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Update: Polyarteriitis nodosa

Update: polyarteritis nodosa

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Zusammenfassung

Die Polyarteriitis nodosa (PAN) ist eine nekrotisierende Arteriitis mittelgroßer Gefäße, die unbehandelt oftmals letal verläuft. Sie manifestiert sich häufig an der Haut (Noduli oder Ulzera), am peripheren Nervensystem (Mononeuritis multiplex) und durch Stenosen und Mikroaneurysmen der viszeralen Gefäße. Die Diagnose erfordert eine komplexe diagnostische Aufarbeitung zur Abgrenzung von infektiösen, malignen, medikamenteninduzierten und anderen inflammatorischen Erkrankungen. Die PAN kann in weitere Varianten unterteilt werden (idiopathisch, Hepatitis-B-assoziiert, mit hereditären inflammatorischen Syndromen assoziiert und eine isoliert kutane Verlaufsform). Während idiopathische und hereditär inflammatorische Formen einer immunsuppressiven Therapie bedürfen, wird die Hepatitis-B-assoziierte Variante mit Virostatika und Plasmapherese behandelt. Die Prognose der isoliert kutanen Form ist gut und bedarf selten hochpotenter Immunsuppression.

Abstract

Polyarteritis nodosa (PAN) is a necrotizing arteritis of medium-sized vessels, which is often fatal if untreated. It frequently affects the skin (nodules and ulcers), the peripheral nervous system (mononeuritis multiplex) and the visceral vessels (stenoses and microaneurysms). The complex diagnostic work-up requires discriminating PAN from infectious, malignant, drug-induced and other inflammatory conditions. It can be subclassified into further variants (idiopathic, associated with hepatitis B, associated with hereditary inflammatory diseases or isolated cutaneous disease). While idiopathic and hereditary inflammatory variants require immunosuppressive treatment, the hepatitis B-associated variant is treated with virustatic agents and plasmapheresis. The isolated cutaneous variant has a good prognosis and rarely requires highly potent immunosuppressives.

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Correspondence to Jan H. Schirmer.

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Interessenkonflikt

J.H. Schirmer und F. Moosig geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.

Additional information

Redaktion

J. Wollenhaupt, Hamburg (Leitung)

O. Distler, Zürich

M. Fleck, Bad Abbach

J. Grifka, Bad Abbach

CME-Fragebogen

CME-Fragebogen

Was ist ein typischer Biopsiebefund bei der PAN?

Leukozytoklastische Kleingefäßvaskulitis in der Hautbiopsie

Nekrotisierende Arteriitis in der Muskelbiopsie

Nekrotisierende Kapillaritis in der Lungenbiopsie

Nekrotisierende Glomerulonephritis in der Nierenbiospie

Nekrotisierende Vaskulitis von Arteriolen mit prädominanten IgA1-Immunkomplexablagerungen in der Darmbiopsie

Wie viel Prozent der gesamten PAN-Patienten macht die HBV-PAN durchschnittlich insgesamt aus?

20 %

30 %

40 %

50 %

60 %

Mutationen in welchem Gen sind mit hereditären Varianten der PAN assoziiert?

CECR1 („cat eye syndrome chromosome region candidate 1“)

LAMP2 (Lysosomal Associated Membrane Protein 2)

HLA-B51 (Human Leukocyte Antigen-B51)

TFR2 (Transferrin-Rezeptor 2)

BTK (Bruton Tyrosinkinase)

Welcher der folgenden Befunde ist für die PAN besonders charakteristisch?

Serologischer Nachweis von Anti-HBs-Antikörpern

Angiographischer Nachweis von Mikroaneurysmen der Niere

Histologischer Nachweis einer Vaskulitis von Arteriolen und Venolen in der Nervenbiopsie

Computertomographischer Nachweis von unregelmäßiger perirenaler Fibrose („hairy kidneys“)

Pathergiephänomen

Welches Scoringsystem hat prognostische Aussagekraft bei PAN?

CHCC(Chapel Hill Consensus Conference)-Klassifikation von 2012

BVAS (Birmingham Vasculitis Activity Score)

DEI (Disease Extent Index)

DCVAS (Diagnostic and Classification Criteria for Vasculitis)

FFS (Five Factor Score)

Bei welcher Variante der PAN ist die Plasmaseparation Teil der etablierten Therapie?

Idiopathische PAN

HBV-PAN

Kutane Arteriitis

PAN bei FMF

PAN bei ADA2-Defizienz

Welche Therapie scheint nach bisherigen Daten insbesondere wirksam bei der ADA2-Defizienz zu sein?

Cyclophosphamid

Glukokortikoide

Etanercept

Minocyclin

Colchicin

Welche der folgenden Aussagen zu Verlauf und Prognose der PAN ist richtig?

Die kutane Arteriitis hat eine gute Prognose und ein niedriges Rezidivrisiko.

Die HBV-PAN hat eine schlechtere Prognose als die idiopathische PAN.

Die HBV-PAN hat ein höheres Rezidivrisiko als die idiopathische PAN.

Bei der idiopathischen PAN treten operationsbedürftige intestinale Komplikationen häufiger auf als bei der HBV-PAN.

Die kutane Arteriitis geht häufig in die systemische idiopathische Verlaufsform über.

Welche der folgenden Manifestationen ist typisch für die PAN?

Orchitis

Glomerulonephritis

Hypophysitis

Pulmonale Arteriitis

Sialadenitis

Welche der folgenden Antwortmöglichkeiten ist bei PAN nicht mit einer erhöhten Mortalität assoziiert?

Alter > 65 Jahre

Herzinsuffizienz

Mononeuritis multiplex

Niereninsuffizienz

Beteiligung des Gastrointestinaltraktes

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Schirmer, J.H., Moosig, F. Update: Polyarteriitis nodosa. Z Rheumatol 77, 397–408 (2018). https://doi.org/10.1007/s00393-018-0469-7

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