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Morbidität und Mortalität beim Cushing-Syndrom

Morbidity and mortality in Cushing’s syndrome

  • Schwerpunkt: Nebennierenerkrankungen
  • Published:
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Zusammenfassung

Das endogene Cushing-Syndrom ist eine seltene, unbehandelt tödlich verlaufende endokrine Erkrankung. Es lässt sich in Adrenocorticotropin(ACTH)-abhängige (zentrales und ektopes Cushing-Syndrom) sowie ACTH-unabhängige Subtypen (einseitige oder beidseitige Nebennierenadenome) einteilen. Das klinische Erscheinungsbild der Patienten umfasst typische körperliche Stigmata des Kortisolexzesses mit Symptomen der katabolen Stoffwechsellage (Myopathie, Striae, Pergamenthaut, Osteoporose) und Komponenten des metabolischen Syndroms (Diabetes mellitus, Adipositas, arterielle Hypertonie, Hypercholesterinämie). Die biochemische Diagnostik erfolgt in drei Schritten: 1. Sicherung der biochemischen Diagnose mittels 1 mg-Dexamethason-Hemmtest, Kortisolbestimmung im 24 h-Sammelurin und Messung des spätabendlichen Speichelkortisols. 2. Unterscheidung des ACTH-abhängigen Cushing-Syndroms vom ACTH-unabhängigen adrenalen Cushing-Syndrom durch Messung des Plasma-ACTH. 3. Weitere Subdifferenzierung durch den Kortikotropin-Releasing-Hormon-Test, Sinus-petrosus-Katheter sowie weitere bildgebende Verfahren. Therapieentscheidungen werden interdisziplinär getroffen. Erstlinientherapie bei allen Subtypen ist, wenn möglich, die Operation; als weitere Optionen stehen bei allen Formen zudem die medikamentöse Therapie und die bilaterale Adrenalektomie zur Verfügung. Trotz adäquater Behandlung ist die Erkrankung mit einer langfristig erhöhten Morbidität und Mortalität assoziiert. Ein interdisziplinäres und multimodales Therapiemanagement ist langfristig erforderlich, um die erhöhte Mortalität und eingeschränkte Lebensqualität positiv zu beeinflussen.

Abstract

Endogenous Cushing’s syndrome is a rare endocrine disorder that is fatal if left untreated. It can be distinguished into adrenocorticotropic hormone (ACTH)-dependent (central and ectopic Cushing’s syndrome) and ACTH-independent subtypes (unilateral or bilateral adrenal adenomas). The clinical presentation of patients includes typical stigmata of cortisol excess with physical symptoms of catabolic metabolism (myopathy, striae, parchment skin, osteoporosis) and components of metabolic syndrome (diabetes mellitus, obesity, arterial hypertension, hypercholesterolemia). Biochemical diagnosis is performed in three steps: 1. Confirmation of the diagnosis by 1‑mg dexamethasone suppression test, 24‑h urine free cortisol, and measurement of late-night salivary cortisol. 2. Differentiation of ACTH-dependent Cushing’s syndrome from ACTH-independent adrenal Cushing’s syndrome by measurement of plasma ACTH. 3. Further subtyping by corticotropin-releasing hormone (CRH) test, inferior petrosal sinus sampling, and imaging modalities. Therapeutic decisions are made on an interdisciplinary basis. First-line therapy for all subtypes is surgery when possible; additional options for all forms include drug therapy and bilateral adrenalectomy. Despite adequate treatment, Cushing’s syndrome is associated with increased long-term morbidity and mortality. Interdisciplinary and multimodal therapy management is necessary in the long term to positively influence mortality and reduced quality of life.

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Authors and Affiliations

  1. Medizinische Klinik und Poliklinik IV, LMU Klinikum, Ziemssenstraße 5, 80336, München, Deutschland

    Frederick Vogel, Leah Braun &  Martin Reincke

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  1. Frederick Vogel
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  2. Leah Braun
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  3. Martin Reincke
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Correspondence to Martin Reincke.

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F. Vogel, L. Braun und M. Reincke geben an, dass kein Interessenkonflikt besteht.

Für diesen Beitrag wurden von den Autoren keine Studien an Menschen oder Tieren durchgeführt. Für die aufgeführten Studien gelten die jeweils dort angegebenen ethischen Richtlinien.

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Martin Reincke, München

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Vogel, F., Braun, L. & Reincke, M. Morbidität und Mortalität beim Cushing-Syndrom. Internist 63, 34–42 (2022). https://doi.org/10.1007/s00108-021-01222-7

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  • DOI: https://doi.org/10.1007/s00108-021-01222-7

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