Autoimmune Hepatitis and Primary Sclerosing Cholangitis: An Overlap Syndrome Resistant to Azathioprine and Intolerant to Tacrolimus with Sepsis Mimicking Flare UP-A Clinical Dilemma
Patients with autoimmune liver disease in a frequency of 7% -18% fit into the diagnostic criteria for more than one condition. Up to 12.5% of Autoimmune Hepatitis (AIH) and Primary Sclerosing Cholangitis (PSC) cohorts have a label of AIH/PSC overlap. There can be an unpredictable interval of many years between the diagnosis of the two conditions. There are scoring systems but no established criteria for the diagnosis of AIH/PSC overlap. Therefore, a diagnosis based on combination of biochemistry, autoantibody profile, cholangiogram, and liver histologyis made. Histopathology staging of AIH/PSC overlap influences therapeutic options and prognosis. There is beneficial role of immunosuppression, even though there is a higher relapse rate and evidence of progressive liver disease despite immunosuppression in some cases. Liver related outcomes in this overlap are better than PSC alone but are poorer than AIH. We herein report a rare presentation of an overlap syndrome with AIH and PSC in a patient in whom, treatment with first line therapy showed incomplete response and the salvage therapy was not tolerated, presenting with Acute Kidney Injury (AKI) and a clinical picture of sepsis resembling AIH flare.
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Mathews ST, Poonia D, Kumar N, Bhavana M, Ahlawat RS, Singh H et al. Autoimmune Hepatitis and Primary Sclerosing Cholangitis: An Overlap Syndrome Resistant to Azathioprine and Intolerant to Tacrolimus with Sepsis Mimicking Flare up-A Clinical Dilemma. J Adv Res Med 2020; 7(2): 11-14.
Carbone M, Neuberger JM. Autoimmune liver disease, autoimmunity and liver transplantation. J Hepatol
; 60(1): 210-223.
Manns MP, Czaja AJ, Gorham JD, Krawitt EL, Mieli-Vergani G, Vergani D et al. Diagnosis and management
of autoimmune hepatitis. Hepatol Baltim Md 2010; 51(6): 2193-2213.
Hübener S, Oo YH, Than NN, Hübener P, Weiler-Normann C, Lohse AW et al. Efficacy of 6-Mercaptopurine as
Second-Line Treatment for Patients With Autoimmune Hepatitis and Azathioprine Intolerance. Clin
Gastroenterol Hepatol 2016; 14(3): 445-453.
Montano-Loza AJ, Carpenter HA, Czaja AJ. Features associated with treatment failure in type 1 autoimmune
hepatitis and predictive value of the model of endstage liver disease. Hepatol Baltim Md 2007; 46(4):
Chapman R, Fevery J, Kalloo A, Nagorney DM, Boberg KM, Shneider B et al. American Association for the
Study of Liver Diseases. Diagnosis and management of primary sclerosing cholangitis. Hepatol 2010; 51(2):
Czaja AJ. Frequency and nature of the variant syndromes of autoimmune liver disease. Hepatol 1998; 28(2):
Kaya M, Angulo P, Lindor KD. Overlap of autoimmune hepatitis and primary sclerosing cholangitis: an
evaluation of a modified scoring system. J Hepatol 2000; 33(4): 537-542.
Yeoman AD, Westbrook RH, Al-Chalabi T, Carey I, Heaton ND, Portmann BC et al. Diagnostic value and utility of
the simplified International Autoimmune Hepatitis Group (IAIHG) criteria in acute and chronic liver disease.
Hepatol 2009; 50(2): 538-545.
Chazouillères O. Diagnosis of primary sclerosing cholangitis--autoimmune hepatitis overlap syndrome:
to score or not to score? J Hepatol 2000; 33(4): 661-663.
Czaja AJ, Carpenter HA. Autoimmune hepatitis with incidental histologic features of bile duct injury. Hepatol
; 34(4 Pt 1): 659-665.
Lewin M, Vilgrain V, Ozenne V, Lemoine M, Wendum D, Paradis V et al. Prevalence of sclerosing cholangitis
in adults with autoimmune hepatitis: a prospective magnetic resonance imaging and histological study.
Hepatol 2009; 50(2): 528-537.
Yagihashi A, Yoshida Y, Terasawa K, Ujike Y, Konno A, Ogura K et al. The effect of FK 506 on peripheral blood
T-lymphocyte subsets in orthotopic liver transplant patients. Transplant Proc 1991; 23(6): 2937-2938.
Richardson PD, James PD, Ryder SD. Mycophenolate mofetil for maintenance of remission in autoimmune
hepatitis in patients resistant to or intolerant of azathioprine. J Hepatol 2000; 33(3): 371-375.
Jothimani D, Cramp ME, Cross TJ. Role of mycophenolate mofetil for the treatment of autoimmune hepatitis-an observational study. J Clin Exp Hepatol 2014; 4(3): 221-225.
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