Capillary leak syndrome medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: M. Hassan, M.B.B.S
Overview
An episode of systemic capillary leak syndrome (SCLS) usually consists of two distinct phases, the capillary leak phase, and the recruitment phase.
The capillary leak phase may be managed with intravenous administration of saline solution plus hetastarch or albumin and colloids (to increase the remaining blood flow to vital organs like the kidneys), as well as glucocorticoids (steroids like methylprednisolone, to reduce or stop the capillary leak).
The recruitment phase is managed with diuretics to help the patient discharge the accumulated fluids quickly.
Medical Therapy
An episode of systemic capillary leak syndrome (SCLS) usually consists of two distinct phases:
The Capillary Leak Phase
The initial stage is the capillary leak phase, lasting from 1 to 3 days, during which up to 70% of total plasma volume may invade cavities in the trunk and extremities. The most common clinical features are fatigue, lightheadedness up to and including syncope (fainting), limb, abdominal or generalized pain, facial or other edema, dyspnea, and hypotension that results in circulatory shock and potentially in cardiopulmonary collapse and other organ distress or damage. Acute renal failure is a risk due to acute tubular necrosis consequent to hypovolemia and rhabdomyolysis.
The loss of fluid out of the capillaries has similar effects on the circulation as dehydration, slowing both the flow of oxygen delivered to tissues and organs as well as the output of urine. Urgent medical attention in this phase consists of fluid resuscitation efforts, mainly the intravenous administration of saline solution plus hetastarch or albumin and colloids (to increase the remaining blood flow to vital organs like the kidneys), as well as glucocorticoids (steroids like methylprednisolone, to reduce or stop the capillary leak). However, it is important to avoid overly aggressive intravenous fluid administration during this leak phase, because it may cause massive swelling of the extremities and thus serious collateral damage because of induced compartment syndromes.
The Recruitment Phase
The second stage features the reabsorption of the initially extravasated fluid and it usually lasts just as long as the leak phase. Intravascular fluid overload leads to polyuria and can cause flash pulmonary edema with possibly fatal consequences. The severity of the problem depends on to the quantity of fluid supplied in the initial phase, the damage that may have been sustained by the kidneys, and the promptness with which diuretics are administered to help the patient discharge the accumulated fluids quickly.[1]
| Treatment | Mechanism of Action | Efficacy in Systemic Capillary Leak Syndrome | Adverse effects |
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Endothelial signal transduction | |||
| Bevacizumab | ↓VEGF activity | No (chronic form) | hypertension, asymptomatic proteinuria, thromboembolic events, gastrointestinal perforation, and wound healing complications |
| Epoprostenol | prostacyclin analogue; ↑ endothelial cAMP, vascular smooth muscle relaxation | Yes, in acute setting | headache, hypotension and catheter-related infections |
| Theophylline + Terbutaline | phosphodiesterase inhibition,β-receptor stimulation; ↑ endothelial cAMP | Yes, acute and maintenance therapy | Theophylline: gastrointestinal distress, insomnia, and tremor
Terbutaline: nervousness, dizziness, drowsiness, difficulty falling asleep or staying asleep, weakness, headache |
Immune modulation | |||
| Corticosteroids | anti-inflammatory | ± (chronic form) | osteoporosis, fractures, and osteonecrosis, adrenal suppression, cushingoid features, diabetes and hyperglycemia, myopathy, glaucoma and cataracts, psychiatric disturbance, immunosuppression, cardiovascular adverse effects, gastrointestinal adverse effects, dermatologic adverse effects, growth suppression |
| Infliximab | ↓TNF activity | Yes, in acute setting | diarrhea, constipation, hepatotoxicity, heart failure, hypertension, anemia, leukopenia, neutropenia, thrombocytopenia, demyelination disease, paradoxical reaction,
tuberculosis reactivation, lymphomas, reactivation of hepatitis B, psoriasis, lupus-like syndrome, vitiligo |
| IVIG | Anti-inflammatory; anti-idiotypic mechanisms | Yes, maintenance therapy | headache, fever, chills, and fatigue |
| Plasmapheresis | ↓circulating monoclonal paraprotein | Temporary | hypocalcemia or hypomagnesemia, hypothermia, transfusion reactions, fluid and electrolyte imbalance, bleeding diatheses due to hypofibrinogenemia and thrombocytopenia |
Hematological intervention | |||
| Melphalan/prednisone | ↓ plasma cell clone | Yes, in setting of myeloma, plasma cell leukemia | nausea, vomiting, diarrhea, alopecia, pruritus, bone marrow suppression, oral ulceration, rash and hypersensitivity reactions |
References
- ↑ Druey KM, Greipp PR (2010). "Narrative review: the systemic capillary leak syndrome". Ann Intern Med. 153 (2): 90–8. doi:10.7326/0003-4819-153-2-201007200-00005. PMC 3017349. PMID 20643990.