Treatment of the Systemic Capillary Leak Syndrome with Terbutaline and Theophylline: A Case Series

1 June 1999 | Volume 130 Issue 11 | Pages 905-909

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.

Author and Article Information

From the Mayo Clinic and Mayo Foundation, Rochester, Minnesota.

Presented in part at the 45th Annual Scientific Session of the American Heart Association, Orlando, Florida, 24-27 March 1996.

Acknowledgments: The authors thank staff and residents of the Mayo Clinic and referring staff who participated in the care of these patients.

Grant Support: By research grant CA62242 from the National Institutes of Health.

Requests for Reprints: Philip R. Greipp, MD, Mayo Clinic, 200 First Street SW, Rochester, MN 55905.

Current Author Addresses: Drs. Tahirkheli and Greipp: Mayo Clinic, 200 First Street SW, Rochester, MN 55905.