Autoimmunologiczne zapalenie wątroby – Rokowania, prognozy i postęp choroby

Autoimmunologiczne zapalenie wątroby
Rokowania, prognozy i postęp choroby

Autoimmunologiczne zapalenie wątroby (AIH) jest przewlekłą chorobą charakteryzującą się immunologicznym uszkodzeniem miąższu wątroby, które bez leczenia prowadzi do 10-letniego przeżycia na poziomie około 50%, natomiast terapia zwiększa je powyżej 90%. Kluczowymi czynnikami prognostycznymi są obecność marskości wątroby (10-letnie przeżycie bez przeszczepu wynosi 94% u pacjentów bez marskości, 79% u z wyrównaną marskością i 44% u z niewyrównaną marskością), wiek rozpoznania (gorsze rokowanie u pacjentów <18 lat), obecność przeciwciał SLA/LP oraz poziomy trójglicerydów (TG ≤0,95 mmol/L wiąże się z gorszym przeżyciem, HR=3,79, 95% CI: 1,53-9,42). Wczesna odpowiedź biochemiczna, definiowana normalizacją aminotransferaz i IgG, jest kluczowa dla dobrego rokowania, a niekompletna normalizacja ALT po 6 miesiącach koreluje z gorszymi wynikami. Dodatkowo, poziomy ferrytyny i IgG w surowicy stanowią predyktory odpowiedzi na leczenie, co może ułatwić stratifikację ryzyka i decyzje terapeutyczne.

Prognoza autoimmunologicznego zapalenia wątroby

Wskaźniki przeżywalności w AIH
Czynniki prognostyczne wpływające na przebieg choroby
Czynniki biochemiczne i prognostyczne modele predykcyjne
Nawroty po przeszczepieniu wątroby
Znaczenie nieinwazyjnych metod diagnostycznych

Podsumowanie czynników prognostycznych w AIH

Kolejne rozdziały

Prognoza autoimmunologicznego zapalenia wątroby

Autoimmunologiczne zapalenie wątroby (AIH) jest przewlekłą chorobą zapalną charakteryzującą się immunologicznie uwarunkowanym niszczeniem miąższu wątroby, które może prowadzić do marskości, niewydolności wątroby, a nawet zgonu. Odpowiednie leczenie znacząco poprawia rokowanie i przeżywalność pacjentów.¹²

Wskaźniki przeżywalności w AIH

Bez odpowiedniego leczenia dziesięcioletnie przeżycie u pacjentów z objawowym AIH wynosi około 50%. Natomiast u pacjentów poddanych terapii dziesięcioletnie przeżycie przekracza 90%.¹ Zgodnie z modelami prognostycznymi, 10-letni wskaźnik przeżycia związanego z chorobą wątroby wynosi 91% (95% CI 88%-94%), a przeżycie bez konieczności przeszczepienia wątroby (LT-free survival) wynosi 86% (95% CI 82%-90%).² W jednym z badań wykazano, że przeżycie po przeszczepieniu wątroby wynosi 75% w 8-letniej obserwacji, co potwierdza dobre wyniki leczenia u pacjentów wymagających transplantacji.³

Obserwacje wskazują, że ogólna przeżywalność i standaryzowany współczynnik umieralności (SMR) pozostają na podobnym poziomie w długoterminowej obserwacji. Należy jednak zauważyć, że pacjenci z AIH nadal wykazują zwiększoną długoterminową śmiertelność z powodu choroby wątroby, również w grupie osób powyżej 65. roku życia.⁴

Czynniki prognostyczne wpływające na przebieg choroby

Zidentyfikowano kilka kluczowych czynników prognostycznych, które wpływają na odpowiedź na leczenie i długoterminowe rokowanie w AIH:

Marskość wątroby

Obecność marskości wątroby w momencie rozpoznania (występująca u około 25-30% pacjentów) jest istotnym czynnikiem prognostycznym związanym z gorszym rokowaniem i zwiększonym ryzykiem konieczności przeszczepienia wątroby.⁵⁶ Średnie 10-letnie przeżycie bez przeszczepu wątroby wynosi 94% (95% CI 90%-97%) u pacjentów bez marskości, 79% (95% CI 69%-88%) u pacjentów z wyrównaną marskością oraz 44% (95% CI 26%-63%) u pacjentów z niewyrównaną marskością (p≤0.001).⁷ Badania dotyczące rokowania u pacjentów z marskością przyniosły niejednoznaczne wyniki – jedno z badań wykazało, że 10-letnie przeżycie w AIH z marskością jest porównywalne do pacjentów bez marskości, podczas gdy inne badanie wykazało, że 10-letnie przeżycie jest obniżone do 64%.⁸

Wiek zachorowania

Pacjenci zdiagnozowani w dzieciństwie mają wyższe ryzyko nawrotów (P=0.003) i większe prawdopodobieństwo konieczności przeszczepienia wątroby (P=0.014), a także skróconą oczekiwaną długość życia (P<0.001).⁹ Rozpoznanie w wieku poniżej 18 lat, obok marskości wątroby stwierdzonej przy pierwszej diagnozie oraz obecności przeciwciał przeciwko rozpuszczalnemu antygenowi wątrobowemu/antygenowi wątrobowo-trzustkowemu (SLA/LP), stanowi główny czynnik ryzyka niekorzystnego krótko- i długoterminowego rokowania.¹⁰

Markery serologiczne

Wykrycie przeciwciał przeciwko rozpuszczalnemu antygenowi wątrobowemu/antygenowi wątrobowo-trzustkowemu (SLA/LP) wiąże się istotnie ze zmniejszonym ogólnym przeżyciem i przeżyciem bez przeszczepu wątroby (P=0.037; P=0.021).¹¹ Z kolei obecność allelu DRB1*04:01 została zidentyfikowana jako czynnik związany z korzystnym przebiegiem klinicznym.¹²

Grupy etniczne

Prezentacja kliniczna i odpowiedź na terapię mogą różnić się w zależności od rasy. Afroamerykanie zazwyczaj mają bardziej agresywny przebieg choroby, wiążący się z gorszymi wynikami leczenia.¹³ Podobnie do innych autoimmunologicznych chorób wątroby, AIH nie jest jednolite; populacje wysokiego ryzyka, takie jak Afroamerykanie lub osoby z wczesnym początkiem choroby, niekompletną normalizacją testów wątrobowych i zaawansowaną chorobą w momencie diagnozy, mają gorsze ogólne przeżycie.¹⁴

Czynniki biochemiczne i prognostyczne modele predykcyjne

Odpowiedź biochemiczna

Niekompletna odpowiedź na leczenie, definiowana jako niepełne ustąpienie objawów klinicznych, nieprawidłowe wyniki badań biochemicznych (AST/ALT i IgG) oraz ewentualnie nieprawidłowe wyniki histologiczne, ma istotne znaczenie prognostyczne. Aktualne wytyczne sugerują normalizację aminotransferaz i poziomów IgG jako kluczowy cel terapeutyczny, ponieważ kliniczny wpływ niekompletnej odpowiedzi został powiązany z progresją włóknienia, zgonem związanym z wątrobą lub koniecznością przeszczepienia wątroby.¹⁵

Wczesna odpowiedź biochemiczna ma istotne znaczenie prognostyczne – pacjenci wykazujący odpowiedź biochemiczną już po 3 miesiącach leczenia mają doskonałe długoterminowe przeżycie.¹⁶ Natomiast niekompletna normalizacja ALT w ciągu 6 miesięcy terapii była niezależnie związana z gorszymi wynikami w badaniu obejmującym 133 pacjentów z AIH z Nowej Zelandii.¹⁷ Mimo eliminacji czynników przyczyniających się do uszkodzenia wątroby, optymalizacji terapii pierwszego rzutu i zapewnienia przestrzegania zaleceń medycznych, nieprawidłowe wyniki testów wątrobowych będą obecne u 93-94% leczonych pacjentów po 2 latach.¹⁸

