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<p>Idiopathic Systemic Capillary Leak Syndrome (ISCLS) is a poorly understood paroxysmal permeability disorder. This study aimed to characterize the clinical features and severity markers of ISCLS in critically and non-critically ill patients. We analyzed prospectively and retrospectively collected data on ISCLS cases from the Italian IRIS-CLS Registry (January 1995–December 2023). A total of 124 acute episodes were identified in 32 patients; 61 episodes in 26 patients having complete records were included in the analysis. Monoclonal gammopathy was found in 20/23 adults but was absent in all three pediatric patients, who exhibited IgA deficiency instead. At admission, patients presented with hypotension (systolic arterial pressure 87&#xa0;mmHg [80–105]), tachycardia (110&#xa0;bpm [96–130]), and hemoconcentration (hematocrit 57% [48–62.7]). ICU admission was required in 70.5%, and cardiac involvement occurred in 49.2% of episodes. Fluid volume infused correlated positively with rhabdomyolysis severity, and the need for renal replacement therapy was more frequent in patients receiving ≥ 3 L. No pharmacologic treatment was effective during the crises. Mortality was 9.8% per episode, and the day-1 Sequential Organ Failure Assessment score was a predictor of death (<i>p</i> = 0.0181). Immunoglobulin prophylaxis reduced attack frequency from 0.67 (0.4–1.07) to 0.4 (0.1–0.52) per year (<i>p</i> = 0.033). Two adults (8.7%) developed multiple myeloma during follow-up. This study provides a comprehensive overview of ISCLS in both critically and non-critically ill patients. We highlight clinical-biochemical-instrumental red-flags and emphasize the need for increased awareness and early recognition since effective treatments for acute shock remain elusive.</p>

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Clarkson disease in critically and non-critically ill patients: insights from the Italian IRIS-CLS registry

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年3月8日

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  • Riccardo Colombo,
  • Jonathan Montomoli,
  • Teresa Lanzi,
  • Antonella Tosoni,
  • Claudia Agabiti Rosei,
  • Giuseppe Visani,
  • Franco Verlicchi,
  • Chiara Cogliati,
  • Manuela Nebuloni,
  • Maddalena Alessandra Wu

摘要

Idiopathic Systemic Capillary Leak Syndrome (ISCLS) is a poorly understood paroxysmal permeability disorder. This study aimed to characterize the clinical features and severity markers of ISCLS in critically and non-critically ill patients. We analyzed prospectively and retrospectively collected data on ISCLS cases from the Italian IRIS-CLS Registry (January 1995–December 2023). A total of 124 acute episodes were identified in 32 patients; 61 episodes in 26 patients having complete records were included in the analysis. Monoclonal gammopathy was found in 20/23 adults but was absent in all three pediatric patients, who exhibited IgA deficiency instead. At admission, patients presented with hypotension (systolic arterial pressure 87 mmHg [80–105]), tachycardia (110 bpm [96–130]), and hemoconcentration (hematocrit 57% [48–62.7]). ICU admission was required in 70.5%, and cardiac involvement occurred in 49.2% of episodes. Fluid volume infused correlated positively with rhabdomyolysis severity, and the need for renal replacement therapy was more frequent in patients receiving ≥ 3 L. No pharmacologic treatment was effective during the crises. Mortality was 9.8% per episode, and the day-1 Sequential Organ Failure Assessment score was a predictor of death (p = 0.0181). Immunoglobulin prophylaxis reduced attack frequency from 0.67 (0.4–1.07) to 0.4 (0.1–0.52) per year (p = 0.033). Two adults (8.7%) developed multiple myeloma during follow-up. This study provides a comprehensive overview of ISCLS in both critically and non-critically ill patients. We highlight clinical-biochemical-instrumental red-flags and emphasize the need for increased awareness and early recognition since effective treatments for acute shock remain elusive.