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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Interessenkonflikt
J.H. Schirmer und F. Moosig geben an, dass kein Interessenkonflikt besteht.
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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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DOI: https://doi.org/10.1007/s00393-018-0469-7