Markery metaboliczne

Badania wskazują, że wskaźniki związane z metabolizmem mogą mieć wartość prognostyczną w AIH. Szczególnie poziom trójglicerydów (TG) okazał się być ściśle związany z ciężkością choroby i ogólnym przeżyciem pacjentów z marskością wątroby w przebiegu AIH. Analiza przeżycia wykazała, że poziomy TG były związane z ogólnym przeżyciem pacjentów z AIH, przy czym niższa 5-letnia przeżywalność (log-rank P≤0.05) była obserwowana u pacjentów w grupie z TG≤0.95 mmol/L (współczynnik ryzyka (HR)=3.79, 95% CI: 1.528-9.423).¹⁹ Wyniki sugerują, że niższe poziomy TG były czynnikiem ryzyka zgonu u pacjentów z AIH, niezależnie od tego, czy mieli marskość, czy niewyrównaną marskość wątroby.²⁰

Poziom TG jest łatwo wykrywalnym biochemicznym wskaźnikiem klinicznym, który może być stosowany jako nowy wskaźnik zaawansowanego stadium choroby wątroby lub długoterminowego rokowania u pacjentów z AIH.²¹

Ferrytyna i hipergammaglobulinemia

Nieprawidłowa homeostaza żelaza lub hiperferrytynemii wiąże się z gorszymi wynikami w innych przewlekłych chorobach wątroby i po przeszczepieniu wątroby.²² Warto zauważyć, że podstawowy poziom ferrytyny w surowicy był prawidłowy u około połowy dorosłych pacjentów i u większości dzieci z pAIH.²³

Hiperferrytynemii (2,09 razy powyżej górnej granicy normy; iloraz szans (OR) = 8,82; 95% przedział ufności (CI): 2,25-34,52) i niższe poziomy immunoglobulin (1,89 razy powyżej górnej granicy normy; OR = 6,78; CI: 1,87-24,59) w momencie rozpoznania były niezależnie związane z osiągnięciem pełnej remisji biochemicznej po standardowej terapii.²⁴ Wartość predykcyjna wzrosła, gdy obie zmienne zostały połączone w pojedynczy wynik odpowiedzi na leczenie. Pacjenci z niskim wynikiem odpowiedzi na leczenie (≤1) mieli znacząco wyższe skumulowane wskaźniki remisji zarówno w kohorcie treningowej (p≤0.001), jak i walidacyjnej (p = 0.024).²⁵

Ferrytyna w surowicy jest pierwszym predyktorem późniejszej odpowiedzi biochemicznej po standardowej terapii w nieleczonym AIH-1. Połączenie obu wartości, ferrytyny w surowicy i IgG, uzupełnia się wzajemnie i ułatwia bardziej precyzyjne przewidywanie. Stratyfikacja ryzyka odpowiedzi na leczenie już w momencie diagnozy za pomocą tych dwóch rutynowych parametrów krwi, które można określić niedrogo, może ułatwić wcześniejsze przejście na terapię ratunkową.²⁶

Modele prognostyczne

Opracowano i zwalidowano holendersko-belgijski model prognostyczny dla długoterminowego przeżycia bez przeszczepu u pacjentów z AIH w momencie diagnozy. Ten nowy model przeżycia AIH składający się z wieku, pochodzenia etnicznego, marskości wątroby i ALT w momencie diagnozy może dokładnie przewidzieć długoterminowe przeżycie bez przeszczepu u pacjentów z AIH.²⁷

W przypadku dzieci z autoimmunologicznym zapaleniem wątroby (pAIH), wyższe wyniki AIH w momencie rozpoznania (>16 punktów) mogą przewidywać niepełną odpowiedź na standardowe leczenie. Dodatkowo, niższe poziomy IL-2 mogą dodatkowo wskazywać na niepełną odpowiedź na leczenie.²⁸²⁹ Ponieważ utrzymująca się aktywność zapalna jest związana z progresją histologiczną choroby i zmniejszoną przeżywalnością w AIH, wczesna identyfikacja pacjentów z niewystarczającą odpowiedzią na standardową terapię jest klinicznie istotna.³⁰

Opracowano również nieinwazyjne modele diagnostyczne AIH u pacjentów z nieznanym uszkodzeniem wątroby, które nie wymagają biopsji wątroby. Te modele mają akceptowalną dokładność (p≤0.05) i dobrą użyteczność kliniczną.³¹

Nawroty po przeszczepieniu wątroby

Nawrót AIH (rAIH) po przeszczepieniu jest częsty i wynosi od 8 do 12 procent w rok po transplantacji. 5-letnie ryzyko nawrotu wynosi 36-68%. Mimo wysokiego wskaźnika nawrotów, niewydolność przeszczepu wymagająca retransplantacji występuje tylko u 13-23%, a 5-letnia przeżywalność dorosłych z nawracającym autoimmunologicznym zapaleniem wątroby jest doskonała i wynosi 89-100%.³²

Wyniki są generalnie dobre u pacjentów wymagających przeszczepienia wątroby, a ogólne 5-letnie przeżycie po przeszczepieniu przekracza 80%.³³ Nawrót AIH w przeszczepionej wątrobie może wystąpić i wydaje się być częstszy po odstawieniu prednizonu. Ponowne wprowadzenie prednizonu i optymalizacja inhibitorów kalcyneuryny zwykle indukuje remisję. Pacjenci ci mają podobne rokowanie jak pacjenci po przeszczepieniu, u których nie występuje nawrót AIH.³⁴

Znaczenie nieinwazyjnych metod diagnostycznych

Wartość nieinwazyjnych narzędzi w diagnostyce marskości związanej z AIH i przewidywaniu wyników klinicznych jest w dużej mierze nieznana. Aktualne punkty odcięcia dla pomiaru sztywności wątroby (LSM) nie mają dobrej zdolności dyskryminacyjnej do diagnozy marskości związanej z AIH z/bez nadciśnienia wrotnego, szczególnie długo po rozpoczęciu leczenia.³⁵ Niemniej jednak, nieinwazyjne narzędzia (kryteria Baveno VI, rozszerzone kryteria Baveno VI i ciągły model ANTICIPATE-PBC) do segregacji pacjentów do endoskopii są przydatne u pacjentów z marskością związaną z AIH.³⁶

Podsumowanie czynników prognostycznych w AIH

Podsumowując, najważniejsze czynniki wpływające na rokowanie w autoimmunologicznym zapaleniu wątroby to:

Wczesna odpowiedź biochemiczna na leczenie standardowe³⁷
Obecność marskości wątroby w momencie rozpoznania³⁸³⁹
Wiek w momencie rozpoznania (gorsze rokowanie u pacjentów zdiagnozowanych przed 18. rokiem życia)⁴⁰
Obecność przeciwciał SLA/LP⁴¹
Poziomy trójglicerydów (TG ≤0.95 mmol/L związane z gorszym rokowaniem)⁴²
Poziomy ferrytyny w surowicy i immunoglobulin G⁴³⁴⁴
Przynależność etniczna (gorsze rokowanie u Afroamerykanów)⁴⁵⁴⁶
Wyższe wyniki w skali AIH (>16 punktów) u dzieci⁴⁷⁴⁸
Niższe poziomy IL-2 u dzieci⁴⁹

