Outcome of Patients with Systemic Sclerosis Admitted to the Intensive Care Unit

Tarik Hissem¹, Alice Berezne², David Grimaldi³, Alain Cariou¹, Julien Charpentier¹, Jean-Daniel Chiche¹, Yannick Allanore⁴, Jean-Paul Mira¹, Frédéric Pène⁵ and Luc Mouthon⁶, ¹Intensive care unit - Cochin University Hospital, Paris, France, ²Paris Descartes University, Internal Medicine department, Cochin Hospital, Paris, France, ³Intensive Care Unit - Cochin University Hospital, Paris, France, ⁴Paris Descartes University, Rheumatology A Department and INSERM U1016, Cochin Hospital, Paris, France, ⁵Intensive care unit - Cochin university hospital, Paris, France, ⁶National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, Paris, France

Meeting: 2014 ACR/ARHP Annual Meeting

Keywords: Intensive care, pulmonary fibrosis and systemic sclerosis

Session Information

Title: Systemic Sclerosis, Fibrosing Syndromes and Raynaud's - Clinical Aspects and Therapeutics: Systemic Sclerosis Measures and Outcomes

Session Type: Abstract Submissions (ACR)

Background/Purpose

Studies on the outcome of patients with systemic sclerosis (SSc) in intensive care unit (ICU) are scarce in the literature.

Objective: To assess the characteristics and outcomes of patients with SSc admitted to ICU.

Methods

We performed a single-center retrospective study over a 4-year period (November 2006-December 2010). All patients with SSc admitted to the ICU were enrolled. The underlying disease’s features, reasons for ICU admission and organ failure supports were collected. We studied their short-term (ICU and hospital mortality) and long-term (6-month and 1-year mortality) outcomes, and we sought to identify prognostic factors. Variables were expressed as median (IQR) or number (percentage) as appropriate.

Results

Thirty five patients (age 51 [40-63] years, 77% female, SAPSII 35 [23-51]) were enrolled in the study. Twenty two (63%) and 13 (37%) patients displayed diffuse cutaneous and limited cutaneous SSc, respectively. The time from diagnosis to ICU admission was 80 [27-110] months. Twenty six (74%) patients had pulmonary fibrosis with the following baseline characteristics: diffusion capacity of lung for CO 33% [20-45], forced vital capacity 52% [39-66]. Ten patients had pulmonary hypertension with a systolic pulmonary artery pressure of 55 mmHg [45-63]. Only two (6%) had chronic renal insufficiency requiring long-term hemodialysis.

The main reasons for ICU admission were acute respiratory failure (69%) and acute renal failure (17%). During hospitalisation in ICU, 10 (28%) required endotracheal intubation and mechanical ventilation, 10 (28%) required hemodialysis and 9 (26%) required vasopressors. The overall ICU and hospital mortality rates were 26% and 34% respectively. Six-month and 1-year mortality rates were 40% and 46% respectively. None of the disease characteristics impacted the hospital outcome. Survivors and decedents were mostly different in terms of organ failures, most especially respiratory failure. Invasive mechanical ventilation was associated with a 100-percent hospital mortality rate and was the only independent prognostic factor.

Conclusion

This study reports on the most important cohort of patients with SSc in the ICU. Respiratory failure related to advanced-stage pulmonary fibrosis represents the main reason for ICU admission. In this setting, mechanical ventilation is constantly associated with hospital mortality. These results urge to reappraise the indications for ICU and mechanical ventilation in this high-risk subgroup of patients.

Disclosure:

T. Hissem,
None;

A. Berezne,
None;

D. Grimaldi,
None;

A. Cariou,
None;

J. Charpentier,
None;

J. D. Chiche,
None;

Y. Allanore,
None;

J. P. Mira,
None;

F. Pène,
None;

L. Mouthon,
None.

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