Neue Entwicklungen und Perspektiven in der Akromegalie

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Was ist neu?

Diagnostik Bei Verdacht auf eine Akromegalie wird zunächst das Hormon Insulin-like growth factor 1 (IGF-1) als wesentlicher Mediator des Wachstumshormons (GH) bestimmt. Ist es erhöht, schließt sich eine Bestätigungsdiagnostik mittels GH-Suppressionstest an. Neue Arbeiten empfehlen für diesen Test niedrigere GH-Grenzwerte als früher, zudem sollen potenzielle Einflussgrößen (z. B. Body-Mass-Index) stärker berücksichtigt werden. Perspektivisch könnten Erkrankte mittels einer automatisierten Gesichtserkennung ggf. leichter identifiziert werden.

Komorbiditäten Bei einem unkontrollierten GH-Exzess sind Lebensqualität und -erwartung zum Teil erheblich reduziert. Eine Akromegalie sowie deren typische Folgeerkrankungen (z. B. Schlafapnoe, Kardiomyopathie, Arthropathie) müssen daher frühzeitig erkannt werden. Kürzlich wurden neue Empfehlungen für ein standardisiertes diagnostisches Vorgehen publiziert.

Therapie Die operative Adenomentfernung durch einen erfahrenen Hypophysenchirurgen ist Therapie der Wahl. Bei residueller Erkrankung kann perspektivisch eine Kombination aus volumetrischer Magnetresonanztomografie (MRT) und 11C-Methionin-Positronen-Emissions-Tomografie (PET) eine Folgeoperation erleichtern. Für die typische Zweitlinientherapie mit Somatostatin-Analoga (SSA) ist nun erstmals auch ein oral einzusetzendes Präparat verfügbar. Neue Daten belegen die Wirksamkeit und Sicherheit einer Hypophysenbestrahlung.

Spezielle Patientenpopulationen Schwangere und ältere Patienten bedürfen besonderer Aufmerksamkeit. Gemäß aktueller Daten wirkt sich die COVID-Pandemie auch bei einer Akromegalie nachteilig auf Diagnostik und Therapie aus.

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Most cited references 15

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Acromegaly

Annamaria Colao, Ludovica Grasso, Andrea Giustina … (2019)

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A Consensus on the Diagnosis and Treatment of Acromegaly Comorbidities: An Update

Andrea Giustina, Ariel R Barkan, Albert Beckers … (2019)

The aim of the Acromegaly Consensus Group was to revise and update the consensus on diagnosis and treatment of acromegaly comorbidities last published in 2013. The Consensus Group, convened by 11 Steering Committee members, consisted of 45 experts in the medical and surgical management of acromegaly. The authors received no corporate funding or remuneration. This evidence-based Consensus was developed using the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) system to describe both the strength of recommendations and the quality of evidence following critical discussion of the current literature on the diagnosis and treatment of acromegaly comorbidities. Acromegaly Consensus Group participants conducted comprehensive literature searches for English-language papers on selected topics, reviewed brief presentations on each topic, and discussed current practice and recommendations in breakout groups. Consensus recommendations were developed based on all presentations and discussions. Members of the Scientific Committee graded the quality of the supporting evidence and the consensus recommendations using the GRADE system. Evidence-based approach consensus recommendations address important clinical issues regarding multidisciplinary management of acromegaly-related cardiovascular, endocrine, metabolic, and oncologic comorbidities, sleep apnea, and bone and joint disorders and their sequelae, as well as their effects on quality of life and mortality.

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Prolonged diagnostic delay in acromegaly is associated with increased morbidity and mortality

Daniela Esposito, Oskar Ragnarsson, Gudmundur Johannsson … (2020)

Context Clinical features of acromegaly develop insidiously. Its diagnosis may therefore be delayed. Objective Our aim was to study diagnostic delay and its impact on morbidity and mortality in a nationwide cohort of patients with acromegaly. Design Adult patients diagnosed with acromegaly between 2001 and 2013 were identified in the Swedish National Patient Registry. Diagnostic codes for predefined comorbidities associated with acromegaly were recorded between 1987 and 2013. Diagnostic delay was calculated as the time between the first registered comorbidity and the diagnosis of acromegaly. Results A total of 603 patients (280 men, 323 women) with acromegaly were included. Mean ( s.d. ) diagnostic delay was 5.5 (6.2) years (median (minimum, maximum) 3.3 (0.0–25.9)) Diagnostic delay was 1–<5 years in 23% patients; 5–<10 years in 17%; and ≥10 years in 24%. No delay was recorded in 36% of patients. Overall, mean ( s.d. ) number of comorbidities was 4.1 (2.5) and was higher in patients with longer diagnostic delay ( P < 0.0001). Overall, observed number of deaths was 61 (expected 42.2), resulting in a standardized mortality ratio (SMR) of 1.45 (95% CI: 1.11–1.86). Increased mortality was only found in patients with the longest diagnostic delay (1.76, 95% CI: 1.12–2.65). In the other groups, no statistically significant increase in mortality was recorded, with the numerically lowest SMR observed in patients without diagnostic delay (1.18; 95% CI: 0.68–1.92). Conclusions The diagnosis of acromegaly is delayed in most patients. Prolonged diagnostic delay is associated with increased morbidity and mortality.

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Author and article information

Journal

Title: DMW - Deutsche Medizinische Wochenschrift

Abbreviated Title: Dtsch Med Wochenschr

Publisher: Georg Thieme Verlag KG

ISSN (Print): 0012-0472

ISSN (Electronic): 1439-4413

Publication date Created: August 03 2021

Publication date Created: August 2021

Publication date (Electronic): August 03 2021

Publication date (Print): August 2021

Volume: 146

Issue: 15

Pages: 950-954

Affiliations

[1 ]Universitätsklinikum Würzburg, Medizinische Klinik und Poliklinik I, Lehrstuhl für Endokrinologie und Diabetologie, Würzburg; Medicover Oldenburg MVZ, Oldenburg

Article

DOI: 10.1055/a-1495-2715

SO-VID: 3533157a-8ea1-4cc6-b3a9-d9a9064bda92

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