Modele prognostyczne obejmujące wiek, przynależność etniczną, obecność marskości wątroby i poziom ALT w momencie diagnozy mogą dokładnie przewidzieć długoterminowe przeżycie bez przeszczepu u pacjentów z AIH.⁵⁰

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Materiały źródłowe

#1 Management of Difficult Cases of Autoimmune Hepatitis
https://pmc.ncbi.nlm.nih.gov/articles/PMC5410647/
Autoimmune hepatitis (AIH) is a complex autoimmune disease characterized by immune-mediated destruction of hepatic parenchyma which can result in cirrhosis, liver failure, and death. […] Similar to other autoimmune liver diseases all AIH is not the same; high risk populations such as African Americans, or those with early disease onset, incomplete normalization of liver tests, and advanced disease at diagnosis have worse overall survival. […] Incomplete response to a treatment regimen is defined by incomplete recovery of clinical symptoms, biochemical data (AST/ALT and IgG), and possibly histologic findings. Current guidelines suggest normalization of aminotransferases and IgG levels as a key therapeutic aim, as the clinical impact of incomplete response has been linked to fibrosis progression, liver-related death or requirement of liver transplantation.
#1 Autoimmune hepatitis – Wikipedia
https://en.wikipedia.org/wiki/Autoimmune_hepatitis
Prognosis 50% survival if untreated, 90% survival if treated […] Without treatment, the ten-year survival rate for individuals with symptomatic autoimmune hepatitis is 50%. However, with treatment, the ten-year survival rate is above 90%. […] Outcomes with liver transplant are generally favorable with a five-year survival greater than 80 percent. […] Presentation and response to therapy may differ according to race. African Americans appear to present with a more aggressive disease that is associated with worse outcomes.
#2
https://journals.lww.com/hep/fulltext/2015/11000/prediction_of_short__and_long_term_outcome_in.22.aspx
Autoimmune hepatitis (AIH) is a chronic inflammatory disease characterized by a loss of tolerance toward the hepatocellular epithelium. […] The aim of this study was to elucidate the clinical, serological, and genetic features of remission, relapse, and overall and LT-free survival. […] Patients diagnosed in childhood were at higher risk for relapses (P=0.003), requirement for LTs (P=0.014, log rank), and had a reduced life expectancy (P<0.001, log rank). [...] Detection of soluble liver antigen/liver pancreas antigen (SLA/LP) antibodies was significantly associated with reduced overall and LT-free survival (P=0.037; P=0.021). [...] Cirrhosis, which was evident in 25% at first diagnosis, was found to be a predictor of poor survival and requirement for LT (P=0.003; P=0.009). [...] Diagnosis 18 years, histological cirrhosis at first diagnosis and SLA/LP antibodies are major risk factors for a poor short and long-term outcome. [...] DRB1*04:01 positivity has been identified in association with a favorable clinical outcome.
#2 Development and validation of a prognostic score for longâterm transplantâfree survival in autoimmune hepatitis type 1
https://pmc.ncbi.nlm.nih.gov/articles/PMC8281048/
No prognostic score is currently available for longterm survival in autoimmune hepatitis (AIH) patients. […] A DutchBelgian prognostic score for longterm transplantfree survival in AIH patients at diagnosis was developed and validated. […] The 10 year liverrelated survival rate was 91% (95% CI 88%94%), the LTfree survival rate was 86% (95% CI 82%90%). […] Median 10 year LTfree survival was 94% (95% CI 90%97%), 79% (95% CI 69%88%) and 44% (95% CI 26%63%) in patients without cirrhosis, compensated cirrhosis and decompensated cirrhosis respectively (p 0.001). […] This novel DutchBelgian AIH survival model consisting of age, ethnicity, cirrhosis and ALT at diagnosis can accurately predict the longterm LTfree survival in AIH patients.
#3 Autoimmune Hepatitis| Cleveland Clinic
https://my.clevelandclinic.org/departments/digestive/medical-professionals/hepatology/autoimmune-hepatitis
In patients responsive to treatment, AIH has a good prognosis. The majority of treated patients will achieve remission and the 10-year survival rate approaches 83.8% to 94%. […] In patients with established cirrhosis at the beginning of treatment, data on prognosis have been conflicting. While in 1 study, treatment in AIH with cirrhosis showed a 10-year life expectancy comparable to non-cirrhotics, another has shown that the 10-year life expectancy is reduced at 64%. […] The outcomes are generally good in those patients who require liver transplantation. In these patients, survival is 75% at 8 years. Recurrence of AIH in the transplanted liver can occur and appears to be more common when prednisone is discontinued. Reintroduction of prednisone and optimization of calcineurin inhibitors usually induces remission. These patients have a similar prognosis as transplanted patients who do not have recurrent AIH.
#4 PWE-077âLong-term outcomes in autoimmune hepatitis: an update | Gut
https://gut.bmj.com/content/66/suppl_2/a165
Short-term outcome of treated AIH is good but liver disease may progress despite treatment. […] Overall survival and SMR are similar to those we previously reported. AIH patients still have excess long-term mortality due to their liver disease, including those presenting at age over 65 (new observation). Patients presenting since 2007 compared to those presenting earlier, are older, less likely to have cirrhosis and have a trend towards lower all-cause short-term mortality.
#5
https://journals.lww.com/hep/fulltext/2015/11000/prediction_of_short__and_long_term_outcome_in.22.aspx
Autoimmune hepatitis (AIH) is a chronic inflammatory disease characterized by a loss of tolerance toward the hepatocellular epithelium. […] The aim of this study was to elucidate the clinical, serological, and genetic features of remission, relapse, and overall and LT-free survival. […] Patients diagnosed in childhood were at higher risk for relapses (P=0.003), requirement for LTs (P=0.014, log rank), and had a reduced life expectancy (P<0.001, log rank). [...] Detection of soluble liver antigen/liver pancreas antigen (SLA/LP) antibodies was significantly associated with reduced overall and LT-free survival (P=0.037; P=0.021). [...] Cirrhosis, which was evident in 25% at first diagnosis, was found to be a predictor of poor survival and requirement for LT (P=0.003; P=0.009). [...] Diagnosis 18 years, histological cirrhosis at first diagnosis and SLA/LP antibodies are major risk factors for a poor short and long-term outcome. [...] DRB1*04:01 positivity has been identified in association with a favorable clinical outcome.
#6
https://journals.lww.com/hepcomm/fulltext/2022/06000/noninvasive_prediction_of_outcomes_in_autoimmune.12.aspx
The value of noninvasive tools in the diagnosis of autoimmune hepatitis (AIH) related cirrhosis and the prediction of clinical outcomes is largely unknown. […] Approximately 30% of patients with autoimmune hepatitis (AIH) present with cirrhosis at diagnosis, and a further 10% develop cirrhosis during follow-up. […] Therefore, patients with AIH require close and lifelong follow-up. […] The current LSM cutoff points do not have good discriminative capacity for the diagnosis of AIH related cirrhosis with/without PHT, especially long-term after treatment initiation. […] Nonetheless, noninvasive tools (Baveno VI, expanded Baveno VI criteria, and ANTICIPATEPBC continuous model) to triage patients for endoscopy are useful in patients with AIH related cirrhosis.
#7 Development and validation of a prognostic score for longâterm transplantâfree survival in autoimmune hepatitis type 1
https://pmc.ncbi.nlm.nih.gov/articles/PMC8281048/
No prognostic score is currently available for longterm survival in autoimmune hepatitis (AIH) patients. […] A DutchBelgian prognostic score for longterm transplantfree survival in AIH patients at diagnosis was developed and validated. […] The 10 year liverrelated survival rate was 91% (95% CI 88%94%), the LTfree survival rate was 86% (95% CI 82%90%). […] Median 10 year LTfree survival was 94% (95% CI 90%97%), 79% (95% CI 69%88%) and 44% (95% CI 26%63%) in patients without cirrhosis, compensated cirrhosis and decompensated cirrhosis respectively (p 0.001). […] This novel DutchBelgian AIH survival model consisting of age, ethnicity, cirrhosis and ALT at diagnosis can accurately predict the longterm LTfree survival in AIH patients.
