Update endokrine Orbitopathie

Die endokrine Orbitopathie (EO) ist die häufigste extrathyreoidale Manifestation des Morbus Basedow. Für Therapieentscheidungen wird die Erkrankung in eine aktive und eine inaktive Phase eingeteilt sowie eine Unterscheidung zwischen einer milden, moderaten und schweren visusbedrohenden Manifestation vorgenommen, wobei die Lebensqualität der Patienten bei den beiden letzteren erheblich eingeschränkt ist.

Abstract

Graves’ orbitopathy (GO) is the most common extrathyroideal manifestation of Graves’ disease. Therapy depends on activity (active, inactive) and severity (mild, moderate-to-severe, sight-threatening). Mild cases are treated with supplementation of selenium and a “watchful waiting” strategy. Moderate-to-severe cases show a significant decrease in the patients’ quality of life and have to be treated if active with anti-inflammatory therapies. First choice in these cases are intravenous steroids (cumulative 4 – 5 g) in combination with orbital irradiation. Non-responders after 6 weeks should be treated with additional immunomodulatory drugs. In patients with sight-threatening GO mostly require orbital decompression. Patients with inactive disease can undergo rehabilitative surgery to improve their quality of life. Currently, new therapeutic approaches derived from results of basic research are being investigated in clinical trials and might improve the therapy of GO patients in the near future: At present, teprotumumab is undergoing a phase III clinical trial. Furthermore, the blockage of TSH-receptors with specific antibodies is being investigated in a phase I trial and “small molecule like TSHR antagonists” are being evaluated in “in vivo” models.

Kernaussagen

• Die endokrine Orbitopathie (EO) ist die häufigste extrathyreoidale Manifestation des Morbus Basedow.

• Für Therapieentscheidungen wird die Erkrankung in eine aktive und eine inaktive Phase eingeteilt und eine Unterscheidung zwischen einer milden, moderaten und schweren visusbedrohenden Manifestation vorgenommen.

• Milde Form:

  • Bei der milden Form der Erkrankung kann der Spontanverlauf unter Selen-Supplementation abgewartet werden.

• Moderate und schwere Manifestationen:

  • Die Lebensqualität der Patienten ist erheblich beeinträchtigt.

  • In der aktiven Phase der Erkrankung ist bei diesen Patienten eine antientzündliche Therapie angezeigt.

    • Die Primärtherapie besteht aktuell noch aus einer i. v.-Steroidstoßtherapie (kumulativ 4 – 5 g) in Kombination mit einer Orbitaspitzenbestrahlung bei Motilitätsstörungen.

    • 6 Wochen nach Beginn der i. v.-Steroidtherapie sollte bei ungenügendem Ansprechen eine immunsuppressive Therapie hinzugefügt werden.

    • Bei schwerer visusbedrohender Manifestation ist meist eine knöcherne Orbitadekompression die Therapie der Wahl.

  • Im inaktiven Krankheitsstadium kann Patienten mit bleibenden Defekten mit einer Reihe von ophthalmochirurgischen Maßnahmen sehr gut geholfen werden.

Schlüsselwörter

Morbus Basedow - immunologisch bedingte Entzündung - Orbita

Keywords

Graves’ orbitopathy - Basedow’s disease - immunological inflammation - orbita

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