Zusammenfassung
Hintergrund
IgG4-assoziierte Autoimmunerkrankungen sind seltene systemische Multiorganerkrankungen und können jedes Organsystem betreffen. Ihre Diagnosehäufigkeit nimmt mit zunehmendem Bekanntheitsgrad deutlich zu.
Fragestellung
Im Abdomen bietet das pankreatikohepatobiliäre System ein wesentliches Organsystem für die Ausprägung IgG4-assoziierter Autoimmunerkrankungen. Im Vordergrund steht die Autoimmunpankreatitis Typ 1, weniger bekannt sind die IgG4-assoziierte Cholangitis und die IgG4-assoziierte Hepatopathie, die auch in Kombination auftreten können.
Material und Methode
Für die Diagnostik der pankreatikohepatobiliären IgG4-assoziierten Autoimmunerkrankungen existieren verschiedene, überwiegend asiatische, histologisch zentrierte Diagnosesysteme wie HISORt oder ICDC (International Consensus Diagnostic Criteria), in welchen die Bildgebung jedoch einen wichtigen Stellenwert einnimmt.
Ergebnisse
Neben der generalisierten Organschwellung sind weitere morphologische, aber auch funktionelle bildgebende Kriterien besonders für die Autoimmunpankreatitis zunehmend bekannt geworden wie das „late enhancement“ oder das bildgebende Ansprechen auf eine Steroidtherapie. Auch die Magnetresonanzcholangiopankreatikographie (MRCP) und die endoskopische retrograde Cholangiopankreatikographie (ERP) können bei IgG4-assoziierten Autoimmunerkrankungen im pankreatikohepatobiliären System wertvolle Hinweise auf deren Vorliegen liefern.
Schlussfolgerungen
IgG4-assozierte Autoimmunerkrankungen des pankreatikohepatobiliären Systems sind eine seltene Erkrankungsgruppe, deren radiologisches Erscheinungsbild zu kennen auch zu einer zunehmenden Diagnosehäufigkeit führt. Davon abzugrenzen sind die nicht nekrotisierende Pankreatitis und das Pankreaskarzinom, was oft schwierig ist, für den Patienten aber eine wesentliche therapeutische Konsequenz nach sich zieht.
Abstract
Background
IgG4-related diseases are rare systemic multiorgan diseases and can thus affect any organ system. The incidence of diagnosis has significantly increased due to increasing awareness.
Objective
In the abdomen the hepatopancreaticobiliary system provides an essential organ system for the expression of IgG4-associated autoimmune diseases. The focus here is autoimmune pancreatitis type 1 but IgG4-associated sclerosing cholangitis and IgG4-associated hepatopathy, which can also occur in combination are less well-known.
Methods
Various mostly Asiatic, histologically-based diagnostic systems, such as HISORt or international consensus diagnostic criteria (ICDC) are available for the diagnostics of hepatopancreaticobiliary IgG4-related autoimmune diseases, in which imaging techniques playing an increasingly important role.
Results
In addition to generalized organ swelling further morphological and also functional imaging criteria have become increasingly well-known for autoimmune pancreatitis, such as late enhancement or the imaging response to steroid therapy. Magnetic resonance cholangiopancreatography (MRCP) and endoscopic retrograde cholangiopancreatography (ERP) can provide valuable information for the diagnostics of IgG4-related diseases in the hepatopancreaticobiliary system.
Conclusion
IgG4-related autoimmune diseases of the hepatopancreaticobiliary system are a rare group of diseases in which increasing knowledge of the radiological appearance also leads to an increasingly frequency of diagnosis. IgG4-related diseases must be distinguished from non-necrotizing pancreatitis and pancreatic cancer, which is often difficult but has significant therapeutic consequences for the patients.
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Grenacher, L. Pankreatikohepatobiliäre Erkrankungen bei IgG4‑assoziierten Autoimmunerkrankungen. Radiologe 56, 1052–1060 (2016). https://doi.org/10.1007/s00117-016-0185-8
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DOI: https://doi.org/10.1007/s00117-016-0185-8
Schlüsselwörter
- Pankreatitis
- Hepatopathie
- Cholangitis
- LPSP (lymphoplasmatische sklerosierende Pankreatitis)
- AIP (Autoimmunpankreatitis)