#8 Autoimmune Hepatitis| Cleveland Clinic
https://my.clevelandclinic.org/departments/digestive/medical-professionals/hepatology/autoimmune-hepatitis
In patients responsive to treatment, AIH has a good prognosis. The majority of treated patients will achieve remission and the 10-year survival rate approaches 83.8% to 94%. […] In patients with established cirrhosis at the beginning of treatment, data on prognosis have been conflicting. While in 1 study, treatment in AIH with cirrhosis showed a 10-year life expectancy comparable to non-cirrhotics, another has shown that the 10-year life expectancy is reduced at 64%. […] The outcomes are generally good in those patients who require liver transplantation. In these patients, survival is 75% at 8 years. Recurrence of AIH in the transplanted liver can occur and appears to be more common when prednisone is discontinued. Reintroduction of prednisone and optimization of calcineurin inhibitors usually induces remission. These patients have a similar prognosis as transplanted patients who do not have recurrent AIH.
#9
https://journals.lww.com/hep/fulltext/2015/11000/prediction_of_short__and_long_term_outcome_in.22.aspx
Autoimmune hepatitis (AIH) is a chronic inflammatory disease characterized by a loss of tolerance toward the hepatocellular epithelium. […] The aim of this study was to elucidate the clinical, serological, and genetic features of remission, relapse, and overall and LT-free survival. […] Patients diagnosed in childhood were at higher risk for relapses (P=0.003), requirement for LTs (P=0.014, log rank), and had a reduced life expectancy (P<0.001, log rank). [...] Detection of soluble liver antigen/liver pancreas antigen (SLA/LP) antibodies was significantly associated with reduced overall and LT-free survival (P=0.037; P=0.021). [...] Cirrhosis, which was evident in 25% at first diagnosis, was found to be a predictor of poor survival and requirement for LT (P=0.003; P=0.009). [...] Diagnosis 18 years, histological cirrhosis at first diagnosis and SLA/LP antibodies are major risk factors for a poor short and long-term outcome. [...] DRB1*04:01 positivity has been identified in association with a favorable clinical outcome.
#10
https://journals.lww.com/hep/fulltext/2015/11000/prediction_of_short__and_long_term_outcome_in.22.aspx
Autoimmune hepatitis (AIH) is a chronic inflammatory disease characterized by a loss of tolerance toward the hepatocellular epithelium. […] The aim of this study was to elucidate the clinical, serological, and genetic features of remission, relapse, and overall and LT-free survival. […] Patients diagnosed in childhood were at higher risk for relapses (P=0.003), requirement for LTs (P=0.014, log rank), and had a reduced life expectancy (P<0.001, log rank). [...] Detection of soluble liver antigen/liver pancreas antigen (SLA/LP) antibodies was significantly associated with reduced overall and LT-free survival (P=0.037; P=0.021). [...] Cirrhosis, which was evident in 25% at first diagnosis, was found to be a predictor of poor survival and requirement for LT (P=0.003; P=0.009). [...] Diagnosis 18 years, histological cirrhosis at first diagnosis and SLA/LP antibodies are major risk factors for a poor short and long-term outcome. [...] DRB1*04:01 positivity has been identified in association with a favorable clinical outcome.
#11
https://journals.lww.com/hep/fulltext/2015/11000/prediction_of_short__and_long_term_outcome_in.22.aspx
Autoimmune hepatitis (AIH) is a chronic inflammatory disease characterized by a loss of tolerance toward the hepatocellular epithelium. […] The aim of this study was to elucidate the clinical, serological, and genetic features of remission, relapse, and overall and LT-free survival. […] Patients diagnosed in childhood were at higher risk for relapses (P=0.003), requirement for LTs (P=0.014, log rank), and had a reduced life expectancy (P<0.001, log rank). [...] Detection of soluble liver antigen/liver pancreas antigen (SLA/LP) antibodies was significantly associated with reduced overall and LT-free survival (P=0.037; P=0.021). [...] Cirrhosis, which was evident in 25% at first diagnosis, was found to be a predictor of poor survival and requirement for LT (P=0.003; P=0.009). [...] Diagnosis 18 years, histological cirrhosis at first diagnosis and SLA/LP antibodies are major risk factors for a poor short and long-term outcome. [...] DRB1*04:01 positivity has been identified in association with a favorable clinical outcome.
#12
https://journals.lww.com/hep/fulltext/2015/11000/prediction_of_short__and_long_term_outcome_in.22.aspx
Autoimmune hepatitis (AIH) is a chronic inflammatory disease characterized by a loss of tolerance toward the hepatocellular epithelium. […] The aim of this study was to elucidate the clinical, serological, and genetic features of remission, relapse, and overall and LT-free survival. […] Patients diagnosed in childhood were at higher risk for relapses (P=0.003), requirement for LTs (P=0.014, log rank), and had a reduced life expectancy (P<0.001, log rank). [...] Detection of soluble liver antigen/liver pancreas antigen (SLA/LP) antibodies was significantly associated with reduced overall and LT-free survival (P=0.037; P=0.021). [...] Cirrhosis, which was evident in 25% at first diagnosis, was found to be a predictor of poor survival and requirement for LT (P=0.003; P=0.009). [...] Diagnosis 18 years, histological cirrhosis at first diagnosis and SLA/LP antibodies are major risk factors for a poor short and long-term outcome. [...] DRB1*04:01 positivity has been identified in association with a favorable clinical outcome.
#13 Autoimmune hepatitis – Wikipedia
https://en.wikipedia.org/wiki/Autoimmune_hepatitis
Prognosis 50% survival if untreated, 90% survival if treated […] Without treatment, the ten-year survival rate for individuals with symptomatic autoimmune hepatitis is 50%. However, with treatment, the ten-year survival rate is above 90%. […] Outcomes with liver transplant are generally favorable with a five-year survival greater than 80 percent. […] Presentation and response to therapy may differ according to race. African Americans appear to present with a more aggressive disease that is associated with worse outcomes.
#14 Management of Difficult Cases of Autoimmune Hepatitis
https://pmc.ncbi.nlm.nih.gov/articles/PMC5410647/
Autoimmune hepatitis (AIH) is a complex autoimmune disease characterized by immune-mediated destruction of hepatic parenchyma which can result in cirrhosis, liver failure, and death. […] Similar to other autoimmune liver diseases all AIH is not the same; high risk populations such as African Americans, or those with early disease onset, incomplete normalization of liver tests, and advanced disease at diagnosis have worse overall survival. […] Incomplete response to a treatment regimen is defined by incomplete recovery of clinical symptoms, biochemical data (AST/ALT and IgG), and possibly histologic findings. Current guidelines suggest normalization of aminotransferases and IgG levels as a key therapeutic aim, as the clinical impact of incomplete response has been linked to fibrosis progression, liver-related death or requirement of liver transplantation.
#15 Management of Difficult Cases of Autoimmune Hepatitis
https://pmc.ncbi.nlm.nih.gov/articles/PMC5410647/
Autoimmune hepatitis (AIH) is a complex autoimmune disease characterized by immune-mediated destruction of hepatic parenchyma which can result in cirrhosis, liver failure, and death. […] Similar to other autoimmune liver diseases all AIH is not the same; high risk populations such as African Americans, or those with early disease onset, incomplete normalization of liver tests, and advanced disease at diagnosis have worse overall survival. […] Incomplete response to a treatment regimen is defined by incomplete recovery of clinical symptoms, biochemical data (AST/ALT and IgG), and possibly histologic findings. Current guidelines suggest normalization of aminotransferases and IgG levels as a key therapeutic aim, as the clinical impact of incomplete response has been linked to fibrosis progression, liver-related death or requirement of liver transplantation.
