Zusammenfassung
Eine Augenbeteiligung findet sich bei bis zu einem Drittel der Sarkoidosepatienten, meist im Frühstadium der Erkrankung, sodass die zugrunde liegende Systemerkrankung oft noch nicht bekannt ist. Wegen der therapeutischen Konsequenzen ist eine frühzeitige Sicherung der zugrunde liegenden systemischen Grunderkrankung anzustreben. Bei Visusbeeinträchtigung sind invasive Maßnahmen wie eine bronchoalveoläre Lavage oder ultraschallgesteuerte transbronchiale Lungenbiopsie gerechtfertigt, wenn noduläre konjunktivale oder kutane Veränderungen nicht vorliegen, an denen die Diagnose gesichert werden könnte. Ausreichend sensitive Biomarker der Krankheitsaktivität existieren nicht. Eine erhöhte Aktivität des Angiotensin-Converting-Enzyms (ACE) und Lysozyms untermauern die Diagnose und sind für die Beurteilung der Krankheitsaktivität im Verlauf geeignet, jedoch nicht diagnosespezifisch. Bei Patienten mit neu aufgetretener Uveitis ohne bekannte Systemerkrankung sollten speckige, granulomatöse Hornhautbeschläge bei anteriorer Uveitis, „snow balls“ und eine Glaskörperbasisverdichtung mit einer Periphlebitis retinae bei intermediärer Uveitis sowie multifokale cremige Aderhautläsionen von etwa 500 µm Größe in verschiedenen Aktivitätsstadien an die Diagnose einer Sarkoidose denken und eine Tuberkulose ausschließen lassen. Neben erhöhten ACE- und Lysozymspiegeln werden eine Kalzämie und Kalziurie, eine kutane Anergie sowie auffällige Leberenzymwerte und typische hiläre Lungenveränderungen als diagnostische Kriterien genutzt. Unbehandelt kann die Erkrankung bei chronischem Verlauf zu schwerem permanentem Sehverlust führen. Die anteriore Uveitis lässt sich in der Regel durch topische Steroide gut kontrollieren und ist selbstlimitierend, wohingegen die intermediäre, posteriore und Panuveitis typischerweise im Zusammenhang mit einem chronischen Verlauf stehen und eine systemische Therapie erfordern. Zunehmend früher werden bei nicht ausreichendem Ansprechen auf Steroide zur Vermeidung bleibender Organschäden immunmodulierende und Biologikatherapien mit hervorragenden Kurz- und Langzeitergebnissen selbst bei therapierefraktären Fällen eingesetzt.
Abstract
Ocular involvement in sarcoidosis is present in up to one third of patients and is frequently manifested before the underlying systemic disease has been diagnosed. With a view to the therapeutic consequences an early diagnosis of the underlying disease is advantageous. In cases of visual loss early diagnostic measures include invasive procedures, such as ultrasound-guided bronchoalveolar lavage and transbronchial biopsy, if nodular conjunctival or cutaneous manifestations which would confirm the diagnosis are not present. Sufficiently sensitive biomarkers of disease activity have not yet been identified. As elevated activity of angiotensin-converting enzyme (ACE) and lysozyme are associated with disease activity they may be supportive of but not specific for the diagnosis. Other indicators include calcemia, calciuria, augmented activity of hepatic enzymes, cutaneous anergy and hilar changes in the lungs. In patients with newly diagnosed anterior uveitis presenting with granulomatous mutton-fat corneal precipitates, snow balls and vitreous body base condensates with mild retinal periphlebitis in intermediate uveitis as well as multifocal choroiditis (creamy choroidal lesions at different stages of activity and approximately 500 µm in diameter), a diagnosis of sarcoidosis has to be considered and tuberculosis should be excluded, even in the absence of a known systemic disease. Anterior uveitis is frequently a self-limiting condition, which can be well-controlled with topical steroids. On the other hand, intermediate, posterior and panuveitis are typically associated with a chronic course and require systemic therapy to prevent severe loss of vision. If the response to systemic corticosteroids is insufficient, treatment with immunomodulatory agents and biologics is initiated at progressively earlier stages, with a view to averting permanent organ damage and frequently with a positive impact on the short and long-term outcomes, even in therapy-refractive cases.
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J.G. Garweg erhält Beraterhonorare für verschiedene pharmazeutische Konzerne (Alcon, Allergan, Novartis, Bayer) und nimmt als Principal Investigator an verschiedenen internationalen und nationalen, teils durch die Pharmaindustrie gesponserten Studien teil. Die hier präsentierte Arbeit hat nichts mit dieser Tätigkeit zu tun, ein Interessenkonflikt besteht nicht.
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Garweg, J.G. Sarkoidose und Uveitis. Ophthalmologe 114, 525–533 (2017). https://doi.org/10.1007/s00347-016-0405-7
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DOI: https://doi.org/10.1007/s00347-016-0405-7