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

Riccardo Colombo, Jonathan Montomoli, Teresa Lanzi, Antonella Tosoni, Claudia Agabiti Rosei, Giuseppe Visani, Franco Verlicchi, Chiara Cogliati, Manuela Nebuloni, Maddalena Alessandra Wu
Author Information
  1. Riccardo Colombo: Division of Anesthesiology and Intensive Care, ASST Fatebenefratelli Sacco, Luigi Sacco Hospital, University of Milan, Milan, Italy. ORCID
  2. Jonathan Montomoli: Division of Anesthesiology and Intensive Care, Infermi Hospital, Romagna Local Health Authority, Rimini, Italy. ORCID
  3. Teresa Lanzi: Division of Internal Medicine, ASST Fatebenefratelli Sacco, Luigi Sacco Hospital, University of Milan, Via G.B. Grassi 74, 20157, Milan, Italy.
  4. Antonella Tosoni: Pathology Unit, ASST Fatebenefratelli Sacco, Luigi Sacco Hospital, University of Milan, Milan, Italy.
  5. Claudia Agabiti Rosei: UOC 2 Medicine, ASST Spedali Civili Di Brescia, Brescia, Italy. ORCID
  6. Giuseppe Visani: Division of Hematology and Stem Cell Transplantation, AST 1, Pesaro, Italy.
  7. Franco Verlicchi: Transfusion Medicine Faenza-Lugo, Transfusion Service Ravenna, Romagna Health Unit, Ravenna, Italy. ORCID
  8. Chiara Cogliati: Division of Internal Medicine, ASST Fatebenefratelli Sacco, Luigi Sacco Hospital, University of Milan, Via G.B. Grassi 74, 20157, Milan, Italy. ORCID
  9. Manuela Nebuloni: Pathology Unit, ASST Fatebenefratelli Sacco, Luigi Sacco Hospital, University of Milan, Milan, Italy. ORCID
  10. Maddalena Alessandra Wu: Division of Internal Medicine, ASST Fatebenefratelli Sacco, Luigi Sacco Hospital, University of Milan, Via G.B. Grassi 74, 20157, Milan, Italy. maddalena.wu@unimi.it. ORCID

Abstract

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.

Keywords

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Word Cloud

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