#16 Management of Difficult Cases of Autoimmune Hepatitis
https://pmc.ncbi.nlm.nih.gov/articles/PMC5410647/
In fact, utilization of early biochemical response may have merit in this arena, as Kanzler et al observed that patients exhibiting a biochemical response in only 3 months have excellent long-term survival. […] Furthermore, incomplete normalization of ALT within 6 months of therapy in a study of 133 AIH patients from New Zealand was independently associated with poor outcomes. […] The demarcation of incomplete response requires further management considerations beyond that of biochemical follow-up. […] Despite elimination of contributing hepatic disease or insults, optimization of first-line therapies, and assurance of medical adherence, abnormal liver tests will be present in 93-94% of treated patients at 2 years. […] These patients, along with those intolerant and failing standard therapy are candidates for alternative immunosuppressive treatments.
#17 Management of Difficult Cases of Autoimmune Hepatitis
https://pmc.ncbi.nlm.nih.gov/articles/PMC5410647/
In fact, utilization of early biochemical response may have merit in this arena, as Kanzler et al observed that patients exhibiting a biochemical response in only 3 months have excellent long-term survival. […] Furthermore, incomplete normalization of ALT within 6 months of therapy in a study of 133 AIH patients from New Zealand was independently associated with poor outcomes. […] The demarcation of incomplete response requires further management considerations beyond that of biochemical follow-up. […] Despite elimination of contributing hepatic disease or insults, optimization of first-line therapies, and assurance of medical adherence, abnormal liver tests will be present in 93-94% of treated patients at 2 years. […] These patients, along with those intolerant and failing standard therapy are candidates for alternative immunosuppressive treatments.
#18 Management of Difficult Cases of Autoimmune Hepatitis
https://pmc.ncbi.nlm.nih.gov/articles/PMC5410647/
In fact, utilization of early biochemical response may have merit in this arena, as Kanzler et al observed that patients exhibiting a biochemical response in only 3 months have excellent long-term survival. […] Furthermore, incomplete normalization of ALT within 6 months of therapy in a study of 133 AIH patients from New Zealand was independently associated with poor outcomes. […] The demarcation of incomplete response requires further management considerations beyond that of biochemical follow-up. […] Despite elimination of contributing hepatic disease or insults, optimization of first-line therapies, and assurance of medical adherence, abnormal liver tests will be present in 93-94% of treated patients at 2 years. […] These patients, along with those intolerant and failing standard therapy are candidates for alternative immunosuppressive treatments.
#19 Role of triglycerides as a predictor of autoimmune hepatitis with cirrhosis | Lipids in Health and Disease | Full Text
https://lipidworld.biomedcentral.com/articles/10.1186/s12944-022-01716-9
Metabolism-related indicators have been suggested as possible prognostic indicators of liver disease in recent relevant studies, but their value in predicting autoimmune hepatitis (AIH) cirrhosis is unclear. This study evaluated the role of lipid levels in determining the prognosis of AIH-related cirrhosis. […] The survival analysis showed that TG levels were associated with the overall survival of patients with AIH, as a lower 5-year survival rate (log-rank P0.05) was observed for patients in the TG0.95 mmol/L group (hazard ratio (HR)=3.79, 95% CI: 1.528-9.423). […] Serum TG levels are closely related to the disease severity and overall survival of patients with AIH cirrhosis and may be used as a new indicator of advanced liver disease and long-term prognosis. […] The results suggested that lower TG levels were a risk factor for death in patients with AIH regardless of whether they had cirrhosis or decompensated cirrhosis.
#20 Role of triglycerides as a predictor of autoimmune hepatitis with cirrhosis | Lipids in Health and Disease | Full Text
https://lipidworld.biomedcentral.com/articles/10.1186/s12944-022-01716-9
Metabolism-related indicators have been suggested as possible prognostic indicators of liver disease in recent relevant studies, but their value in predicting autoimmune hepatitis (AIH) cirrhosis is unclear. This study evaluated the role of lipid levels in determining the prognosis of AIH-related cirrhosis. […] The survival analysis showed that TG levels were associated with the overall survival of patients with AIH, as a lower 5-year survival rate (log-rank P0.05) was observed for patients in the TG0.95 mmol/L group (hazard ratio (HR)=3.79, 95% CI: 1.528-9.423). […] Serum TG levels are closely related to the disease severity and overall survival of patients with AIH cirrhosis and may be used as a new indicator of advanced liver disease and long-term prognosis. […] The results suggested that lower TG levels were a risk factor for death in patients with AIH regardless of whether they had cirrhosis or decompensated cirrhosis.
#21 Role of triglycerides as a predictor of autoimmune hepatitis with cirrhosis | Lipids in Health and Disease | Full Text
https://lipidworld.biomedcentral.com/articles/10.1186/s12944-022-01716-9
In conclusion, this study confirmed the effects of blood lipid levels on patients with AIH. Low TG levels were related to advanced liver disease while predicting the long-term survival of patients. TG is a clinically easily detectable biochemical index that may be used as a new indicator of end-stage liver disease or the long-term prognosis of patients with AIH.
#22 Hyperferritinemia and hypergammaglobulinemia predict the treatment response to standard therapy in autoimmune hepatitis | PLOS One
https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0179074
Hyperferritinemia and hypergammaglobulinemia predict the treatment response to standard therapy in autoimmune hepatitis. […] An abnormal iron homeostasis or hyperferritinemia is associated with worse outcome in other chronic liver diseases and after liver transplantation. […] A hyperferritinemia (2.09 times upper limit of normal; Odds ratio (OR) = 8.82; 95% confidence interval (CI): 2.2534.52) and lower immunoglobulins (1.89 times upper limit of normal; OR = 6.78; CI: 1.8724.59) at baseline were independently associated with the achievement of complete biochemical remission upon standard therapy. […] The predictive value increased when both variables were combined to a single treatment response score. […] Patients with a low treatment response score (1) had significantly higher cumulative remission rates in the training (p0.001) and the validation cohort (p = 0.024).
#23 Baseline IL-2 and the AIH score can predict the response to standard therapy in paediatric autoimmune hepatitis | Scientific Reports
https://www.nature.com/articles/s41598-017-18818-5
We note baseline serum ferritin was normal in about half of the adult patients and in the majority of children with pAIH. […] However, higher AIH scores at diagnosis could be validated internally as a predictor of incomplete treatment response to standard therapy in pAIH. […] Additionally, lower IL-2 levels may further indicate an incomplete treatment response.
#24 Hyperferritinemia and hypergammaglobulinemia predict the treatment response to standard therapy in autoimmune hepatitis | PLOS One
https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0179074
Hyperferritinemia and hypergammaglobulinemia predict the treatment response to standard therapy in autoimmune hepatitis. […] An abnormal iron homeostasis or hyperferritinemia is associated with worse outcome in other chronic liver diseases and after liver transplantation. […] A hyperferritinemia (2.09 times upper limit of normal; Odds ratio (OR) = 8.82; 95% confidence interval (CI): 2.2534.52) and lower immunoglobulins (1.89 times upper limit of normal; OR = 6.78; CI: 1.8724.59) at baseline were independently associated with the achievement of complete biochemical remission upon standard therapy. […] The predictive value increased when both variables were combined to a single treatment response score. […] Patients with a low treatment response score (1) had significantly higher cumulative remission rates in the training (p0.001) and the validation cohort (p = 0.024).
