<p>Cushing’s syndrome is a&#xa0;rare but clinically significant condition associated with considerable morbidity and mortality if not promptly recognized and treated. Precise differentiation between pituitary and ectopic adrenocorticotropic hormone (ACTH) production is essential for determining the appropriate treatment approach. When MRI findings are inconclusive, inferior petrosal sinus sampling (IPSS) remains the most sensitive method for localizing the ACTH source. Pharmacological stimulation, typically with ovine CRH (oCRH), is crucial to enhance diagnostic accuracy. However, ongoing supply shortages of oCRH have led to an increased use of unstimulated IPSS procedures, compromising diagnostic reliability and raising the risk of false-negative results. Therefore, alternative stimulants such as desmopressin and recombinant human CRH (hCRH) are gaining importance. Studies have shown that desmopressin can reliably induce a&#xa0;central-to-peripheral ACTH gradient, especially in cases of pituitary microadenomas, and is a&#xa0;cost-effective, widely available alternative. Initial data on hCRH are also promising. Consequently, IPSS without pharmacological stimulation can no longer be recommended. General practitioners play a&#xa0;key role in the early detection of Cushing’s syndrome and should ensure timely referral to an endocrine specialist in cases of clinical suspicion. Diagnostic quality can only be maintained through interdisciplinary collaboration and the use of validated, readily available alternatives in the long term.</p>

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Diagnostik und Therapie des endogenen Hypercortisolismus (Cushing-Syndrom)

  • Lukas Andereggen,
  • Emanuel Christ

摘要

Cushing’s syndrome is a rare but clinically significant condition associated with considerable morbidity and mortality if not promptly recognized and treated. Precise differentiation between pituitary and ectopic adrenocorticotropic hormone (ACTH) production is essential for determining the appropriate treatment approach. When MRI findings are inconclusive, inferior petrosal sinus sampling (IPSS) remains the most sensitive method for localizing the ACTH source. Pharmacological stimulation, typically with ovine CRH (oCRH), is crucial to enhance diagnostic accuracy. However, ongoing supply shortages of oCRH have led to an increased use of unstimulated IPSS procedures, compromising diagnostic reliability and raising the risk of false-negative results. Therefore, alternative stimulants such as desmopressin and recombinant human CRH (hCRH) are gaining importance. Studies have shown that desmopressin can reliably induce a central-to-peripheral ACTH gradient, especially in cases of pituitary microadenomas, and is a cost-effective, widely available alternative. Initial data on hCRH are also promising. Consequently, IPSS without pharmacological stimulation can no longer be recommended. General practitioners play a key role in the early detection of Cushing’s syndrome and should ensure timely referral to an endocrine specialist in cases of clinical suspicion. Diagnostic quality can only be maintained through interdisciplinary collaboration and the use of validated, readily available alternatives in the long term.