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Diagnostik und Therapie der AA-Amyloidose

Diagnostics and therapy of AA amyloidosis

  • Schwerpunkt: Amyloid und Amyloidosen
  • Published:
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Zusammenfassung

Die AA-Amyloidose kann bei jeder chronisch-entzündlichen Erkrankung nach einigen Jahren auftreten. Zu den häufigsten Grunderkrankungen gehören Krankheiten des rheumatischen Formenkreises, chronisch-entzündliche Lungen- und Darmerkrankungen, das familiäre Mittelmeerfieber und andere seltene periodische Syndrome. Die AA-Amyloidose betrifft häufig Nieren, Gastrointestinaltrakt und das Herz. Eine effiziente Therapie der Grunderkrankung kann zu einer Normalisierung der Entzündungsreaktion führen und die Progression der Erkrankung verlangsamen oder verhindern, falls die Diagnose frühzeitig gestellt wird. Bei entzündlich-rheumatischen Erkrankungen oder periodischen Syndromen ist hierfür häufig eine Therapie mit Antikörpern gegen TNF-α oder gegen IL-1β erforderlich. Bei Patienten mit einem familiären Mittelmeerfieber kann eine Therapie mit Colchicum Schübe verhindern und das Risiko für die Entstehung und Progression einer AA-Amyloidose deutlich reduzieren. Die Therapie der AA-Amyloidose der Niere mit Eprodisat wird gegenwärtig in Studien untersucht.

Abstract

AA amyloidosis can be the consequence of any chronic inflammatory disorder. It is most commonly associated with chronic inflammatory rheumatic, pulmonary or gastrointestinal diseases, familial Mediterranean fever or other rare periodic syndromes. AA amyloidosis often affects the kidneys, gastrointestinal tract and the heart. Effective therapy of the underlying disease can normalize the inflammatory reaction and can slow or inhibit the deterioration of organ function if the diagnosis is made at an early stage of the disease. In rheumatoid diseases and in some periodic syndromes the use of antibodies against TNFα or IL-1β might be helpful. Patients with familial Mediterranean fever should regularly take colchicine to prevent attacks and to reduce the risk for development or progression of AA amyloidosis. Eprodisate is currently being investigated for AA amyloidosis and renal involvement.

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Abbreviations

AEF:

„Amyloid-enhancing factor“

CRP:

C-reaktives Protein

IL-1β:

Interleukin-1 beta

NT-Pro-BNP:

N-terminales pro-natriumuretisches Peptid

RA:

Rheumatoide Arthritis

RAGE:

„Receptor of advanced glycation end products“

SAA:

Serum-Amyloid A

TNF-α:

Tumor-Nekrose-Faktor alpha

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Interessenkonflikt

Der korrespondierende Autor weist auf folgende Beziehungen hin: N.B. und H.M.L. erhielten Reisekostenübernahmen und Vortragshonorare von Abbott, Actelion, Essex, MSD, Novartis, Roche, UCB und Wyeth im Wert von jeweils weniger als 3000 EUR.

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  1. Medizinische Klinik V, Sektion Rheumatologie, Universitätsklinikum Heidelberg, Im Neuenheimer Feld 410, 69120, Heidelberg, Deutschland

    N. Blank & H.M. Lorenz

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  1. N. Blank
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  2. H.M. Lorenz
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Correspondence to N. Blank.

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Blank, N., Lorenz, H. Diagnostik und Therapie der AA-Amyloidose. Pathologe 30, 219–225 (2009). https://doi.org/10.1007/s00292-009-1140-5

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