RESUMO
Background: Recurrence risk of systemic lupus erythematosus (SLE)-associated venous thromboembolism (VTE) is unclear. Aim: To determine the recurrence risk of SLE-associated VTE overall and by presence of provoking factors and SLE flares. Methods: A multicenter, retrospective cohort study was conducted among patients with first SLE-associated VTE who discontinued anticoagulation. SLE flares were defined as Systemic Lupus Erythematosus Disease Activity Index 2000 greater than 4. The primary outcome was recurrent VTE. Incidence rates and cumulative incidences were calculated by presence of provoking factors and antiphospholipid syndrome (APS) at index VTE. The hazard ratio (HR) for recurrence after SLE flare-associated index VTE was estimated with Cox regression, adjusted for provoking factor presence and APS. Results: Eighty patients were included with 21 recurrent VTEs in median 8 years. For provoked index VTE, the recurrence rate in patients without APS was 1.1 per 100 person-years (PY; 95% confidence interval [CI], 0.1-3.1) and in the presence of APS 3.5 per 100 PY (95% CI, 0.9-8.9), yielding cumulative incidences of 7.5% (95% CI, 1.2%-21.7%) and 31.4% (95% CI, 6.3%-61.6%) respectively. For unprovoked index VTE, these analogous rates were 3.8 per 100 PY (95% CI, 1.2-9.0) and 16.7 per 100 PY (95% CI, 4.5-42.7), with cumulative incidences of 33.7% (95% CI, 10.7%-58.9%) and 54.2% (95% CI, 10.7%-84.5%), respectively. Forty-six index VTEs were flare associated, and the adjusted HR for recurrence was 0.4 (95% CI, 0.1-1.8) compared to those without flares at their index VTE. Conclusion: Antiphospholipid syndrome is the main determinant for recurrence risk of SLE-associated VTE irrespective of presence of a provoking factor. Future research should attempt to confirm that flare-associated VTE has a lower recurrence risk.
RESUMO
BACKGROUND: Primary medical treatment of idiopathic retroperitoneal fibrosis (RPF) increasingly is accepted. However, the optimum treatment strategy is still unclear. STUDY DESIGN: Case series. SETTING & PARTICIPANTS: Single tertiary care referral center. 24 patients with idiopathic RPF treated with prednisone for 1 year, if needed, with (urgent) renal drainage from June 1991 through October 2006. OUTCOME & MEASUREMENTS: Clinical improvement, laboratory parameters, repeated computed tomographic (CT) scanning. Treatment was considered successful if the following criteria were met at the end of the 1-year treatment period: significant subjective clinical improvement, (near-)normalization of acute-phase reactants, improvement in renal function with disappearance of ureteral obstruction, and CT-documented mass regression. Recurrence is defined as need for retreatment because of return of signs and symptoms after the 1-year treatment period in patients with initial treatment success. RESULTS: 22 patients reported significant to complete resolution of symptoms after median treatment duration of 2.0 weeks (0.7 to 3.0). Follow-up showed decreases in erythrocyte sedimentation rate, C-reactive protein level (both P < 0.0001), and serum creatinine level (P = 0.0230) at 6 weeks, which persisted during the treatment period. Repeated CT scanning showed mass regression in 19 patients during the treatment period. Six patients were considered treatment failures, and there were 23 recurrences 10 months (7 to 14) after prednisone withdrawal in 13 of 18 patients with initial treatment success. At the end of follow-up (median, 55 months), 7 patients had impaired renal function; 1 patient reached end-stage renal disease. The mortality rate was 8%. LIMITATIONS: There was no comparison with other treatments. CONCLUSION: One-year treatment with prednisone is associated with a high rate of initial success, but a high recurrence rate. Despite frequent disease relapse, long-term renal and patient outcome was good.