P150 | Idiopathic systemic capillary leak syndrome: insights from a comprehensive patient cohort

Background: Idiopathic systemic capillary leak syndrome (ISCLS), also known as Clarkson’s disease, is a rare life-threatening condition characterized by recurrent shock episodes stemming from endothelial barrier dysfunction. Despite similarities to other shock states, ISCLS requires distinct management strategies. This study aims to elucidate the clinical presentation, course, and management of critically ill ISCLS patients in Italy.Materials and Methods: Case series collected at the Luigi Sacco Hospital in Milan, a referral center for ISCLS.Results: Data from 16 patients (50% female) with 29 episodes were collected over 20 years. The mean age at diagnosis was 52 years (range 27-69). The crises were triggered by an upper respiratory infection in 19 episodes, of whom 6 were due to SARS-CoV-2 infection. Twelve patients had monoclonal gammopathy, mostly IgG kappa type. At ICU admission, patients were hypotensive with hemoconcentration and hypoalbuminemia: mean systolic arterial pressure was 84.7 mmHg (range 60-110), heart rate 115 bpm (range 70-150), hematocrit 57.8% (range 35-77), and serum albumin 2.5 gr/dL (range 0.6-3.4). The mean ICU stay was 6.7 days (range 1-27). Five patients died, all of them during episodes triggered by SARS-CoV-2.Conclusions: Our study provides a detailed description of the unique features of ISCLS, a frequently misdiagnosed fatal condition whose timely recognition is crucial for prompt appropriate management. Our data highlight the detrimental impact of infection by SARS-CoV-2 on patient outcomes.