• T Shalev, Y Haviv, E Segal, M Ehrenfeld, R Pauzner, Y Levy, P Langevitz, and Y Shoenfeld.
• Department of Medicine B and The Center for Autoimmune Diseases, The Chaim Sheba Medical Center, Tel Hashomer, Israel.
• Clin Exp Rheumatol. 2006 Jul 1;24(4):380-6.

ObjectivePatients with systemic rheumatic disease constitute a small percentage of admissions to the medical intensive care units (ICUs). Systemic sclerosis (SSc) is one of the rheumatic diseases that together with secondary complications may lead to a critical illness requiring hospitalization in the ICU. We present the features, clinical course and outcome of critically ill patients with scleroderma that were admitted to the ICU.MethodsThe medical records of nine patients with diagnosis of scleroderma (8 female, 1 male), admitted to the intensive care unit of Sheba Medical Center during the 11-year interval between 1991 and 2002, were reviewed.ResultsThe mean age of the patients at the time of admission to the ICU was 48 +/- 13 [SD] years. The mean duration of SSc from diagnosis to the ICU admission was 8 +/- 8 years. Six patients had diffuse SSc, two patients had limited SSc and one patient had juvenile diffuse morphea. The main reasons for admission to the ICU were: infection/ septic syndrome (n = 4), scleroderma renal crisis (SRC) with pulmonary congestion (n = 2), acute renal failure associated with diffuse alveolar hemorrhage namely scleroderma- pulmonary - renal syndrome (SPRS) (n = 1), iatrogenic pericardial tamponade (n = 1), mesenteric ischemia (n = 1). The patients had high severity illness score (mean APACHE II 25 +/- 3). Eight out of nine patients (89%) that were admitted to the ICU died during the hospitalization, six (66.6%) of them died in the ICU. Septic complications as the main cause of death were determined in five patients (62.5%), while four of them had pneumonia and acute respiratory failure along with underlying severe pulmonary fibrosis. Lungs and kidneys were the most common severely affected organs by SSc in our patients.ConclusionThe outcome of scleroderma patients admitted to the ICU was extremely poor. Infectious complication was the most common cause of death in our patients. Although infections are treatable, the high mortality rate for this group of patients was dependent on the severity of the underlying visceral organ involvement, particularly severe pulmonary fibrosis. The severity of this involvement is a poor outcome predictor. An early diagnosis and an appropriate treatment of such complications may help to reduce the mortality in scleroderma patients.

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