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

Background: The systemic capillary leak syndrome is a rare idiopathic disor der characterized by recurrent episodes of hypotension and hemoconcentratio n 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 systemi c 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 p atients (25%) died: one during an acute episode and one of complications re lated to long-term corticosteroid therapy. The other six patients are alive and healthy. The frequency and severity of the episodes decreased by a med ian of 30-fold. Recurrences were associated with decreased serum theophylli ne levels, possibly caused by enzyme induction or autoinduction. The extend ed-release form of medication was more successful. Sympathomimetic side eff ects were significant. Conclusions: A regimen of terbutaline and theophylline seems to be effectiv e prophylaxis against the systemic capillary leak syndrome. Maintenance of therapeutic drug levels was associated with favorable results.