Permanent immunosuppression in SLA/LP-positive autoimmune hepatitis is required although overall response and survival are similar

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Permanent immunosuppression in SLA/LP-positive autoimmune hepatitis is required although overall response and survival are similar. / Zachou, Kalliopi; Weiler-Normann, Christina; Muratori, Luigi; Muratori, Paolo; Lohse, Ansgar W; Dalekos, George N.

In: LIVER INT, Vol. 40, No. 2, 02.2020, p. 368-376.

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@article{e2e236c9571a499099a4626471834a98,
title = "Permanent immunosuppression in SLA/LP-positive autoimmune hepatitis is required although overall response and survival are similar",
abstract = "BACKGROUND AND AIMS: Antibodies against soluble liver antigen/liver pancreas (anti-SLA/LP) are highly specific for autoimmune hepatitis (AIH) and have been linked with a more severe clinical course of the disease, frequent relapses after treatment withdrawal and worse outcome. To address definitely the clinical significance of anti-SLA/LP, we investigated a large number of anti-SLA/LP-positive and -negative patients followed in three referral centres.METHODS: Prospectively collected data from 89 anti-SLA/LP-positive AIH patients (29 from Hamburg-Germany, 20 from Bologna-Italy and 40 from Larissa-Greece) were analysed retrospectively. Age- and sex-matched anti-SLA/LP-negative patients served as disease controls (n = 230; 1:2.5 ratio).RESULTS: In respect to baseline characteristics, anti-SLA/LP-positive patients were more frequently asymptomatic compared to anti-SLA/LP-negative (P < .05). However, anti-SLA/LP-positive patients did not differ from anti-SLA/LP-negative in terms of the overall response to treatment, disease progression and survival even though, they were less likely to achieve corticosteroid withdrawal (P < .05), needed longer treatment duration to achieve first complete response (P < .001) and relapsed more frequently after treatment withdrawal compared to anti-SLA/LP-negative patients (P = <.001).CONCLUSIONS: We showed that anti-SLA/LP antibodies do not characterize a group of AIH patients with distinct features and cannot identify patients with a more severe form of the disease or worse survival. Most importantly, however, anti-SLA/LP-positive patients appear to require lifelong immunosuppression as they are less likely to achieve the cessation of corticosteroids and present higher relapse rates after treatment withdrawal. Therefore, close long-term monitoring should be advised in all anti-SLA/LP-positive patients after withdrawal of immunosuppressive treatment.",
author = "Kalliopi Zachou and Christina Weiler-Normann and Luigi Muratori and Paolo Muratori and Lohse, {Ansgar W} and Dalekos, {George N}",
note = "{\textcopyright} 2019 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.",
year = "2020",
month = feb,
doi = "10.1111/liv.14280",
language = "English",
volume = "40",
pages = "368--376",
journal = "LIVER INT",
issn = "1478-3223",
publisher = "Wiley-Blackwell",
number = "2",

}

RIS

TY - JOUR

T1 - Permanent immunosuppression in SLA/LP-positive autoimmune hepatitis is required although overall response and survival are similar

AU - Zachou, Kalliopi

AU - Weiler-Normann, Christina

AU - Muratori, Luigi

AU - Muratori, Paolo

AU - Lohse, Ansgar W

AU - Dalekos, George N

N1 - © 2019 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

PY - 2020/2

Y1 - 2020/2

N2 - BACKGROUND AND AIMS: Antibodies against soluble liver antigen/liver pancreas (anti-SLA/LP) are highly specific for autoimmune hepatitis (AIH) and have been linked with a more severe clinical course of the disease, frequent relapses after treatment withdrawal and worse outcome. To address definitely the clinical significance of anti-SLA/LP, we investigated a large number of anti-SLA/LP-positive and -negative patients followed in three referral centres.METHODS: Prospectively collected data from 89 anti-SLA/LP-positive AIH patients (29 from Hamburg-Germany, 20 from Bologna-Italy and 40 from Larissa-Greece) were analysed retrospectively. Age- and sex-matched anti-SLA/LP-negative patients served as disease controls (n = 230; 1:2.5 ratio).RESULTS: In respect to baseline characteristics, anti-SLA/LP-positive patients were more frequently asymptomatic compared to anti-SLA/LP-negative (P < .05). However, anti-SLA/LP-positive patients did not differ from anti-SLA/LP-negative in terms of the overall response to treatment, disease progression and survival even though, they were less likely to achieve corticosteroid withdrawal (P < .05), needed longer treatment duration to achieve first complete response (P < .001) and relapsed more frequently after treatment withdrawal compared to anti-SLA/LP-negative patients (P = <.001).CONCLUSIONS: We showed that anti-SLA/LP antibodies do not characterize a group of AIH patients with distinct features and cannot identify patients with a more severe form of the disease or worse survival. Most importantly, however, anti-SLA/LP-positive patients appear to require lifelong immunosuppression as they are less likely to achieve the cessation of corticosteroids and present higher relapse rates after treatment withdrawal. Therefore, close long-term monitoring should be advised in all anti-SLA/LP-positive patients after withdrawal of immunosuppressive treatment.

AB - BACKGROUND AND AIMS: Antibodies against soluble liver antigen/liver pancreas (anti-SLA/LP) are highly specific for autoimmune hepatitis (AIH) and have been linked with a more severe clinical course of the disease, frequent relapses after treatment withdrawal and worse outcome. To address definitely the clinical significance of anti-SLA/LP, we investigated a large number of anti-SLA/LP-positive and -negative patients followed in three referral centres.METHODS: Prospectively collected data from 89 anti-SLA/LP-positive AIH patients (29 from Hamburg-Germany, 20 from Bologna-Italy and 40 from Larissa-Greece) were analysed retrospectively. Age- and sex-matched anti-SLA/LP-negative patients served as disease controls (n = 230; 1:2.5 ratio).RESULTS: In respect to baseline characteristics, anti-SLA/LP-positive patients were more frequently asymptomatic compared to anti-SLA/LP-negative (P < .05). However, anti-SLA/LP-positive patients did not differ from anti-SLA/LP-negative in terms of the overall response to treatment, disease progression and survival even though, they were less likely to achieve corticosteroid withdrawal (P < .05), needed longer treatment duration to achieve first complete response (P < .001) and relapsed more frequently after treatment withdrawal compared to anti-SLA/LP-negative patients (P = <.001).CONCLUSIONS: We showed that anti-SLA/LP antibodies do not characterize a group of AIH patients with distinct features and cannot identify patients with a more severe form of the disease or worse survival. Most importantly, however, anti-SLA/LP-positive patients appear to require lifelong immunosuppression as they are less likely to achieve the cessation of corticosteroids and present higher relapse rates after treatment withdrawal. Therefore, close long-term monitoring should be advised in all anti-SLA/LP-positive patients after withdrawal of immunosuppressive treatment.

U2 - 10.1111/liv.14280

DO - 10.1111/liv.14280

M3 - SCORING: Journal article

C2 - 31626725

VL - 40

SP - 368

EP - 376

JO - LIVER INT

JF - LIVER INT

SN - 1478-3223

IS - 2

ER -