Handling shock in idiopathic systemic capillary leak syndrome (Clarkson’s disease): less is more

Abstract

Idiopathic systemic capillary leak syndrome (ISCLS) presents with recurrent potentially life-threatening episodes of hypovolemic shock associated with severe hemoconcentration and hypoproteinemia. Timely recognition is of paramount importance because ISCLS, despite resembling other kinds of hypovolemic shock, requires a peculiar approach, to prevent life-threatening iatrogenic damage. Due to the rarity of this condition with only scattered cases described worldwide, evidence-based recommendations are still lacking. Here, we summarize our 40 years’ experience in treating shock in ISCLS patients to derive a therapeutic algorithm. Records from 12 ISCLS patients (mean follow-up is 6 years, with a mean age at symptoms’ onset of 51.5 years) were informative for treatment modalities and outcome of 66 episodes of shock. Episodes are divided in three phases and treatment recommendations are the following: prodromal symptoms-signs (growing malaise, oligo-anuria, orthostatic dizziness) last 6–12 h and patients should maintain rigorous bed rest. The acute shock phase lasts 24–36 h. Patients should be admitted to ICU, placed on restrictive infusion of fluids favoring cautious boluses of high-molecular-weight plasma expanders when SAP < 70 mmHg; monitored for cerebral/cardiac perfusion, myocardial edema and signs of compartment syndrome. The post-acute (recovery) phase may last from 48 h to 1 week. Monitor for cardiac overload to prevent cardiac failure; in case of persistent renal failure, hemodialysis may be necessary; consider albumin infusion. Complications listed by frequency in our patients were acute renal failure, compartment syndrome and neuropathy, rhabdomyolysis, myocardial edema, pericardial–pleural–abdominal effusion, cerebral involvement, acute pulmonary edema and deep vein thrombosis.

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Acknowledgements

We thank Dr. Andrea Zanichelli and Dr. Antonio Castelli, whose support was precious to deal with challenging clinical pictures. We express our gratitude to Dr. Chiara Suffritti for assistance with analyses of blood samples and to Dr. Guido Di Stefano for his helpful contribution for data collection. We wish to acknowledge the help received from Dr. Andrea Zancanaro, who provided data about a patient followed in his Center.

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Correspondence to Maddalena Alessandra Wu.

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Wu, M.A., Colombo, R., Podda, G.M. et al. Handling shock in idiopathic systemic capillary leak syndrome (Clarkson’s disease): less is more. Intern Emerg Med 14, 723–730 (2019). https://doi.org/10.1007/s11739-019-02113-4

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Keywords

  • Idiopathic systemic capillary leak syndrome
  • Clarkson’s disease
  • Shock
  • Diagnosis
  • Treatment