Sequential Organ Failure Assessment
Data are presented as odds ratio (95 % confidence interval)
consisted mainly of acute respiratory failure or/and acute
renal failure. Overall, respiratory failure was present in
two-thirds of our patients. In accordance with this, in
the studies of Khan et al. [
] and Monti et al. [
clinical presentations such as acute respiratory failure
related to diffuse intraalveolar hemorrhage were also
reported to be the first manifestation of AAV at ICU
Immunosuppressive therapy–related infection in the ICU
The rate of acquired infection hovered at 30 % and was
surprisingly less than that of other populations usually
admitted to the ICU [
]. This low rate of infection may
be explained by the low exposure of patients to chronic
immunosuppressive therapies: Only four patients had
been receiving chronic immunosuppressive therapy for
more than 6 months before ICU admission. In a
retrospective series, Cruz et al. found that patients admitted for
an infectious process tended to have a higher mortality
]. Similarly, Befort et al. recently reported that cause
of death was related mainly to an infectious process in
61 % of ICU patients [
]. Prolonged exposure to
immunosuppressive therapies such as corticosteroids before ICU
admission is also known to be independently associated
with a higher risk of death [
]. Conversely, results from
the CORTAGE trial confirmed that low cumulative doses
of corticosteroids and limited doses of cyclophosphamide
at 500 mg per pulse were associated with a lower
occurrence of infection in the elderly [
Cyclophosphamide has long been the standard
induction treatment in acute manifestations of severe AAV.
Randomized controlled trials have also shown that
rituximab was noninferior to cyclophosphamide therapy for
remission induction in these patients [
the latter study excluded patients with either alveolar
hemorrhage sufficiently severe to require mechanical
ventilation or with a serum creatinine level greater than
350 μmol/L. Patient subset analyses including
onefourth of participants with diffuse alveolar hemorrhage
or those with major renal disease did not reveal any
between-arm differences in remission rate . In these
studies, there were no significant differences between
the two treatments with respect to adverse events. In the
particular setting of the ICU, one can speculate whether
rituximab would not be safer than cyclophosphamide for
infectious adverse events [
It is noteworthy that a high number of our patients
were treated with PLEX as an adjunct for frequent acute
respiratory failure and/or acute renal failure at patient
admission. Patients with respiratory failure due to diffuse
alveolar hemorrhage are thought to benefit from PLEX,
and the rate of renal recovery in AAV presenting with
renal failure has furthermore been shown to be
increased with PLEX [
]. The latter is the subject of a
large, ongoing, multicenter randomized controlled trial
to confirm these data in this patient population
(PEXIVAS; ClinicalTrials.gov identifier NCT00987389).
Despite increased use, intensivists do not routinely
prescribe immunosuppressive therapies for the management
of severe vasculitis. In the ICU, their prescription in
instances of multiple organ failure could seem
counterintuitive at first glance and most often is associated with
an increased complication rate and potentially with a
negative outcome. In view of our results, this paradigm
appears not to be justified for acute manifestations of
SVV. Previous studies have furthermore found highly
heterogeneous results with regard to ICU mortality (11–
52 %). One major reason may be related to the
heterogeneity of the included population. Indeed, most of these
studies involved, on one hand, mixed samples including
relapse and new diagnoses of various classes of
necrotizing vasculitis and, on the other hand, acute manifestations
of the disease as well as chronic
immunosuppressiverelated infections [
6, 7, 9, 18, 24
]. Owing to the high
degree of homogeneity of our population, only a small
number of factors appeared to be associated with ICU
mortality. As expected, a high SAPS II score, which is a
nonspecific ICU severity score assessed at admission, was
associated with worst outcome. This score was also
systematically found to be predictive of ICU mortality in all
other previous studies [
6, 7, 9, 18, 25
]. In univariable and
multivariable analysis, SOFA score measured on the day
of cyclophosphamide administration in the ICU was
strongly associated with a poorer outcome. The delayed
administration of cyclophosphamide in the ICU was also
associated with a higher mortality rate. Considering that
all patients included in this study presented with an acute
manifestation of SVV, it is not surprising that delayed
administration of the induction immunosuppressive
treatment was associated with death. Similar to the results of
the studies of Cruz et al. [
] and Khan et al. [
], BVAS was
also a poor predictor of ICU mortality. Indeed, a number
of items in this score are a reflection much more of
vasculitis activity than of an acute life-threatening manifestation
of SVV. Similarly, it was not surprising that FFS was not
associated with poor outcome. In the present series, as in
others, cardiac symptoms or gastrointestinal involvement,
two main criteria included in the FFS, were rare or not
found at ICU admission [
The present study is limited by its retrospective nature.
Considering the very low incidence rate of SVV with the
prespecified inclusion criteria, it would be difficult to
conduct a prospective study. Nonetheless, we report a
large retrospective analysis of 82 patients at 20 different
centers, hence limiting center bias.
Due to the limited number of events recorded in our
moderate sample size, we could not adjust for other
numerous potential confounders in the multivariable
analysis. Adjusting for confounders not identified as
significant in this analysis could have weakened the
It is usual to report the outcome of small-vessel
vasculitis at 12 and 60 months because the efficacy of the
immunosuppressive therapies can be assessed only after
prolonged follow-up. In the present study, we decided to
report the outcome only at 90 days for the following
reasons. First, the outcome at 90 days represents the
specific consequences of ICU stay. Second, with a
retrospective multicenter study design, data for longer-term
outcomes are most often not fully available.
Patients admitted to the ICU for life-threatening
complications at the initial phase of SVV have an 82 % survival
rate. Mortality is positively related to the intensity of
organ failure. Delayed immunosuppressant use in the
ICU appears to be associated with mortality. Thus,
the present study sheds new light on the potential
importance of a rapid approach in the treatment of
Patients admitted to the ICU for acute manifestation
of small-vessel vasculitis have an 82 % survival rate.
Even in the case of multiple organ failure, delayed
administration of immunosuppressants is associated
Additional file 1: Outcome of patients admitted to the ICU for
acute manifestation of small-vessel vasculitis. (DOCX 23 kb)
Additional file 2: Table 6 Comparison of 90-day survivors and
nonsurvivors with regard to disease management and adverse
events. (DOCX 17 kb)
AAV: antineutrophil cytoplasmic antibody–associated vasculitis;
BVAS: Birmingham Vasculitis Activity Score; CI: confidence interval; FFS:
FiveFactor Score; GBM: glomerular basement membrane; GPA: granulomatosis
with polyangiitis; ICD: International Classification of Diseases; ICU: intensive
care unit; IQR: interquartile range; OR: odds ratio; PLEX: plasma exchange;
SAPS: Simplified Acute Physiology Score; SOFA: Sequential Organ Failure
Assessment; SVV: small-vessel vasculitides.
The authors declare that they have no competing interests.
AK and EB acquired the clinical data. NG and NeA performed statistical
analysis and interpretation of the clinical data. VD, NT, PT, PA, SE, GG, SG,
NaA, AD, ASM, EA, JPQ, JBH, GL, RS, ND, XP, DW, and BL drafted the
manuscript for important intellectual content. All authors read and approved
the final manuscript.
We thank Pierre Pothier for editing the manuscript.
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