Posterior reversible encephalopathy syndrome in systemic lupus erythematosus.

OBJECTIVE

To study the clinical profile of posterior reversible encephalopathy syndrome (PRES) in patients with systemic lupus erythematosus (SLE) and analyze the risk factors and outcomes associated with it.

METHODS

We identified patients with SLE and PRES from January 2006 to October 2010. Data were collected on demographic details, lupus characteristics, PRES-related features, laboratory abnormalities, treatment details, and outcomes.

RESULTS

We studied 13 patients (all female) ages 14-37 years (median 23 yrs; 4 were aged < 18 yrs with juvenile SLE). Duration of lupus ranged from 1.5 to 36 months (median 6 mo). Six patients had PRES as a part of their initial presentation of lupus. All had active lupus and hypertension; 9 had nephritis. Four patients were on treatment with cyclophosphamide therapy when they developed PRES. Antihypertensives and antiepileptics were the mainstay of treatment along with supportive care. Immunosuppressive therapy was guided by lupus-related major organ manifestations. Two patients had focal neurological deficits; one had persistent hemiparesis at followup. One patient died.

CONCLUSION

PRES occurs in young lupus patients and in the early part of the disease. Focal deficits are not uncommon. It can be the presenting manifestation of lupus. Management is predominantly symptomatic. Immunosuppression is directed by other major organ manifestations. Early diagnosis and appropriate management is productive.