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Idiopathisches systemisches Capillary-leak-Syndrom (Clarkson-Syndrom)

Eine seltene Ursache rezidivierender, vital bedrohlicher Ödeme

Idiopathic systemic capillary leak syndrome (Clarkson disease)

A rare cause of recurrent life-threatening edema

  • Seltene Erkrankungen
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Zusammenfassung

Der vorliegende Fallbericht beschreibt das episodische Auftreten schwerster generalisierter Ödeme bei einer jungen Patientin, die im Rahmen dieser akuten Krankheitsschübe Hypotonien mit einer massiven Hämokonzentration (Hämatokrit > 50 %, Hämoglobin > 20 g/dl) und Hypalbuminämien entwickelte. Nachdem die ersten beiden Krankheitsexazerbationen überstanden waren, fand sich eine vollständige Rückbildung der beschriebenen Befunde. Nach einem längeren symptomfreien Intervall kam es zu einer erneuten Exazerbation, in deren Folge durch die schwere Hypotonie und massiven Ödeme kritische Organischämien auftraten. Trotz aller Therapiemaßnahmen entwickelte sich unter anderem ein schweres Kompartmentsyndrom der unteren Extremitäten mit konsekutiver Rhabdomyolyse. Die Patientin verstarb letztlich an den Folgen des therapierefraktären Herz-Kreislauf-Versagens. Das vorliegende idiopathische systemische Capillary-leak-Syndrom (SCLS, auch als Clarkson-Syndrom bezeichnet) ist eine sehr seltene und potenziell lebensbedrohliche Erkrankung mit hoher Mortalität. Weltweit sind seit der Erstbeschreibung der Erkrankung etwa 500 Fälle publiziert worden. Die Pathophysiologie dieser Erkrankung bleibt trotz der bisherigen Erklärungsansätze weiterhin unklar. Regulationsprozesse der endothelialen Permeabilität scheinen maßgeblich gestört zu sein. Auffällig oft haben die betroffenen Patienten eine monoklonale Gammopathie unbestimmter Signifikanz. Das Wissen um die begrenzten Behandlungsmöglichkeiten ist jedoch für die Prognose und das Gesamtüberleben der Patienten von fundamentaler Bedeutung.

Abstract

This case report describes the episodic occurrence of severe generalized edema in a young female patient, who developed hypertension with a massive hemoconcentration (hematocrit >0.5, hemoglobin >20g/dl) and hypoalbuminemia during the course of these acute disease phases. After the first two disease exacerbations were overcome, there was a complete regression of symptoms. After a long symptom-free interval, a new exacerbation occurred as a result of which critical organ ischemia occurred due to the severe hypotension and massive edema. Despite all treatment measures a severe compartment syndrome of the lower extemities with subsequent rhabdomyelosis developed. The patient ultimately died as a result of treatment-refractory cardiovascular failure. The idiopathic systemic capillary leak syndrome (SCLS, also known as Clarkson disease) is a rare and potentially life-threatening disease with a high mortality. Since the first description of the disease only approximately 500 cases have been published worldwide. The pathophysiology of this disease remains unclear despite all previous attempts at clarification. Regulation processes of endothelial permeability seem to be essentially disturbed. Affected patients have a monoclonal gammopathy of undetermined signficance conspicuously often; however, the knowledge of the limited treatment options is of fundamental importance for the prognosis and overall survival of patients

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  1. Vivantes Humboldt Klinikum Berlin, Am Nordgraben 2, 13509, Berlin Reinickendorf, Deutschland

    S. Dasdelen

  2. Helios Universitätsklinikum Wuppertal, Wuppertal, Deutschland

    S.-O. Grebe

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S. Dasdelen und S.-O. Grebe geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren. Alle Patienten, die über Bildmaterial oder anderweitige Angaben innerhalb des Manuskripts zu identifizieren sind, haben hierzu ihre schriftliche Einwilligung gegeben.

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Dasdelen, S., Grebe, SO. Idiopathisches systemisches Capillary-leak-Syndrom (Clarkson-Syndrom). Internist 59, 725–735 (2018). https://doi.org/10.1007/s00108-018-0409-2

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