#25 Hyperferritinemia and hypergammaglobulinemia predict the treatment response to standard therapy in autoimmune hepatitis | PLOS One
https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0179074
Hyperferritinemia and hypergammaglobulinemia predict the treatment response to standard therapy in autoimmune hepatitis. […] An abnormal iron homeostasis or hyperferritinemia is associated with worse outcome in other chronic liver diseases and after liver transplantation. […] A hyperferritinemia (2.09 times upper limit of normal; Odds ratio (OR) = 8.82; 95% confidence interval (CI): 2.2534.52) and lower immunoglobulins (1.89 times upper limit of normal; OR = 6.78; CI: 1.8724.59) at baseline were independently associated with the achievement of complete biochemical remission upon standard therapy. […] The predictive value increased when both variables were combined to a single treatment response score. […] Patients with a low treatment response score (1) had significantly higher cumulative remission rates in the training (p0.001) and the validation cohort (p = 0.024).
#26 Hyperferritinemia and hypergammaglobulinemia predict the treatment response to standard therapy in autoimmune hepatitis | PLOS One
https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0179074
In conclusion, baseline levels of serum ferritin and immunoglobulins, which are part of the diagnostic work-up of AIH, can be used to predict the treatment response upon standard therapy in AIH-1, although confirmation from larger multicenter studies is pending. […] To our knowledge, SF is the first predictor of subsequent BR upon standard therapy in untreated AIH-1. […] The combination of both values, SF and IgG, complements each other and facilitates a more precise prediction. […] A risk stratification for the treatment response already at the time point of diagnosis with the two clinical routine blood parameters SF and IgG, which can be determined inexpensively, hopefully facilitates an earlier switch to a salvage therapy as discussed above.
#27 Development and validation of a prognostic score for longâterm transplantâfree survival in autoimmune hepatitis type 1
https://pmc.ncbi.nlm.nih.gov/articles/PMC8281048/
No prognostic score is currently available for longterm survival in autoimmune hepatitis (AIH) patients. […] A DutchBelgian prognostic score for longterm transplantfree survival in AIH patients at diagnosis was developed and validated. […] The 10 year liverrelated survival rate was 91% (95% CI 88%94%), the LTfree survival rate was 86% (95% CI 82%90%). […] Median 10 year LTfree survival was 94% (95% CI 90%97%), 79% (95% CI 69%88%) and 44% (95% CI 26%63%) in patients without cirrhosis, compensated cirrhosis and decompensated cirrhosis respectively (p 0.001). […] This novel DutchBelgian AIH survival model consisting of age, ethnicity, cirrhosis and ALT at diagnosis can accurately predict the longterm LTfree survival in AIH patients.
#28 Baseline IL-2 and the AIH score can predict the response to standard therapy in paediatric autoimmune hepatitis | Scientific Reports
https://www.nature.com/articles/s41598-017-18818-5
Although autoimmune hepatitis (AIH) can be treated with corticosteroid-based first-line therapy, incomplete remission is associated with progressive liver fibrosis. […] Therefore, we analysed baseline parameters, including iron homeostasis and cytokine levels, in 60 children with paediatric AIH (pAIH). […] Although baseline immunoglobulins were lower in pAIH children with subsequent complete biochemical remission (BR) upon standard first-line therapy, only lower AIH scores (16 points) could predict BR upon standard therapy in our training and validation cohorts. […] In conclusion, the baseline AIH score could be validated as a predictor of treatment response in pAIH. […] Additionally, low baseline IL-2 may help identify children who need salvage therapy. […] Since persistent inflammatory activity is associated with histological disease progression and reduced survival in AIH, the early identification of patients with an insufficient response to standard therapy is clinically important.
#29 Baseline IL-2 and the AIH score can predict the response to standard therapy in paediatric autoimmune hepatitis | Scientific Reports
https://www.nature.com/articles/s41598-017-18818-5
We note baseline serum ferritin was normal in about half of the adult patients and in the majority of children with pAIH. […] However, higher AIH scores at diagnosis could be validated internally as a predictor of incomplete treatment response to standard therapy in pAIH. […] Additionally, lower IL-2 levels may further indicate an incomplete treatment response.
#30 Baseline IL-2 and the AIH score can predict the response to standard therapy in paediatric autoimmune hepatitis | Scientific Reports
https://www.nature.com/articles/s41598-017-18818-5
Although autoimmune hepatitis (AIH) can be treated with corticosteroid-based first-line therapy, incomplete remission is associated with progressive liver fibrosis. […] Therefore, we analysed baseline parameters, including iron homeostasis and cytokine levels, in 60 children with paediatric AIH (pAIH). […] Although baseline immunoglobulins were lower in pAIH children with subsequent complete biochemical remission (BR) upon standard first-line therapy, only lower AIH scores (16 points) could predict BR upon standard therapy in our training and validation cohorts. […] In conclusion, the baseline AIH score could be validated as a predictor of treatment response in pAIH. […] Additionally, low baseline IL-2 may help identify children who need salvage therapy. […] Since persistent inflammatory activity is associated with histological disease progression and reduced survival in AIH, the early identification of patients with an insufficient response to standard therapy is clinically important.
#31 A diagnostic model of autoimmune hepatitis in unknown liver injury based on noninvasive clinical data | Scientific Reports
https://www.nature.com/articles/s41598-023-31167-w
All the diagnostic criteria of autoimmune hepatitis (AIH) include histopathology. […] Our new model can predict AIH without a liver biopsy. It is an objective, simple and reliable method that can effectively be applied in the clinic. […] The calibration plot suggested that the model had an acceptable accuracy (p0.05). The decision curve analysis suggested that the model had great clinical utility if the value of probability was 0.45. […] Although our model did not include a pathology factor, the predictive power was not much different from the 2008 IAIHG simplified scoring system. […] The validated population in our model showed good discrimination, accuracy, and clinical utility by estimating the risk of AIH. […] The model proposed in this paper could be valuable and clinically meaningful.
#32 Management of Difficult Cases of Autoimmune Hepatitis
https://pmc.ncbi.nlm.nih.gov/articles/PMC5410647/
The recurrence of AIH (rAIH) after transplantation is common and ranges from 8 to 12 percent at one year after transplantation. The 5-year risk of recurrence is 36-68%. Despite high rate of recurrence, graft failure requiring re-transplantation occurs in only 13-23% and the 5-year survival of adults with recurrent autoimmune hepatitis is excellent at 89-100%.
#33 Autoimmune hepatitis – Wikipedia
https://en.wikipedia.org/wiki/Autoimmune_hepatitis
Prognosis 50% survival if untreated, 90% survival if treated […] Without treatment, the ten-year survival rate for individuals with symptomatic autoimmune hepatitis is 50%. However, with treatment, the ten-year survival rate is above 90%. […] Outcomes with liver transplant are generally favorable with a five-year survival greater than 80 percent. […] Presentation and response to therapy may differ according to race. African Americans appear to present with a more aggressive disease that is associated with worse outcomes.
#34 Autoimmune Hepatitis| Cleveland Clinic
https://my.clevelandclinic.org/departments/digestive/medical-professionals/hepatology/autoimmune-hepatitis
