Summary
We present the case of a 42-year-old female patient with the diagnoses of autoimmune
hepatitis type I and autoimmunethyroiditis. Furthermore this patient had an unusual
combination of coagulation disorders with homozygous Factor V Leiden mutation (APC
resistance) and presence of antiphospholipid antibodies, leading to deep vein thromboses
and miscarriages. Only few cases with the combination of autoimmune hepatitis and
antiphospholipid antibodies have been described and almost all of them had become
symptomatic with the antiphospholipid syndrome. As both autoimmune phenomenons furthermore
share similar HLA-patterns, their coincidence is probably not as uncommon as the limited
number of case reports suggests. Therefore attention in patients with autoimmune hepatitis
should be focused on thrombophilia.
Autoimmunhepatitis kombiniert mit Gerinnungsdefekten und Immunthyreopathie
Vorgestellt wird der Fall einer 42-jährigen Patientin mit der Erstdiagnose einer Autoimmunhepatitis
Typ I und Autoimmunthyreoiditis. Zusätzlich wies die Patientin mit der homozyogoten
Mutation für den Faktor-V-Leiden (APC-Resistenz) sowie dem Vorhandensein von Phospholipidantikörpern
eine ungewöhnlich schwere Kombination von Gerinnungsdefekten auf, die in der Vorgeschichte
zu 3 tiefen Beinvenenthrombosen und 3 Spontanaborten geführt hatte. Die wenigen bislang
beschriebenen Fälle mit gemeinsamem Auftreten von Autoimmunhepatitis und Phospholipidantikörpern
waren fast immer mit einem Antiphospholipid-Syndrom symptomatisch geworden. Da beide
Autoimmunphänomene außerdem gemeinsame HLA-Muster aufweisen, ist deren Koinzidenz
möglicherweise größer als angenommen. Daher sollte bei Patienten mit Autoimmunhepatitis
gezielte Aufmerksamkeit auf eine eventuelle Thromboseneigung gerichtet werden.
Key words
Autoimmune Hepatitis - Antiphospholipid - Anticardiolipin - Factor V Leiden - APC-Resistance
- Thyroiditis - Thrombosis
Schlüsselwörter
Autoimmunhepatitis - Antiphospholipid - Kardiolipinantikörper - Faktor-V-Leiden -
APC-Resistenz - Thyreoiditis - Thrombose
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Address for correspondence
Dr. A. Widjaja
Department of Gastroenterology and Hepatology Hannover Medical School
Carl-Neuberg Straße 1
30625 Hannover
Email: widjaja.adji@mh-hannover.de