Summary
Background: Controlled trials have firmly
established the need for immunosuppressive therapy in autoimmune hepatitis.
However, reports about long-term management and prognosis of the disease are
scarce.
Patients and methods: We reviewed the
charts of 103 consecutive patients with a well-documented long-term course of
autoimmune hepatitis who had been carefully managed over a mean observation
period of 95 months (12-405 months).
Results: Under immunosuppressive therapy 94
patients (91.2 %) reached complete remission after a mean
treatment duration of 3 ± 3 months. 28 of the 103 patients
(27.2 %) were eligible for a trial of treatment withdrawal after
a mean treatment duration of 32.2 months (range: 12-81 months). 21 of
these patients (75 %) had a relapse following treatment
withdrawal. 13.6 % of patients had intolerance of or severe side
effects to azathioprine. There was no increase in tumor risk during a
cumulative observation period of 423 patient-years of azathioprine therapy.
Corticosteroid side effects occurred mostly during induction therapy, but were
usually minor and resolved upon dose reduction. During a cumulative observation
period of 842 patient-years no liver related deaths occurred and no patient had
to be referred to liver transplantation, even though 30 patients
(29.1 %) had histological evidence of cirrhosis at presentation.
The overall 5- and 10-year survival of patients with autoimmune hepatitis was
identical to an age- and sex-matched control population.
Conclusion: Our study shows that the
majority of patients with AIH do achieve a complete remission within 3 months,
but require long-term or permanent immunosuppressive therapy that is usually
well tolerated. Long-term survival in well-managed patients is excellent.
Management und Prognose der autoimmunen Hepatitis: Erfahrungen
eines Zentrums
Einleitung: Kontrollierte Studien belegen
den Wert einer immunsuppressiven Therapie zur Behandlung der autoimmunen
Hepatitis. Allerdings liegen nur wenige Berichte über
Langzeitverläufe und Prognose dieser Erkrankung vor.
Patienten und Methodik: Die Krankenakten
von 103 konsekutiven Patienten mit lückenlos dokumentiertem Verlauf einer
autoimmunen Hepatitis, die wir über eine mittlere Beobachtungszeit von 95
Monaten (12-405 Monaten) betreuten, wurden ausgewertet.
Ergebnisse: 94 Patienten
(91,2 %) erreichten unter immunsuppressiver Therapie eine
komplette Remission innerhalb von 3 ± 3 Monaten. Bei 28
Patienten (27,2 %) wurde nach einer mittleren Behandlungsdauer
von 32,2 Monaten die immunsuppressive Therapie schrittweise beendet. 21 dieser
Patienten (75 %) hatten ein Rezidiv der autoimmunen Hepatitis. 14
Patienten (13,6 %) konnten aufgrund von Unverträglichkeit
oder Nebenwirkungen nicht mit Azathioprin behandelt werden. Während einer
kumulativen Beobachtungsdauer von 423 Patientenjahren unter Azathioprintherapie
beobachteten wir kein signifikant erhöhtes Tumorrisiko. Nebenwirkungen auf
Kortikosteroide traten meist während der Induktionsphase auf, waren jedoch
meist geringfügig und reversibel nach Dosisreduktion. Obwohl bereits 30
Patienten (29,1 %) bei Diagnosestellung eine Leberzirrhose
aufwiesen, traten während einer kumulativen Beobachtungszeit von 842
Patientenjahren keine Todesfälle als Komplikation der Lebererkrankung auf.
Kein Patient musste lebertransplantiert werden. Das 5- und
10-Jahres-Überleben unterschied sich nicht von dem eines
Kontrollkollektivs.
Schlussfolgerungen: Die Mehrzahl der
Patienten mit autoimmuner Hepatitis erreicht innerhalb der ersten 3 Monate nach
Therapiebeginn eine komplette Remission. Allerdings ist meist eine
Langzeittherapie notwendig, die als niedrig dosierte Kombinationstherapie gut
vertragen wird. Die Langzeitprognose von Patienten mit autoimmuner Hepatitis
ist bei entsprechender Patientenführung exzellent.
Key words
Autoimmune
Hepatitis - Immunosuppressive Therapy - Side
Effects - Relapse - Survival
Schlüsselwörter
Antoimmune
Hepatitis - immunosuppressive
Therapie - Nebenwirkungen - Rezidiv - Überlebensrate
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Address for correspondence
Ansgar W. Lohse, MD
I. Department of Medicine
Johannes Gutenberg
University
55101 MainzGermany
Email: lohse@mail.uni-mainz.de
