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Hypertrophe Osteoarthropathie

Marie-Bamberger-Syndrom

Hypertrophic osteoarthropathy

Bamberger-Marie disease

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Zusammenfassung

Die hypertrophe Osteoarthropathie (HOA) ist die klassische paraneoplastische Erkrankung in der Rheumatologie, charakterisiert durch die Trias aus Trommelschlegelfingern mit Uhrglasnägeln, Gelenk- und Knochenschmerzen sowie proliferativer Periostitis. Diese Konstellation sollte Anlass zu einer intensiven Tumorsuche sein, wobei in der Regel intrathorakale Malignome zugrunde liegen. Wir berichten hier über einen Patienten mit HOA im Rahmen eines neuroendokrinen Ösophaguskarzinoms. Differenzialdiagnostisch kommen eine Reihe anderer nichtmaligner Erkrankungen der Lunge, des Herzens oder anderer Organe in Frage. Selten tritt auch eine primäre, familiäre Form der HOA auf, deren genetischer Hintergrund vor Kurzem entschlüsselt wurde. Neue Erkenntnisse zur Pathophysiologie dieser Erkrankung haben auch zu einer Erweiterung der diagnostischen und therapeutischen Möglichkeiten geführt.

Abstract

Hypertrophic osteoarthropathy (HOA) is the classical neoplastic disease in rheumatology characterized by a combination of digital clubbing, joint and bone pain, and proliferative periostitis. This combination of symptoms should initiate an intensive search for an underlying malignant disease usually of thoracic organs. Here we report the case of a patient with HOA and neuroendocrine carcinoma of the esophagus. Other non-malignant disorders of the lungs, heart and other organs should be considered in the differential diagnosis. In addition, rare cases of a primary hereditary form of HOA exist and the genetic background has recently been discovered. Thus, new insights into the pathophysiology have improved diagnostic and therapeutic options for this disorder.

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Manger, B., Lindner, A., Manger, K. et al. Hypertrophe Osteoarthropathie. Z. Rheumatol. 70, 554–560 (2011). https://doi.org/10.1007/s00393-011-0813-7

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