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Seltene Formen der Hypertonie

Vom Phäochromozytom zur Vaskulitis

Rare forms of hypertension

From pheochromocytoma to vasculitis

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Zusammenfassung

Ein kleiner Teil der Patienten mit Hypertonie (< 10 %) weist eine sog. sekundäre Hypertonie auf. Nicht selten ist sie durch renale Ursachen, eine obstruktive Schlafapnoe oder einen Hyperaldosteronismus bedingt. Ausgeschlossen werden müssen in jedem Fall eine medikamenteninduzierte Hypertonie und fehlende Adhärenz in der Medikamenteneinnahme. Alle anderen Formen sind selten und erfordern einen wachen klinischen Blick und die Kenntnis der Differenzialdiagnosen. Die Differenzialdiagnose der sekundären Hypertonieformen muss v. a. bei neu aufgetretenem Hypertonus, Akzeleration einer bereits bekannten Hypertonie oder bei schwieriger medikamentöser Therapie erwogen werden. Unter den seltenen Formen der sekundären Hypertonie stehen endokrine, häufig genetische Ursachen an erster Stelle, z. B. monogenetische tubuläre Formen der Hypertonie und Störungen des Mineralokortikoidstoffwechsels, des Katecholaminstoffwechsels (Phäochromozytom), der Schilddrüse oder, sehr selten, der Reninproduktion (JG-Tumoren). Darüber hinaus können sich entzündliche Gefäßerkrankungen mit einer Hypertonie manifestieren, oft mit einer akzelerierten Form. In der diagnostischen Betreuung dieser Patienten müssen eine klinische Beurteilung mit wachem Auge für konstitutionelle Veränderungen und neu aufgetretene Organschäden sowie eine zielgerichtete Anamnese erfolgen. Insbesondere ein schwerer oder nicht therapierbarer Hypertonus erfordert eine diagnostische Abklärung, zuerst der häufigeren, dann der seltenen Hypertonieformen.

Abstract

Secondary hypertension affects only 5–10 % of hypertensive patients. Screening is expensive and time-consuming and should be performed only in patients for whom there is a high clinical suspicion of secondary hypertension. Clinical signs of secondary forms of hypertension are new-onset hypertension in patients without other risk factors (i.e., family history, obesity, etc.), sudden increase of blood pressure (BP) in a previously stable patient, increased BP in prepubertal children, resistant hypertension, and severe hypertension or hypertensive emergencies. In adults, renal parenchymal and vascular diseases as well as obstructive sleep apnea are the most common causes of secondary hypertension. Medication-induced hypertension and non-adherence to medication have to be ruled out. Of the endocrine causes associated with hypertension, primary aldosteronism is the most common. Other endocrine causes of hypertension such as thyroid disease (hypo- or hyperthyroidism), hypercortisolism (Cushing’s syndrome), hyperparathyroidism, and pheochromocytoma are rare. Monogenetic forms of hypertension are mostly of tubular origin and associated with alterations in mineralocorticoid handling or signaling. Rare causes of hypertension also include inflammatory vascular disease. Acute forms of vasculitis may present as “malignant” hypertension with associated thrombotic microangiopathy and organ damage/failure. It is important to diagnose these rare forms of hypertension in order to prevent acute organ damage in these patients or unnecessary invasive treatment strategies.

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Einhaltung ethischer Richtlinien

Interessenkonflikt. H. Haller, F. Limbourg, B.M. Schmidt und J. Menne geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.

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Authors and Affiliations

  1. Hypertoniezentrum, Klinik für Nieren- und Hochdruckkrankheiten, Medizinische Hochschule Hannover, Carl-Neuberg-Str. 1, 30625, Hannover, Deutschland

    H. Haller, F. Limbourg, B.M. Schmidt & J. Menne

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  1. H. Haller
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  2. F. Limbourg
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Correspondence to H. Haller.

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Haller, H., Limbourg, F., Schmidt, B. et al. Seltene Formen der Hypertonie. Internist 56, 255–262 (2015). https://doi.org/10.1007/s00108-014-3571-1

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  • DOI: https://doi.org/10.1007/s00108-014-3571-1

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