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.