Naeem K. Tahirkheli, MD; Philip R. Greipp, MD
Tahirkheli NK, Greipp PR. Treatment of the Systemic Capillary Leak Syndrome with Terbutaline and Theophylline: A Case Series. Ann Intern Med. 1999;130:905-909. doi: 10.7326/0003-4819-130-11-199906010-00015
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Published: Ann Intern Med. 1999;130(11):905-909.
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%.
To assess the efficacy of a prophylactic regimen for the systemic capillary leak syndrome.
Tertiary referral center.
Eight patients followed over the past 18 years.
Oral terbutaline plus aminophylline or theophylline.
Long-term clinical follow-up.
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.
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.
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Print ISSN: 0003-4819 | Online ISSN: 1539-3704
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