Ann Ibd. Pg. Med 2017. Vol.15, No.

2 133-136

LIVER CIRRHOSIS FROM AUTOIMMUNE HEPATITIS IN A NIGERIAN WOMAN:

A CASE REPORT
A. C. Jemilohun1 and O. G. Adewoye 2

1. Department of Medicine, Ladoke Akintola University of Technology/Ladoke Akintola University of

Technology Teaching Hospital, Ogbomoso, Oyo State, Nigeria
2. Department of Medicine, Ladoke Akintola University of Technology Teaching Hospital, Ogbomoso, Oyo
State, Nigeria

Correspondence: ABSTRACT
Dr. A.C. Jemilohun Autoimmune hepatitis (AIH) is a rare cause of chronic liver disease (CLD).
Department of Medicine, It presents with varied clinical features from acute hepatitis to CLDs like
LAUTECH Teaching Hospital, chronic viral hepatitis and alcoholic liver disease, making it difficult to
Ogbomoso, Oyo State, diagnose in the absence of a high index of suspicion and adequate
Nigeria laboratory support. Autoantibody-mediated hepatocyte injury is the major
Email: chrislohun2010@hotmail.com. feature of AIH.
We present a 44 year old woman with recurrent jaundice, ascites,
splenomegaly, coagulopathy, negative chronic viral hepatitis screening,
elevated IgG and positive anti-smooth muscle antibody. The patient
responded well to immunosuppressive therapy.
This report brings to the fore the need for physicians to maintain a high
index of suspicion and thoroughly evaluate all CLD cases of seemingly
‘unknown’ etiology for AIH in order to prevent progression to end-stage-
liver-disease, since the disease is highly amenable to immunosuppressive
therapy.

Keywords: Autoimmune hepatitis, Autoimmune liver disease, Chronic liver disease, Nigeria

INTRODUCTION
Autoimmune hepatitis (AIH) is a non-contagious explained on the basis of chronic viral infection, alcohol
chronic inflammatory disease that results from consumption, or exposure to hepatotoxic medications
autoantibody-mediated hepatocyte injury. Auto- or chemicals. 2 As such, it is often a diagnosis of
immune hepatitis, primary biliary cirrhosis and primary exclusion.
sclerosing cholangitis are together classified as
autoimmune liver disease.1 The incidence, prevalence and characteristics of AIH
vary in different geographical regions. On a general
AIH is classified into different types on the basis of note, it has a mean incidence of 1-2 per 100,000 and a
the serum autoantibody profiles. The common point prevalence of 11–17 per 100,000. 2 It is
denominator for all the types is the presence of commoner in women (especially young women) than
hypergammaglobulinemia, precisely IgG.2 Type 1 AIH men with a ratio around 4:1 but it occurs in all age
is defined by the presence of antinuclear antibody groups, races and geographical areas. 2–5 The exact
(ANA), anti-smooth muscle antibody (Anti-SMA), or prevalence of the disease is not known in Nigeria as
both and constitutes 80% of AIH cases.2 It is also most studies on CLD focused on viral hepatitis,
commonly associated with other autoimmune diseases alcoholic liver disease and hepatocellular carcinoma.
like autoimmune thyroiditis, celiac disease and ulcerative Only one case report of AIH in Nigeria was found in
colitis, with about 25% having cirrhosis at presentation.2 our literature search.6 A case of a 9-year-old girl with
Type 2 AIH is characterized by the presence of anti- co-occurring wild-type chronic hepatitis B and
liver kidney microsomal (Anti-LKM) 1 and/or anti- antinuclear antibody-positive autoimmune hepatitis was
LKM 3 and/or anti-liver cytosol 1 (Anti-LC1) reported in Senegal, a West African country.7 We present
antibodies.2 It is commoner in children, acute severe a young woman with liver cirrhosis that resulted from
presentation does occur, and progression to cirrhosis autoimmune hepatitis. The rarity of the disease, as well
commonly follows.2 as the scarcity of reported cases in Nigeria, necessitated
this effort.
The diagnosis of AIH is usually suspected when
ongoing hepatocellular inflammation cannot be

