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Ursachen und Therapie der systemischen AA-Amyloidose

Causes and treatment of systemic amyloidosis

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Zusammenfassung

Hintergrund

Systemische Amyloidosen sind seltene Proteinablagerungs-Erkrankungen, die aufgrund der anfangs unspezifischen Symptomatik häufig (zu) spät diagnostiziert werden. Die Unterteilung der Amyloidosen erfolgt nach dem jeweiligen Protein, das sich fehlfaltet und ablagert. Jede chronische Entzündung kann zur Entstehung einer Amyloid-A- (AA) Amyloidose führen.

Ziel

Mit dieser Arbeit möchten wir den aktuellen Kenntnisstand zur Diagnose und Therapie der AA-Amyloidose und Daten der vergangenen 10 Jahre aus unserem Amyloidosezentrum darstellen.

Material und Methoden

Unsere Daten basieren auf einer Auswertung unseres eigenen Patientenkollektivs sowie einer selektiven Literaturrecherche in der PubMed Datenbank zum Thema AA-Amyloidose.

Ergebnisse

Zu den Ursachen einer AA-Amyloidose zählen autoinflammatorische Syndrome, Polyarthritiden und chronisch entzündliche Darm- und Lungenerkrankungen. Die Nierenbeteiligung steht fast immer im Vordergrund und kann durch die Abklärung einer Proteinurie frühzeitig entdeckt werden. Die Therapie richtet sich nach der jeweiligen Grunderkrankung. Bei Patienten ohne erkennbare Grunderkrankung wird eine idiopathische AA-Amyloidose diagnostiziert, die Therapie erfolgt empirisch.

Diskussion

Durch die Fortschritte in Diagnostik und Therapie hat sich die Prognose dieser schwerwiegenden Erkrankung verbessert, jedoch spielt die Frühdiagnose weiterhin eine wichtige Rolle, um eine Dialyse-pflichtige Niereninsuffizienz zu verhindern. Neue Therapieansätze haben zum Ziel, vorhandene Amyloid-Ablagerungen abzubauen.

Abstract

Background

Systemic amyloidoses are rare protein deposition disorders, which are often diagnosed in an advanced stage of the disease due to non-specific symptoms. Any chronic inflammatory disease can lead to an AA-type amyloidosis.

Aim

This paper summarizes the current state of the art of diagnosis and treatment of AA amyloidosis and presents data from the past 10 years of our amyloidosis center.

Material and Methods

Our data represents an analysis of our cohort of patients with amyloidosis and a selective research in the PubMed database for AA amyloidosis.

Results

The underlying diseases comprise autoinflammatory syndromes, polyarthritis, and chronic inflammatory bowel and lung diseases. Renal organ involvement is the most prevalent in AA amyloidosis. It can be detected early through the evaluation of proteinuria. The treatment depends on the individual underlying disease. Patients without an associated inflammatory disease are considered to have idiopathic AA amyloidosis and empiric treatment is mandatory.

Discussion

Survival of this fatal disease has recently improved due to the new diagnostic tools and treatment options; however, early diagnosis plays a crucial role in the prevention of end-stage renal failure. New therapeutic strategies aim to remove existing amyloid deposits.

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Authors and Affiliations

  1. Amyloidose-Zentrum, Universitätsklinikum Heidelberg, Im Neuenheimer Feld 410, 69120, Heidelberg, Deutschland

    N. Blank, U. Hegenbart & S. Schönland

Authors
  1. N. Blank
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  2. U. Hegenbart
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  3. S. Schönland
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Corresponding author

Correspondence to N. Blank.

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Interessenkonflikt

N. Blank erhielt Vortragshonorare von den Firmen SOBI, Novartis, Roche, MSD, Abbott und UCB. U. Hegenbart erhielt Vortragshonorare von den Firmen Janssen und Celgene. S. Schönland erhielt Vortragshonorare und Forschungsunterstützung von den Firmen Janssen und Celgene.

Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.

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Redaktion

B. Manger, Erlangen

B. Swoboda, Erlangen

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Blank, N., Hegenbart, U. & Schönland, S. Ursachen und Therapie der systemischen AA-Amyloidose. Z Rheumatol 75, 141–150 (2016). https://doi.org/10.1007/s00393-015-0024-8

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  • DOI: https://doi.org/10.1007/s00393-015-0024-8

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