Elsevier

Autoimmunity Reviews

Volume 12, Issue 3, January 2013, Pages 380-395
Autoimmunity Reviews

Review
Autoimmune diseases in the intensive care unit. An update

https://doi.org/10.1016/j.autrev.2012.06.002Get rights and content

Abstract

Autoimmune diseases (ADs) are a challenge at the intensive care unit. The management of patients with these diseases in the critical care setting has improved over time since there are new and more aggressive alternatives to treat and diagnose them. We aimed to review the current causes of admission, clinical features, outcomes and variables associated with mortality of patients with ADs admitted to the intensive care unit (ICU). International classification criteria for ADs were used to include patients. Search was done through PubMed, SCOPUS, SciELO, and LILACS databases up to December of 2011.Twenty-nine case series and forty-one case reports were analyzed after quality assessment. Respiratory involvement was the leading cause of admission. Systemic lupus erythematosus (SLE) (33.5% of reported patients), rheumatoid arthritis (25%) and systemic vasculitis (15%) were the most frequent ADs in patients admitted to the ICU in the last decade. Mortality ranged from 17% to 55% in case series including all ADs, but in the ones that only included patients with a specific AD, such as SLE, it reached up to 79%. High APACHE score, multi-organ dysfunction, older age and cytopenia were the most reported variables associated with mortality. In conclusion, ADs should always be considered in patients with life threatening conditions that warrant critical care. Variables influencing mortality should be promptly identified in order to improve the patients' outcomes.

Introduction

Systemic manifestations of autoimmune diseases (ADs), which are a heterogeneous group of entities, can become so severe that they endanger the lives of patients. Of all the patients with ADs presenting to the emergency room, the majority have a rheumatologic AD and up to 25% of them require hospitalization. Of these, up to one third will require care and support in an intensive care unit (ICU) [1]. The reported in-ICU mortality of patients with ADs reaches as high as 55% depending on the series [1], [2], [3]. Besides increased risk of mortality, critical illness is also associated with other long-term outcomes such as persistent cognitive impairment [4], which has a negative impact on quality of life and the ability of those who survived an illness that required ICU to reintegrate into daily life [5], [6].

Patients with any of these diseases usually have multiple risk factors for in-hospital mortality and morbidity and are most likely to end up in the ICU, where they represent a challenge for the entire intensive care team. Moreover, it has been reported that, from 1996 to 2003, between 7 and 42% of individuals with an AD were first diagnosed during their ICU stay [7], [8], [9], [10], [11]. This supports our argument that this group of diseases should always be considered in the differential diagnosis in patients at ICU in order to not delay prompt and accurate treatment.

In 2005, we published a case series report and review of literature of patients with ADs requiring intensive care in a third level hospital [2]. Since then, there are no current reports that describe whether the AD situation in the ICU remains the same or if it has changed over time given the advances in the diagnostic procedures that allow an early diagnosis and aggressive treatment of ADs. The aim of the present study was to update the causes of admission, clinical features, and outcomes of patients with ADs requiring intensive care as well as variables associated with mortality that could be possible prognostic mortality factors. Another goal was to describe the state of the art of alternative therapeutic strategies in the ICU to manage life-threatening conditions and manifestations common to all ADs focusing on therapeutic plasma exchange (TPE) and intravenous immunoglobulins (IVIg).

Section snippets

Search strategy

A search was done of PubMed (2005–2011), SCOPUS (2005–2011), SciELO (2005–2011), and LILACS (2005–2011) up to December of 2011 by two reviewers independently to identify studies that measured mortality and described causes of admission, clinical features, variables associated with mortality, alternative therapeutic strategies, and outcomes of patients with ADs. In the search strategy, the combinations of the following MeSH terms were used: “Systemic Vasculitis” [Mesh], “Vasculitis” [Mesh],

Search results and study characteristics

Using our search strategy, 1240 articles were identified and, of these, 123 were chosen for full text review. One hundred and five articles met inclusion criteria and were included in the review (Fig. 1). The articles were grouped as case series reports [2], [3], [7], [9], [10], [11], [14], [15], [16], [17], [18], [19], [20], [21], [22], [23], [24], [25], [26], [27], [28], [29], [30], [31], [32], [33], [34], [35], [36] (Table 1, Table 2), case reports [37], [38], [39], [40], [41], [42], [43],

Discussion

After retrieving all relevant articles, and doing a thorough review of the information, it became evident that there have not been a significant number of publications on the subject in the period of 2005 to 2011. Therefore, we decided to compare the behavior of ADs in the ICU before and after year 2000. This cutoff point in time was selected because of the change in the prevalence of the most frequently reported ADs from RA to SLE, which indicates a change in the dynamics of these disorders in

Conclusions

In the ICU, ADs generate significant mortality according to one of the latest case series which showed an overall in-ICU mortality of up to 55%. Of this group of diseases, SLE, RA and systemic vasculitis are the most common ones associated with admission to the ICU. Although APACHE II score and other variables such as the previous health status have been associated with mortality, studies are needed to develop a prognosis model that would make it possible to combine all variables that might

Take-home messages

  • The most frequent autoimmune diseases (ADs) found in an intensive care unit (ICU) in the last decade are systemic lupus erythematosus, rheumatoid arthritis and systemic vasculitis.

  • High APACHE score, multi-organ dysfunction, older age and cytopenia are associated with mortality.

  • Variables influencing mortality should be promptly identified to improve outcomes.

  • AD should always be considered in patients with life threatening conditions in ICU.

Acknowledgements

We thank all the members of the Center for Autoimmunity Diseases Research (CREA) for their fruitful discussions. OLQ wishes to thank Juan Carlos Díaz Cortes for his constant support and inspiration. This work was financed by Colciencias (122254531722), and the Universidad del Rosario, Bogota, Colombia.

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