Annals of Ibadan Postgraduate Medicine. Vol. 15 No. 2, December 2017 133

CASE REPORT An abdominal ultrasound scan at our facility showed
A 44-year-old woman with a 4-week history of a normal sized liver (12.6cm) with mildly accentuated
jaundice was referred to our clinic for evaluation. She echotexture. The intrahepatic ducts and the biliary tree
was initially being evaluated for obstructive jaundice were within normal limits. The spleen was enlarged
by a Gastrointestinal Surgeon in our hospital before (14.4cm) with normal echogenicity, both kidneys were
the referral. normal in shape, size and outline, and there was
moderate ascites.
She had a history of dark color urine and pruritus
which was limited to the extremities for which she Viral markers of hepatitis B and C (HBsAg, HBeAg,
was given cholestyramine by the referring doctor. She Anti-HBe, Anti-HBc total, and Anti-HCV) were
no longer had pruritus at the time she presented at our negative. The results of other laboratory tests are as
clinic despite having stopped taking cholestyramine. depicted in Table 1.
There was abdominal pain but no fever or passage of
pale stool. No history of vomiting, diarrhea, or A diagnosis of liver cirrhosis secondary to autoimmune
abdominal swelling. No history of blood transfusion, hepatitis was made at the end of the evaluation. She
intravenous drug abuse, surgery or multiple sexual was commenced on 60mg of Prednisolone which was
partners. Her hemoglobin genotype is AA. She had a to be tapered off to 20mg over 4 weeks. She
similar history of jaundice 7 months before her unilaterally stopped the Prednisolone 12 days after
presentation to us which resolved after receiving commencement because she developed facial swelling.
unspecified treatment at another facility. She was not At clinic presentation after the stoppage of
previously diagnosed with any chronic illness. Prednisolone, jaundice had cleared. She was later placed
on Budesonide 3mg TID and Azathioprine 50mg daily.
She is the fifth of five children in a polygamous family Budesonide was discontinued after 4 weeks. She also
setting. She has five children. No family history of a had oral spironolactone, furosemide and propranolol
similar illness. in the course of treatment. She is currently on only
Azathioprine 50mg daily.
On physical examination, she was cachectic, deeply
jaundiced but not pale. She had bilateral pitting pedal One year since commencement of treatment, she is
edema. The liver span was 8cm by percussion. She stable with no jaundice, ascites or pedal edema. She
had moderate ascites, the blood pressure was normal has also gained some weight despite the loss of
but there was tachycardia (pulse was 112 per minute). previously accumulated fluid. Her weight at first
The remainder of the examination was normal. presentation was 45kg while her current weight is 49kg.

Table 1: Results of relevant laboratory tests of a nigerian female with autoimmune hepatitis

Tests Before Treatment After one year of

treatment
ALT (0-22 IU/L) 347 IU/L 18 IU/L
AST (0-18 IU/L) 162 IU/L 13 IU/L
ALP (0-35 IU/L) - 18 IU/L
Total Bilirubin (up to 20umol/L) 272 umol/L 45 umol/L
Conjugated Bilirubin (up to 5umol/L) 250 umol/L 21 umol/L
Total Protein (58-80g/L) 101g/L 71g/L
Albumin (35-50g/L) 36g/L 45g/L
Platelet 122,000/mm3
Prothrombin Time Test-24s; Control-16s Test-17s; Control- 16s
INR 1.54 1.06
Urine Bilirubin + -
HBsAg Negative Negative
HBV-DNA Undetectable -
ANA Negative -
SMA Positive -
Serum IgG (700-1600) 2230mg/dl -
Alfa fetoprotein 81.7ng/ml -

