Outcome of patients with scleroderma admitted to intensive care unit. A report of nine cases

Tamar Shalev, Yaev Haviv, Eran Segal, Michael Ehrenfeld, Rachel Pauzner, Yair Levy, Pnina Langevitz, Yehuda Shoenfeld*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review


Objective: Patients 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 out-come of critically ill patients with scleroderma that were admitted to the ICU. Methods: The medical records of nine patients with diagnosis of scleroderma (8female, 1 male), admitted to the intensive care unit of Sheba Medical Center during the II-year interval between 1991 and 2002, were reviewed. Results: The 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 hadjuvenile diffuse morphea. The main reasons for admission to the ICU were: infection/septic syndrome (n = 4), scleroderma renal crisis (SRQ with pulmonary congestion (n = 2), acute renalfailure 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 hadpneumonia 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. Conclusion: The outcome of scleroderma patients admitted to the ICU was extremely poor. Infectious complication was the most common cause of death in ourpatients. 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.

Original languageEnglish
Pages (from-to)380-386
Number of pages7
JournalClinical and Experimental Rheumatology
Issue number4
StatePublished - Jul 2006


  • Intensive care unit
  • Mortality
  • Pulmonary fibrosis
  • Scleroderma
  • Scleroderma renal crisis
  • System rheumatic disease
  • Systemic scleroris


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