Autoimmune hepatitis associated with coagulation disorders and immunethyreopathy

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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