Adult respiratory distress syndrome in systemic lupus erythematosus: causes and prognostic factors: a single center, retrospective study

Objectives: To determine the causes and prognostic factors of Adult Respira tory Distress Syndrome (ARDS) in patients with systemic lupus erythematosus (SLE). Methods:,Among 543 Korean SLE patients, who were followed in the Lupus Clin ic of the Catholic Medical Center from 1993 to 1997, patients diagnosed as ARDS were examined retrospectively. During the study period, non-SLE patien ts with ARDS were investigated and then compared to SLE patients with ARDS in terms of clinical variables. Results: Nineteen patients with SLE were revealed to have a history of ARDS (3.5%) and 13 (68.4%) of 19 patients died. Death related to ARDS was found in 34.2% of all deaths (n = 38) from SLE during the study period. The freq uency and causes of ARDS were as follows; 9 sepsis or bacteremia (47.4%), 2 miliary tuberculosis (10.5%), 2 invasive pulmonary aspergillosis (10.5%), 2 acute pulmonary alveolar hemorrhage syndrome (10.5%), 1 acute lupus pneum onitis (5.3%), I massive hemorrhage due to placenta previa (5.3%), 1 aspira tion pneumonitis (5.3%), 1 disseminated intravascular coagulation associate d with systemic vasculitis (5.3%). The main organisms in sepsis were gram n egative bacilli (61.5%) The median steroid dose administered 1 month before ARDS was significantly higher in patients (n = 13) with infectious ARDS co mpared to those (n = 6) with ARDS due to other causes (P = 0.038). Comparis on of the laboratory and clinical variables between the survivors (n = 6) a nd the deceased (n = 13) showed that the survivors had lower SLAM indices a t presentation (P = 0.004) and APACHE (Acute Physiology, Age, Chronic Healt h Evaluation) III scores within 24 h after diagnosis of ARDS (P = 0.024) th an the deceased. The APACHE III scores correlated well with the SLAM indice s (r = 0.615, P = 0.007). Non-SLE patients with ARDS during the study perio d were selected for comparison to SLE patients with ARDS. Age at the onset of ARDS was younger in SLE (n = 19) compared to non-SLE (n = 190) (P < 0.00 1). Duration from ARDS onset to death was shorter in SLE patients (P < 0.00 1). The mortality from ARDS tended to be higher in SLE patients (P = NS). T he first-day APACHE III score was significantly higher in deceased SLE pati ents (n = 13) compared to deceased non-SLE patients (n = 105) (P = 0.001). Conclusions: ARDS was a common premortem event of SLE and showed a high fat ality rate in SLE. The most common cause of ARDS in Korean patients with SL E was sepsis by gram negative bacilli. ARDS in SLE developed at a younger a ge, and progressed more rapidly compared to ARDS in general. The SLAM index and APACHE III score could be useful to predict the prognosis of ARDS in S LE.