Pediatric idiopathic systemic capillary leak syndrome: a case report of long-term successful prophylactic therapy with intravenous immunoglobulins
Year: 2026
Type: article
Study design: Case Report
Abstract: Idiopathic Systemic Capillary Leak Syndrome (ISCLS) is a life-threatening condition characterized by recurrent episodes of peripheral edema, hemoconcentration, hypoalbuminemia, hypovolemic shock and tissue hypoperfusion, due to a sudden increase in vascular permeability. ISCLS is exceedingly rare in children, with around 40 cases reported to date, and its clinical presentation overlaps significantly with septic shock, leading to frequent diagnostic delays. Although intravenous immunoglobulins (IVIG) have proved effective both in the acute phase and as preventive treatment in adults, pediatric experience with IVIG prophylaxis remains very limited. A 6-year-old girl presented with recurrent episodes of fever, generalized edema, hypotension, rhabdomyolysis and hypoalbuminemia, all but one triggered by viral infections, fulfilling the diagnostic criteria for ISCLS. Prompt therapy with high-dose IVIG (2 g/kg) achieved clinical and hemodynamic improvement. After the failure of colchicine treatment, monthly IVIG prophylaxis (1 g/kg) was initiated, with an excellent clinical response: only three further episodes occurred over the following four and a half years, none of them in the last three years. The cytokine profiling during a classic flare showed a marked increase in IL-8, sIL-2Rα, TNF-α and IL-10 values, although with different kinetics. Serial cytokine measurements throughout the IVIG prophylaxis period demonstrated progressive and sustained normalization. The girl remains on IVIG prophylaxis, even though the interval between administrations was progressively extended; she is doing well, has never experienced side effects from the therapy, and her growth is excellent. Our experience proves that monthly IVIG prophylaxis at 1 g/kg can achieve sustained, long-term remission in pediatric ISCLS, with an excellent safety profile. To the best of our knowledge, with a follow-up of four and a half years, this is the longest observation period of IVIG prophylaxis in pediatric ISCLS reported in the literature. The optimal duration of IVIG prophylaxis in children remains to be established: the rarity of the condition, limited knowledge of the natural history of the disease in childhood and lack of controlled observational studies with adequate sample sizes constitute the main obstacles to answering this question. Since the possibility of an evolution towards spontaneous healing cannot be excluded, a gradual tapering approach was preferred to an abrupt discontinuation.
Source: BMC Pediatrics
Institutions: Policlinico San Matteo Fondazione, University of Pavia
Language: English
Subfield: Immunology
Field: Immunology and Microbiology
Domain: Life Sciences
Open Access status: gold
Estimated APC paid: $3,090
OpenAlex ID: W7221821073
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