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Autoantikörperdiagnostik rheumatologischer Systemerkrankungen mit pulmonaler Manifestation

Autoantibody testing in vasculitides and connective tissue diseases with pulmonary manifestations

  • Leitthema
  • Published:
Der Pneumologe Aims and scope

Zusammenfassung

Neben den selteneren ANCA-assoziierten Vaskulitiden (AAV), die sich häufig mit einem pulmorenalen Syndrom und/oder pulmonalen Granulomen manifestieren, stellen die interstitielle Lungenerkrankung und der pulmonale Hypertonus häufigere Komplikationen rheumatologischer Erkrankungen, insbesondere der Kollagenosen dar. Die Bestimmung des ANCA mit Nachweis eines der charakteristischen Zielantigene, Proteinase-3 (PR3) oder Myeloperoxidase (MPO), ist diagnostisch richtungweisend für das Vorliegen einer AAV, während bei dem Verdacht auf eine Kollagenose zunächst antinukleäre Antikörper (ANA) und bei positivem Befund nachfolgend eine ANA-Differenzierung vorgenommen werden sollte. Antikörper der ANA-Differenzierung sind oft hoch-spezifisch für eine bestimmte Kollagenose (wie z. B. Topoisomerase-1-Antikörper bei der systemischen Sklerose). Der Nachweis von CCP-Antikörpern hat die Diagnostik der rheumatoiden Arthritis (RA) wesentlich bereichert. Neben ihrem prognostischen Wert hinsichtlich eines erosiven Verlaufs der RA sind sie mit extraartikulären Manifestationen assoziiert.

Bei rheumatologischen Systemerkrankungen haben Antikörper neben einem diagnostischen oft auch einen prognostischen Stellenwert. Antikörpertiter korrelieren zum Teil mit der Krankheitsaktivität und sollten in die Verlaufsbeurteilung einbezogen werden.

Abstract

The lung is frequently involved in disorders such as connective tissue diseases and small vessel vasculitides. Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides are often associated with alveolar haemorrhage and pulmonary granulomatous disease, whereas connective tissue disorders present with interstitial lung disease and pulmonary hypertension. Detection of ANCA directed against proteinase 3 or myeloperoxidase is helpful in the diagnosis of ANCA-associated vasculitis, and antinuclear antibodies (ANA) should be determined in cases of suspected connective tissue disease. Autoantibodies determined by ANA differentiation are often very specific for a certain type of connective tissue disease, e.g. antitopoisomerase-1 antibodies for systemic sclerosis. Autoantibody titres may correlate with disease activity or be associated with a special pattern of organ involvement and should be included in the diagnostics of connective tissue diseases and vasculitides.

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Abb. 1

Abbreviations

AAV:

ANCA-assoziierte Vaskulitis

ACLA:

Antikardiolipin-Antikörper

AECA:

“Anti-endothelial cell antibody”

AFA:

Antifibroblasten-Antikörper

AK:

Antikörper

ANCA:

Antineutrophiler zytoplasmatischer Antikörper

Anti-dsDNS:

Antikörper gegen doppelsträngige DNS

CCP-AK:

Antikörper gegen zyklische zitrullinierte Peptide

CCR:

„Chemokine receptor“

COP:

„Cryptogen organizing pneumonia“, kryptogen organisierende Pneumonie

CSS:

Churg-Strauss-Syndrom

CVD:

„Collagen vascular disease“, Kollagenose

DGRh:

Deutsche Gesellschaft für Rheumatologie

ECM:

Extrazellulärmatrix

ELAM-1:

„Endothelium leucocyte adhesion molecule 1“

ELISA:

“Enzyme-linked immunosorbent assay”

EUSTAR/EULAR:

EULAR Scleroderma Trials and Research Group Database/European League Against Rheumatism

GN:

Glomerulonephritis

HRCT:

“High-resolution computed tomography”

ICAM-1:

„Intercellular adhesion molecule 1“

IFT:

Immunfluoreszenztest

ILD:

„Interstitial lung disease“; interstitielle Lungenerkrankung

MCTD:

“Mixed connective tissue disease”; Mischkollagenose

MCV-Ak:

Antikörper gegen mutiertes zitrulliniertes Vimentin

MPA:

Mikroskopische Polyangiitis

NSIP:

“Nonspecific interstitial pneumonia”

PDGFR-AK:

“Platelet-derived growth factor antibody”

PH:

Pulmonaler Hypertonus

PM/DM:

Polymyositis/Dermatomyositis

Rag2 −/−:

Defizienz für“recombination activating gene 2”

ROS:

„Reactive oxygen species“

SE:

„Shared epitope“; konservierte Aminosäuresequenz in mit RA-assoziierten Allelen

SSc:

„Systemic sclerosis“; systemische Sklerose; dc SSc: diffus kutane SSc; lc SSc: limitiert kutane SSc

SLE:

Systemischer Lupus erythematodes

UIP:

„Usual interstitial pneumonia“

WG:

Wegener-Granulomatose

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Authors and Affiliations

  1. Poliklinik für Rheumatologie, Universitätsklinikum Schleswig-Holstein, Campus Lübeck, Ratzeburger Allee 160, 23538 , Lübeck, Deutschland

    J.U. Holle, E. Csernok  & W.L. Gross

  2. Klinik für Rheumatologie und Immunologie, Klinikum Bad Bramstedt, Bad Bramstedt, Deutschland

    J.U. Holle

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  1. J.U. Holle
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Holle, J., Csernok , E. & Gross, W. Autoantikörperdiagnostik rheumatologischer Systemerkrankungen mit pulmonaler Manifestation. Pneumologe 6, 213–223 (2009). https://doi.org/10.1007/s10405-008-0302-0

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