The objective of the study was to identify the causes, outcome and prognosis of severe illness in patients with systemic lupus erythematosus (SLE) requiring intensive care unit (ICU) care in a University Hospital over a five-year period. The design was a cohort study. Forty-eight SLE patients requiring ICU management over a five-year period (January 1997-December 2001) were studied prospectively. Of 48 patients, 14 (29.2%) died, predominantly with multiorgan dysfunction syndrome (MODS). Patients whose APACHE II score was equal to or greater than 20 had higher mortality than those with APACHE score below 20 (60 versus 7.1%; and P < 0.01). All the 18 patients whose health status rated as 'good' survived, while 46.7% of 30 patients whose health rated as 'poor' died (P < 0.01). Patients who had thrombocytopenia associated with sepsis and/or disseminated intravascular coagulopathy (DIC) had the highest mortality (75%, five-year survival). In conclusion, SLE patients admitted to the ICU had a lower mortality rate than some of the previous reports. Patients with SLE with high APACHE score, > or =20, poor health status, thrombocytopenia and multiorgan dysfunction syndrome had poor prognosis in the ICU.

Thrombotic thrombocytopenic purpura (TTP) occurring in patients with systemic lupus erythematosus (SLE) is rare and can be difficult to diagnose because of overlapping features of the two disorders. The aim of this study is to further characterize this uncommon association in terms of presenting features, diagnostic difficulties and treatment outcome. This is the largest series from a single centre with 6 patients diagnosed over a 6-year period. Two thirds of the patients had a simultaneous diagnosis of TTP and SLE. Half of the patients had a positive Coombs test along with clear features of TTP. Five patients received plasmapheresis as initial treatment while 1 patient received plasma infusions only. Four out of 5 patients responded to plasmapheresis and only 1 patient required cytotoxic therapy. TTP in association with SLE appears to be underdiagnosed and a positive Coombs test is not against the diagnosis of TTP in this setting. Most of the patients respond well to plasmapheresis. In case of a poor response, cytotoxic drugs should be considered early.