Necrotizing fasciitis in systemic lupus erythematosus.

OBJECTIVES: Necrotizing fasciitis (NF) is an uncommon infection of the subcutaneous tissue and superficial fascia. Any delay in treatment can lead to catastrophic results with high mortality. It is well known that patients with systemic lupus erythematosus (SLE) are at increased risk of infection, from the disease and/or its treatment. The objective of our study was to evaluate the presenting features of NF in SLE patients and to identify possible risk factors for this severe complication. METHODS: We searched for patients with diagnoses of SLE and NF using a computerized patient database at Montefiore Medical Center (MMC), from 1996 to present. We also included patients from the MMC Lupus Clinic with these diagnoses (identified from paper records) from 1994 to present. Of a total of 449 patients with SLE that were followed during this time, 8 patients with NF were identified, and their records were reviewed. RESULTS: Two of the 8 patients (25%) died during hospitalization. A third patient died within 2 months of hospital discharge. All 8 patients were receiving steroids at the time of diagnosis, and 7 of 8 had hypoalbuminemia and lymphopenia. Both patients who died in the hospital and the one patient who died within 2 months of her discharge had lupus nephritis. CONCLUSIONS: NF is an uncommon infection, but one that must be recognized early if the outcome is to be favorable. This series of 8 cases of NF in SLE from a single institution suggests that heightened awareness is warranted, particularly among SLE patients who are immunosuppressed by virtue of their underlying disease, the therapy they require, or both.

The effect of combined estrogen and progesterone hormone replacement therapy on disease activity in systemic lupus erythematosus: a randomized trial.

BACKGROUND: There is concern that exogenous female hormones may worsen disease activity in women with systemic lupus erythematosus (SLE). OBJECTIVE: To evaluate the effect of hormone replacement therapy (HRT) on disease activity in postmenopausal women with SLE. DESIGN: Randomized, double-blind, placebo-controlled noninferiority trial conducted from March 1996 to June 2002. SETTING: 16 university-affiliated rheumatology clinics or practices in 11 U.S. states. PATIENTS: 351 menopausal patients (mean age, 50 years) with inactive (81.5%) or stable-active (18.5%) SLE. INTERVENTIONS: 12 months of treatment with active drug (0.625 mg of conjugated estrogen daily, plus 5 mg of medroxyprogesterone for 12 days per month) or placebo. The 12-month follow-up rate was 82% for the HRT group and 87% for the placebo group. MEASUREMENTS: The primary end point was occurrence of a severe flare as defined by Safety of Estrogens in Lupus Erythematosus, National Assessment-Systemic Lupus Erythematosus Disease Activity Index composite. RESULTS: Severe flare was rare in both treatment groups: The 12-month severe flare rate was 0.081 for the HRT group and 0.049 for the placebo group, yielding an estimated difference of 0.033 (P = 0.23). The upper limit of the 1-sided 95% CI for the treatment difference was 0.078, within the prespecified margin of 9% for noninferiority. Mild to moderate flares were significantly increased in the HRT group: 1.14 flares/person-year for HRT and 0.86 flare/person-year for placebo (relative risk, 1.34; P = 0.01). The probability of any type of flare by 12 months was 0.64 for the HRT group and 0.51 for the placebo group (P = 0.01). In the HRT group, there were 1 death, 1 stroke, 2 cases of deep venous thrombosis, and 1 case of thrombosis in an arteriovenous graft; in the placebo group, 1 patient developed deep venous thrombosis. LIMITATIONS: Findings are not generalizable to women with high-titer anticardiolipin antibodies, lupus anticoagulant, or previous thrombosis. CONCLUSIONS: Adding a short course of HRT is associated with a small risk for increasing the natural flare rate of lupus. Most of these flares are mild to moderate. The benefits of HRT can be balanced against the risk for flare because HRT did not significantly increase the risk for severe flare compared with placebo.