Rheumatologic diseases in the intensive care unit: epidemiology, clinical approach, management, and outcome☆

To evaluate the outcomes of hospitalized patients in two intensive care units (ICU) treated with intravenous immunoglobulin (IVIg) added to standard-of-care therapy. The indications for IVIg therapy were sepsis or autoimmune disease.

We conducted a retrospective study involving adult patients with sepsis and autoimmune diseases, who received IVIg in the ICU at Wolfson and Sheba Medical Centers. A predefined chart was compiled on Excel to include a complete demographic collection, patient comorbidities, chronic medication use, disease severity scores (Charlson Comorbidity Index; SOFA and APACHE II index scores), indication and dosage of IVIg administration, duration of hospitalization and mortality rates.

Patients (n - 111) were divided into 2 groups: patients with sepsis only (n-67) and patients with autoimmune disease only (n-44). Septic patients had a shorter ICU stay, received IVIg early, and had reduced mortality if treated with high dose IVIg. Patients with autoimmune diseases did not have a favorable outcome despite IVIg treatment. In this group, IVIg was administered later than in the sepsis group.

IVIg therapy improved the outcomes for ICU patients with sepsis.

Systemic lupus erythematosus (SLE), primary Sjögren's syndrome (pSS), systemic sclerosis (SSc), idiopathic inflammatory myopathies (IIM) and rheumatoid arthritis (RA) are connective tissue diseases (CTD) whose complications can lead to management in the intensive care unit (ICU).

To estimate by meta-analysis ICU mortality rates for CTD.

A systematic literature review was performed to identify articles studying critically ill CTD patients. A random-effects model was chosen for analysis. Pooled proportion mortality was calculated using aggregated-data meta-analysis with a random-effects model and assessment of heterogeneity with the I² statistic. Risk of bias was assessed using the quality assessment tool.

Of the 5694 individual publications, a sample of 31 independent cohorts was used for the meta-analysis totalling 5007 patients. The main cause for admission was sepsis (43%) followed by “flare-ups” (40%). The overall pooled proportion of mortality of CTD patients across all 31 studies was 33% (95%CI: 28–38%). In the IIM subgroup and that of SSc, mortality was 70% (95%CI: 46–86%) and 40% (95%CI: 25–47%), respectively. In the SLE subgroup, mortality was similar to the overall pooled mortality of 35% (95%CI: 29–42%). Subgroup mortality for RA and pSS patients was respectively 20% (95%CI: 11–33%) and 17% (95%CI: 6–41%); lower than the overall pooled mortality. Heterogeneity in each subgroup remained high.

The overall pooled proportion of mortality of ICU patients with CTD was 33% (95%CI: 28–38%), with a high heterogeneity (I²= 89%). In the subgroup analysis, mortality was higher for patients with IIM and SSc.

Critically ill patients with systemic rheumatic disease (SRD) have benefited from better provision of rheumatic and critical care in recent years. Recent comprehensive data regarding in-hospital mortality rates and, most importantly, long-term outcomes are scarce.

The aim of this study was to assess short and long-term outcome of patients with SRD who were admitted to the ICU.

All records of patients with SRD who were admitted to ICU between 2006 and 2016 were reviewed. In-hospital and one-year mortality rates were assessed, and predictive factors of death were identified.

A total of 525 patients with SRD were included. Causes of admission were most frequently shock (40.8%) and acute respiratory failure (31.8%). Main diagnoses were infection (39%) and SRD flare-up (35%). In-hospital and one-year mortality rates were 30.5% and 37.7%, respectively. Predictive factors that were associated with in-hospital and one-year mortalities were, respectively, age, prior corticosteroid therapy, simplified acute physiology score II ≥50, need for invasive mechanical ventilation, or need for renal replacement therapy. Knaus scale C or D and prior conventional disease modifying antirheumatic drug therapy was associated independently with death one-year after ICU admission.

Critically ill patients with SRD had a fair outcome after an ICU stay. Increased age, prior corticosteroid therapy, and severity of critical illness were associated significantly with short- and long-term mortality rates. The one-year mortality rate was also associated with prior health status and conventional disease modifying antirheumatic drug therapy.

Systemic autoimmune diseases are a heterogeneous group of disorders associated with dysfunction of multiple organs and unpredictable course. Complicated management and treatment become even more challenging when patients require critical care. This study aims to compare outcomes of small-vessel vasculitides (SVV) and other systemic autoimmune diseases (SAD) patients admitted to the intensive care unit (ICU).

Retrospective, observational study conducted in the ICU of Allergy and Immunology Department at the University Hospital in Krakow, Poland, between years 2001–2014, with 5-years follow-up and no lost to follow-up patients.

74 patients with autoimmune diseases were enrolled in the study - 23 with SVV and 51 with SAD. Patients in the SVV group achieved higher scores in APACHE II and III SAPS II and SOFA at ICU admission. The SVV patients required renal replacement techniques, blood products transfusion and immunosuppressive treatment more often. SVV patients had higher ICU mortality (60.9% vs. 35.3%, p = .04), however after discharge from ICU, in long term follow-up (1 year and 5 years) mortality was similar in both studied groups.

Among systemic autoimmune diseases small vessel vasculitides appear to be associated with the highest ICU mortality, higher requirement for advanced procedures and aggressive immunosuppressive therapy.