In patients responsive to treatment, AIH has a good prognosis. The majority of treated patients will achieve remission and the 10-year survival rate approaches 83.8% to 94%. […] In patients with established cirrhosis at the beginning of treatment, data on prognosis have been conflicting. While in 1 study, treatment in AIH with cirrhosis showed a 10-year life expectancy comparable to non-cirrhotics, another has shown that the 10-year life expectancy is reduced at 64%. […] The outcomes are generally good in those patients who require liver transplantation. In these patients, survival is 75% at 8 years. Recurrence of AIH in the transplanted liver can occur and appears to be more common when prednisone is discontinued. Reintroduction of prednisone and optimization of calcineurin inhibitors usually induces remission. These patients have a similar prognosis as transplanted patients who do not have recurrent AIH.
#35
https://journals.lww.com/hepcomm/fulltext/2022/06000/noninvasive_prediction_of_outcomes_in_autoimmune.12.aspx
The value of noninvasive tools in the diagnosis of autoimmune hepatitis (AIH) related cirrhosis and the prediction of clinical outcomes is largely unknown. […] Approximately 30% of patients with autoimmune hepatitis (AIH) present with cirrhosis at diagnosis, and a further 10% develop cirrhosis during follow-up. […] Therefore, patients with AIH require close and lifelong follow-up. […] The current LSM cutoff points do not have good discriminative capacity for the diagnosis of AIH related cirrhosis with/without PHT, especially long-term after treatment initiation. […] Nonetheless, noninvasive tools (Baveno VI, expanded Baveno VI criteria, and ANTICIPATEPBC continuous model) to triage patients for endoscopy are useful in patients with AIH related cirrhosis.
#36
https://journals.lww.com/hepcomm/fulltext/2022/06000/noninvasive_prediction_of_outcomes_in_autoimmune.12.aspx
The value of noninvasive tools in the diagnosis of autoimmune hepatitis (AIH) related cirrhosis and the prediction of clinical outcomes is largely unknown. […] Approximately 30% of patients with autoimmune hepatitis (AIH) present with cirrhosis at diagnosis, and a further 10% develop cirrhosis during follow-up. […] Therefore, patients with AIH require close and lifelong follow-up. […] The current LSM cutoff points do not have good discriminative capacity for the diagnosis of AIH related cirrhosis with/without PHT, especially long-term after treatment initiation. […] Nonetheless, noninvasive tools (Baveno VI, expanded Baveno VI criteria, and ANTICIPATEPBC continuous model) to triage patients for endoscopy are useful in patients with AIH related cirrhosis.
#37 Management of Difficult Cases of Autoimmune Hepatitis
https://pmc.ncbi.nlm.nih.gov/articles/PMC5410647/
In fact, utilization of early biochemical response may have merit in this arena, as Kanzler et al observed that patients exhibiting a biochemical response in only 3 months have excellent long-term survival. […] Furthermore, incomplete normalization of ALT within 6 months of therapy in a study of 133 AIH patients from New Zealand was independently associated with poor outcomes. […] The demarcation of incomplete response requires further management considerations beyond that of biochemical follow-up. […] Despite elimination of contributing hepatic disease or insults, optimization of first-line therapies, and assurance of medical adherence, abnormal liver tests will be present in 93-94% of treated patients at 2 years. […] These patients, along with those intolerant and failing standard therapy are candidates for alternative immunosuppressive treatments.
#38
https://journals.lww.com/hep/fulltext/2015/11000/prediction_of_short__and_long_term_outcome_in.22.aspx
Autoimmune hepatitis (AIH) is a chronic inflammatory disease characterized by a loss of tolerance toward the hepatocellular epithelium. […] The aim of this study was to elucidate the clinical, serological, and genetic features of remission, relapse, and overall and LT-free survival. […] Patients diagnosed in childhood were at higher risk for relapses (P=0.003), requirement for LTs (P=0.014, log rank), and had a reduced life expectancy (P<0.001, log rank). [...] Detection of soluble liver antigen/liver pancreas antigen (SLA/LP) antibodies was significantly associated with reduced overall and LT-free survival (P=0.037; P=0.021). [...] Cirrhosis, which was evident in 25% at first diagnosis, was found to be a predictor of poor survival and requirement for LT (P=0.003; P=0.009). [...] Diagnosis 18 years, histological cirrhosis at first diagnosis and SLA/LP antibodies are major risk factors for a poor short and long-term outcome. [...] DRB1*04:01 positivity has been identified in association with a favorable clinical outcome.
#39 Development and validation of a prognostic score for longâterm transplantâfree survival in autoimmune hepatitis type 1
https://pmc.ncbi.nlm.nih.gov/articles/PMC8281048/
No prognostic score is currently available for longterm survival in autoimmune hepatitis (AIH) patients. […] A DutchBelgian prognostic score for longterm transplantfree survival in AIH patients at diagnosis was developed and validated. […] The 10 year liverrelated survival rate was 91% (95% CI 88%94%), the LTfree survival rate was 86% (95% CI 82%90%). […] Median 10 year LTfree survival was 94% (95% CI 90%97%), 79% (95% CI 69%88%) and 44% (95% CI 26%63%) in patients without cirrhosis, compensated cirrhosis and decompensated cirrhosis respectively (p 0.001). […] This novel DutchBelgian AIH survival model consisting of age, ethnicity, cirrhosis and ALT at diagnosis can accurately predict the longterm LTfree survival in AIH patients.
#40
https://journals.lww.com/hep/fulltext/2015/11000/prediction_of_short__and_long_term_outcome_in.22.aspx
Autoimmune hepatitis (AIH) is a chronic inflammatory disease characterized by a loss of tolerance toward the hepatocellular epithelium. […] The aim of this study was to elucidate the clinical, serological, and genetic features of remission, relapse, and overall and LT-free survival. […] Patients diagnosed in childhood were at higher risk for relapses (P=0.003), requirement for LTs (P=0.014, log rank), and had a reduced life expectancy (P<0.001, log rank). [...] Detection of soluble liver antigen/liver pancreas antigen (SLA/LP) antibodies was significantly associated with reduced overall and LT-free survival (P=0.037; P=0.021). [...] Cirrhosis, which was evident in 25% at first diagnosis, was found to be a predictor of poor survival and requirement for LT (P=0.003; P=0.009). [...] Diagnosis 18 years, histological cirrhosis at first diagnosis and SLA/LP antibodies are major risk factors for a poor short and long-term outcome. [...] DRB1*04:01 positivity has been identified in association with a favorable clinical outcome.
#41
https://journals.lww.com/hep/fulltext/2015/11000/prediction_of_short__and_long_term_outcome_in.22.aspx
Autoimmune hepatitis (AIH) is a chronic inflammatory disease characterized by a loss of tolerance toward the hepatocellular epithelium. […] The aim of this study was to elucidate the clinical, serological, and genetic features of remission, relapse, and overall and LT-free survival. […] Patients diagnosed in childhood were at higher risk for relapses (P=0.003), requirement for LTs (P=0.014, log rank), and had a reduced life expectancy (P<0.001, log rank). [...] Detection of soluble liver antigen/liver pancreas antigen (SLA/LP) antibodies was significantly associated with reduced overall and LT-free survival (P=0.037; P=0.021). [...] Cirrhosis, which was evident in 25% at first diagnosis, was found to be a predictor of poor survival and requirement for LT (P=0.003; P=0.009). [...] Diagnosis 18 years, histological cirrhosis at first diagnosis and SLA/LP antibodies are major risk factors for a poor short and long-term outcome. [...] DRB1*04:01 positivity has been identified in association with a favorable clinical outcome.
#42 Role of triglycerides as a predictor of autoimmune hepatitis with cirrhosis | Lipids in Health and Disease | Full Text
https://lipidworld.biomedcentral.com/articles/10.1186/s12944-022-01716-9
