P150 | Idiopathic systemic capillary leak syndrome: insights from a comprehensive patient cohort
Year: 2025
Type: article
Study design: Observational Study
Abstract: 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.
Source: Italian Journal of Medicine
Authors: Vo Van Kha, Tran Thi Huong Ly, Pham Thi Thanh Hoa, Vo Huynh Nhu, Damas Hendriansyah, Nusratuddin Abdullah, Rudy B. Leonardy, Mardiah Tahir, Samrichard Rambulangi, Firdaus Hamid, Filomena Pietrantonio, Vera Kopsaj, Francesco Rosiello, Aurora Tomassoni, Luca Moriconi, Donatella Padua, Kiki Rizki Amelia, Efendi Lukas, Nurbani Bangsawan, Isharyah Sunarno, Retno Budiarti Farid, Sriwijaya Qadar, Rabab H. Elshaikh, Ashok Kumar Sah, Abdelrahman Mohamed Abukanna, Abdulrahman Algarni, Ammar Abdelmola, Hussam Ali Osman, Tagwa Yousif Elsayed Yousif, Elryah I. Ali, Hiba Ali, Ayman Hussein Alfeel, Thanh Long Nguyen, Victor Storm, Lam Toi Phung, Cong Thien Le, Thi Thu Ha Le, Thi Hoa Nguyen, Son Tung Vu, Thien Thang Tran, Minh Hanh Vu, Van Tuan Nguyen, Francesco Ventrella, Anna Belfiore, Antonio Greco, Franco Mastroianni, Salvatore Lenti, Domenico Ruggiero, Michele Cannone, Vincenzo Ostilio Palmieri, Massimo Errico, Giuseppe Mancia, Guido Grassi, Sverre E. Kjeldsen
Language: English
Subfield: Epidemiology
Field: Medicine
Domain: Health Sciences
Open Access status: diamond
OpenAlex ID: W4413770794