Treatment of the systemic capillary leak syndrome with terbutaline and theophylline - A case series

Background: The systemic capillary leak syndrome is a rare idiopathic disorder characterized by recurrent episodes of hypotension and hemoconcentration due to sudden transient extravasation of 10% to 70% of plasma. Mortality rates 5 years after diagnosis have been reported to be 76%. Objective: To assess the efficacy of a prophylactic regimen for the systemic capillary leak syndrome. Design: Case series. Setting: Tertiary referral center. Patients: Eight patients followed over the past 18 years. Intervention: Oral terbutaline plus aminophylline or theophylline. Measurements: Long-term clinical follow-up. Results: During a median follow-up of 9 years (range, 2 to 18 years), two patients (25%) died: one during an acute episode and one of complications related to long-term corticosteroid therapy. The other six patients are alive and healthy. The frequency and severity of the episodes decreased by a median of 30-fold. Recurrences were associated with decreased serum theophylline levels, possibly caused by enzyme induction or autoinduction. The extended-release form of medication was more successful. Sympathomimetic side effects were significant. Conclusions: A regimen of terbutaline and theophylline seems to be effective prophylaxis against the systemic capillary leak syndrome. Maintenance of therapeutic drug levels was associated with favorable results.