Metabolism-related indicators have been suggested as possible prognostic indicators of liver disease in recent relevant studies, but their value in predicting autoimmune hepatitis (AIH) cirrhosis is unclear. This study evaluated the role of lipid levels in determining the prognosis of AIH-related cirrhosis. […] The survival analysis showed that TG levels were associated with the overall survival of patients with AIH, as a lower 5-year survival rate (log-rank P0.05) was observed for patients in the TG0.95 mmol/L group (hazard ratio (HR)=3.79, 95% CI: 1.528-9.423). […] Serum TG levels are closely related to the disease severity and overall survival of patients with AIH cirrhosis and may be used as a new indicator of advanced liver disease and long-term prognosis. […] The results suggested that lower TG levels were a risk factor for death in patients with AIH regardless of whether they had cirrhosis or decompensated cirrhosis.
#43 Hyperferritinemia and hypergammaglobulinemia predict the treatment response to standard therapy in autoimmune hepatitis | PLOS One
https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0179074
Hyperferritinemia and hypergammaglobulinemia predict the treatment response to standard therapy in autoimmune hepatitis. […] An abnormal iron homeostasis or hyperferritinemia is associated with worse outcome in other chronic liver diseases and after liver transplantation. […] A hyperferritinemia (2.09 times upper limit of normal; Odds ratio (OR) = 8.82; 95% confidence interval (CI): 2.2534.52) and lower immunoglobulins (1.89 times upper limit of normal; OR = 6.78; CI: 1.8724.59) at baseline were independently associated with the achievement of complete biochemical remission upon standard therapy. […] The predictive value increased when both variables were combined to a single treatment response score. […] Patients with a low treatment response score (1) had significantly higher cumulative remission rates in the training (p0.001) and the validation cohort (p = 0.024).
#44 Hyperferritinemia and hypergammaglobulinemia predict the treatment response to standard therapy in autoimmune hepatitis | PLOS One
https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0179074
In conclusion, baseline levels of serum ferritin and immunoglobulins, which are part of the diagnostic work-up of AIH, can be used to predict the treatment response upon standard therapy in AIH-1, although confirmation from larger multicenter studies is pending. […] To our knowledge, SF is the first predictor of subsequent BR upon standard therapy in untreated AIH-1. […] The combination of both values, SF and IgG, complements each other and facilitates a more precise prediction. […] A risk stratification for the treatment response already at the time point of diagnosis with the two clinical routine blood parameters SF and IgG, which can be determined inexpensively, hopefully facilitates an earlier switch to a salvage therapy as discussed above.
#45 Autoimmune hepatitis – Wikipedia
https://en.wikipedia.org/wiki/Autoimmune_hepatitis
Prognosis 50% survival if untreated, 90% survival if treated […] Without treatment, the ten-year survival rate for individuals with symptomatic autoimmune hepatitis is 50%. However, with treatment, the ten-year survival rate is above 90%. […] Outcomes with liver transplant are generally favorable with a five-year survival greater than 80 percent. […] Presentation and response to therapy may differ according to race. African Americans appear to present with a more aggressive disease that is associated with worse outcomes.
#46 Management of Difficult Cases of Autoimmune Hepatitis
https://pmc.ncbi.nlm.nih.gov/articles/PMC5410647/
Autoimmune hepatitis (AIH) is a complex autoimmune disease characterized by immune-mediated destruction of hepatic parenchyma which can result in cirrhosis, liver failure, and death. […] Similar to other autoimmune liver diseases all AIH is not the same; high risk populations such as African Americans, or those with early disease onset, incomplete normalization of liver tests, and advanced disease at diagnosis have worse overall survival. […] Incomplete response to a treatment regimen is defined by incomplete recovery of clinical symptoms, biochemical data (AST/ALT and IgG), and possibly histologic findings. Current guidelines suggest normalization of aminotransferases and IgG levels as a key therapeutic aim, as the clinical impact of incomplete response has been linked to fibrosis progression, liver-related death or requirement of liver transplantation.
#47 Baseline IL-2 and the AIH score can predict the response to standard therapy in paediatric autoimmune hepatitis | Scientific Reports
https://www.nature.com/articles/s41598-017-18818-5
Although autoimmune hepatitis (AIH) can be treated with corticosteroid-based first-line therapy, incomplete remission is associated with progressive liver fibrosis. […] Therefore, we analysed baseline parameters, including iron homeostasis and cytokine levels, in 60 children with paediatric AIH (pAIH). […] Although baseline immunoglobulins were lower in pAIH children with subsequent complete biochemical remission (BR) upon standard first-line therapy, only lower AIH scores (16 points) could predict BR upon standard therapy in our training and validation cohorts. […] In conclusion, the baseline AIH score could be validated as a predictor of treatment response in pAIH. […] Additionally, low baseline IL-2 may help identify children who need salvage therapy. […] Since persistent inflammatory activity is associated with histological disease progression and reduced survival in AIH, the early identification of patients with an insufficient response to standard therapy is clinically important.
#48 Baseline IL-2 and the AIH score can predict the response to standard therapy in paediatric autoimmune hepatitis | Scientific Reports
https://www.nature.com/articles/s41598-017-18818-5
We note baseline serum ferritin was normal in about half of the adult patients and in the majority of children with pAIH. […] However, higher AIH scores at diagnosis could be validated internally as a predictor of incomplete treatment response to standard therapy in pAIH. […] Additionally, lower IL-2 levels may further indicate an incomplete treatment response.
#49 Baseline IL-2 and the AIH score can predict the response to standard therapy in paediatric autoimmune hepatitis | Scientific Reports
https://www.nature.com/articles/s41598-017-18818-5
Although autoimmune hepatitis (AIH) can be treated with corticosteroid-based first-line therapy, incomplete remission is associated with progressive liver fibrosis. […] Therefore, we analysed baseline parameters, including iron homeostasis and cytokine levels, in 60 children with paediatric AIH (pAIH). […] Although baseline immunoglobulins were lower in pAIH children with subsequent complete biochemical remission (BR) upon standard first-line therapy, only lower AIH scores (16 points) could predict BR upon standard therapy in our training and validation cohorts. […] In conclusion, the baseline AIH score could be validated as a predictor of treatment response in pAIH. […] Additionally, low baseline IL-2 may help identify children who need salvage therapy. […] Since persistent inflammatory activity is associated with histological disease progression and reduced survival in AIH, the early identification of patients with an insufficient response to standard therapy is clinically important.
#50 Development and validation of a prognostic score for longâterm transplantâfree survival in autoimmune hepatitis type 1
https://pmc.ncbi.nlm.nih.gov/articles/PMC8281048/
No prognostic score is currently available for longterm survival in autoimmune hepatitis (AIH) patients. […] A DutchBelgian prognostic score for longterm transplantfree survival in AIH patients at diagnosis was developed and validated. […] The 10 year liverrelated survival rate was 91% (95% CI 88%94%), the LTfree survival rate was 86% (95% CI 82%90%). […] Median 10 year LTfree survival was 94% (95% CI 90%97%), 79% (95% CI 69%88%) and 44% (95% CI 26%63%) in patients without cirrhosis, compensated cirrhosis and decompensated cirrhosis respectively (p 0.001). […] This novel DutchBelgian AIH survival model consisting of age, ethnicity, cirrhosis and ALT at diagnosis can accurately predict the longterm LTfree survival in AIH patients.