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DISCUSSION were reassuring. AIH may have cholestatic features that
Autoimmune hepatitis is a chronic inflammatory disease can resemble primary sclerosing cholangitis or primary
of unknown etiology that is characterized by the biliary cirrhosis and overlap with these diseases have
presence of hypergammaglobulinemia, circulating been described in 10%-20% and 2%-8% of cases,
autoantibodies, necroinflammatory changes on hepatic respectively.5
histology and a dramatic response to immuno-
suppressive therapy.2,8 Features like past history of jaundice, pedal edema,
ascites, coagulopathy, splenomegaly and coarsened liver
The exact pathophysiologic mechanism of AIH is on ultrasound in this patient all indicate that the disease
unknown.5 The dominant hypothesis is that AIH is a was long standing and that she had developed liver
disease that develops in a genetically predisposed cirrhosis. While a histological finding of interface
person, who is also exposed to environmental hepatitis is desirable in the diagnosis of AIH, liver
triggering factors.2,5,8 Subsequently, the autoimmune biopsy could not be performed on this patient because
attack is perpetuated possibly via molecular mimicry of coagulopathy at the time of evaluation.
and is favored by the impaired control of regulatory
T-cells.2,5,8 Several triggers have been identified which Anti-inflammatory or immunosuppressive therapy has
include- drugs, viral antigens (hepatitis A, B and C) been the mainstay of treatment of both type 1 and
and herbal agents.2 type 2 disease.2,5 When treated adequately, the 20-year
survival rate for all treated patients exceeds 80%, and
The clinical manifestation of AIH can range from life expectancy is similar to that of age and sex-matched
asymptomatic, acute hepatitis and rarely to fulminant normal subjects from the same geographical area. 2,5
hepatic failure, chronic hepatitis or well-established The treatment is divided into two phases: (i) induction
cirrhosis. About 25–34% may be asymptomatic. 2 of remission, and (ii) remission maintenance. 2,5,9
However, asymptomatic patients commonly become Prednisolone monotherapy and Prednisolone in
symptomatic and thus need to be monitored.2 combination with Azathioprine (AZA) are alternative
induction therapies for AIH; for maintenance,
The immunologic tests that are indispensable for the Prednisolone in combination with AZA and AZA
diagnosis of AIH are serum ANA, SMA, anti-LKM1 monotherapy are superior to Prednisolone
and IgG.2,5,8 Other immunological tests that may be monotherapy.2,5,9 The combination therapy has an
relevant include anti-actin antibody, anti-LKM3, anti- advantage in the reduction of Prednisolone related side
liver cytosol 1, perinuclear antineutrophil cytoplasmic effects. Budesonide may be used as an alternative to
antibodies (pANCA), and antibodies to soluble liver Prednisolone in order to reduce steroid specific side
antigen (SLA) or liver pancreas antigen (LP).2,5,8 effects.2,5,9 We opted for the Budesonide/AZA therapy
because our patient reacted to the Prednisolone
The case at hand had features of chronic hepatocyte monotherapy.
inflammation as evidenced by the moderate elevation
of the transaminases, lower limit serum albumin and Remission of previously symptomatic patients is
high total protein (indicating hypergammaglobuline- defined as a complete nor malization of all
mia). Although the patient had a negative ANA, the inflammatory parameters, including AST, ALT,
presence of elevated serum IgG and a positive SMA bilirubin, IgG, recovery from symptoms and inactive
is enough to diagnose AIH, precisely type 1, in a patient liver histology for at least 2-3 years.9 Some patient could
that had no history of alcohol ingestion and any relapse after cessation of therapy. The ideal thing to
serological marker of hepatitis B and C. The initial do after the first episode of relapse is to use a
dramatic response to prednisolone therapy was quite Prednisolone/AZA combination therapy.2 Alternative
encouraging despite the adverse effect. More reassuring therapies for subsequent relapse include tacrolimus,
is the fact that the patient’s albumin had improved mycophenolate mofetil, cyclosporine, methotrexate,
significantly; the globulin component of the total cyclophosphamide, ursodeoxycholic acid, infliximab
protein, ALT, AST and INR have normalized and the and rituximab. Although there are some encouraging
bilirubin levels nearing normal after one year of results with each of the medications, the treatments
immunosuppressive therapy (Table 1). have not been standardized due to lack of randomized
controlled trials’ data.2,3
The presence of conjugated hyperbilirubinemia and
the initial pruritus could have caused a diagnostic Liver transplantation is the ultimate rescue treatment
dilemma but the disappearance of the pruritus even for all liver diseases but has only a minor role in AIH.9
after cholestyramine was stopped and the finding of Approximately 4% of liver transplantations in both
a normal biliary tree on abdominal ultrasonography the US and Europe are due to AIH.9 The cornerstone
Annals of Ibadan Postgraduate Medicine. Vol. 15 No. 2, December 2017 135
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transplantation by timely diagnosis and adequate Med. 2006; 354:54–66.
immunosuppressive therapy.9 Liver transplantation is, 4. Mieli-Vergani G, Vergani D. Autoimmune
however, required in patients who are refractory to or paediatric liver disease. World J Gastroenterol.
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end-stage liver disease develops.2,5,9 5. van Gerven NM, de Boer YS, Mulder CJ, et al.
Auto immune hepatitis. World J Gastroentero.
CONCLUSION 2016; 22(19):4651–4661.
To the best of our knowledge, this is the second case 6. Otegbayo JA, Akere A, Ola SO, et al.
report of AIH in Nigeria. There is a need to maintain Autoimmune liver disease in a Nigerian woman.
a high suspicion index for the disease and to thoroughly Afr Health Sci. 2010; 10:208–210.
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for AIH in order to prevent progression to end-stage- occurrence of chronic hepatitis B virus infection
liver-disease, since AIH is highly responsive to and autoimmune hepatitis in a young Senegalese
immunosuppressive therapy. girl. Eur J Gastroenterol Hepatol. 2006; 18:927-
929.
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