Dtsch Med Wochenschr 2006; 131: S45-S66
DOI: 10.1055/s-2006-947836
Konsensus | Review article

© Georg Thieme Verlag KG Stuttgart · New York

Interdisziplinäre Leitlinien zur Diagnostik und Therapie der extrazerebralen Amyloidosen

Herausgegeben von der Deutschen Gesellschaft für Amyloid-Krankheiten e. V. (www.amyloid.de)Interdisciplinary guidelines on diagnosis and treatment for extracerebral amyloidosesPublished by the German Society of Amyloid Diseases (www.amyloid.de)C. Röcken , J. Ernst, E. Hund , H. Michels, J. Perz, W. Saeger, O. Sezer , S. Spuler , F. Willig, H. H.-J Schmidt
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Publication History

Publication Date:
12 July 2006 (online)

Within the past 10 years, a new range of knowledge has been achieved in the field of amyloidosis, especially with regard to pathogenesis, diagnosis and therapy. Amyloidosis leads to variable and distinct symptoms and is caused by different underlying conditions. Some amyloidoses are acquired secondary to a chronic condition; others are caused by genetic mutations. Amyloid and amyloidosis occur more frequently than they are perceived. Among the frequent localized forms are the cerebral amyloidosis linked to Alzheimer disease (AD) and the pancreatic amyloidosis linked to diabetes mellitus. Among the most frequent systemic (extracerebral) forms is AL amyloidosis, which often has a poor prognosis and if untreated can rapidly lead to death. Systemic amyloidosis that happen at infancy are mainly AA amyloidosis that can progress to death already at early or at middle adulthood. Amyloidosis can be treated but therapeutic success significantly depends upon early diagnosis and proper classification of the amyloid type. It is mandatory that differential diagnosis demonstrate the presence of amyloid and clearly identify the type of the disease. Development of methods and techniques have contributed to improvements in the diagnosis and treatment. Early diagnosis and proper classification of amyloid is decisive for therapeutic options and upon them depend quality of life and mortality. The therapeutic spectrum is various and includes organ transplantation, chemotherapy, and anti-inflammatory strategies. Gene therapy and biological active substances have to be considered in the near future.

Literatur

Prof. Dr. med. Christoph Röcken

Institut für Pathologie, Charité - Universitätsmedizin Berlin

Charitéplatz 1

10117 Berlin

Phone: 030/450536115

Fax: 030/450536914

Email: [email protected]