|Year : 2021 | Volume
| Issue : 1 | Page : 84-87
Autoimmune hepatitis in a Northern Nigerian woman
Muhammad Manko1, Abdullahi Jabir2, Ahmad K Bello3, Al Mustapha Liman4, Shettima K Mustapha1
1 Gastroenterology Unit Department of Medicine, ABU/ABUTH, Zaria, Nigeria
2 Department of Medicine, ABU/ABUTH, Zaria, Nigeria
3 Gastroenterology Unit Department of Medicine, ABU/ABUTH, Nigeria
4 Department of Pathology, ABU/ABUTH, Zaria, Nigeria
|Date of Submission||04-Jun-2020|
|Date of Decision||17-Jun-2020|
|Date of Acceptance||01-Jul-2020|
|Date of Web Publication||28-Apr-2021|
Gastroenterology Unit Department of Medicine, ABU/ABUTH, ABU/ABUTH, Zaria
Source of Support: None, Conflict of Interest: None
Autoimmune hepatitis is an unrelenting inflammation of the liver of unknown etiology. It is a relatively rare disease that commonly affects women and mostly present as chronic hepatitis. It is basically classified into types 1 and 2. The patient is a 56 year old Northern Nigerian woman with background thyroid disease and rheumatoid arthritis who presented with jaundice and fatigue and elevated liver enzymes with increased serum total protein and low serum albumin. Further evaluation showed non-reactive hepatitis B and C markers, features of chronic liver disease on abdominal ultrasound scanning, elevated anti-nuclear and anti-smooth muscle antibodies and increased serum IgG. Liver histology showed features of chronic hepatitis consistent with AIH. Diagnosis of AIH type 1 was eventually made and patient was started on prednisolone and azathioprine combination therapy with improvement of liver function test few weeks after commencement of therapy. To the best of our knowledge, only 2 cases of AIH has been reported in Nigeria, all from Ibadan, Southern Nigeria. No report of AIH from Northern Nigeria. We therefore report a case of AIH type 1 to increase awareness among clinicians.
Keywords: autoimmune, auto-antibody, chronic hepatitis, liver histology, Northern Nigeria
|How to cite this article:|
Manko M, Jabir A, Bello AK, Liman AM, Mustapha SK. Autoimmune hepatitis in a Northern Nigerian woman. J Med Trop 2021;23:84-7
| Introduction|| |
Autoimmune hepatitis (AIH) is an unrelenting inflammation of the liver of unknown etiology. It is a relatively rare disease with a global distribution affecting all ages, gender and ethnic groups. The prevalence differs according to the region. In Europe the prevalence was estimated at 15-25/100,000 population while in Alaska, North America, the prevalence was highest at 42.9/100,000 persons. The disease appears to be less common in Asia with a prevalence ranging between 3 and 8/100,000 persons. In Africa, there is scarcity of epidemiological data on AIH. Females are affected, approximately, 4 times more commonly than males and in females the disease has a bimodal age distribution; in late teens and around menopause. AIH is classified into type 1 and type 2 based on the pattern of auto-antibody in the patient’s serum. Type 1 AIH which is the most common type is identified by the presence of antinuclear antibody and/or anti-smooth muscle antibody and type 2 is defined by the presence of anti-liver/kidney microsomal antibody type 1. Presentations of AIH at the time of diagnosis include chronic hepatitis, cirrhosis and hepatocellular carcinoma but rarely patients can present with acute liver disease. Common symptoms at presentation are fatigue, general feeling of unwell, anorexia and weight loss. There are no specific distinctive features of AIH, and diagnosis is based on a combination of suggestive biochemical, immunological and histological features as well as exclusion of other causes of liver diseases., To the best of our knowledge, only two cases of autoimmune hepatitis have been reported in Nigeria and these reports were from Ibadan, Southern Nigeria., None has been reported from Northern Nigeria. We therefore report a case of autoimmune hepatitis in a middle-aged woman from Northern Nigeria to increase awareness among clinicians on AIH. This will lead to timely diagnosis and treatment and therefore reduce the morbidity and mortality associated with this disease among our patients.
| Case summary|| |
The patient is a 56 year old Kanuri woman who was referred to the Gastroenterology/Hepatology clinic of our hospital, with complaints of jaundice and fatigue of 3 months duration. She had no history of abdominal pain or swelling and no history of pruritus. There was no history suggestive of upper gastrointestinal bleeding or hepatic encephalopathy and no history of fever. Jaundice had however, subsided at the time of presentation. Review of the systems was unremarkable.
She had a medical history of hyperthyroidism requiring treatment with radioactive iodine 4 years prior to presentation, following which she developed hypothyroidism necessitating administration of levothyroxine. She is also being managed for rheumatoid arthritis for which she has not been on any regular medications. She is not a known diabetic or hypertensive patient. She neither had history of cigarette smoking nor ingestion of alcohol or herbal medications.
Physical examination revealed a middle-aged woman, anicteric with no peripheral stigmata of Chronic Liver Disease (CLD). Abdominal examination was essentially normal with normal liver span and no splenomegaly or ascites. There was no obvious goiter but musculoskeletal system examination revealed swan-neck deformity of the left ring-finger with no other joint anomalies.
The patient had liver function tests (LFTs) done at the onset of symptoms which showed elevated bilirubin and liver enzymes with reversal of albumin:globulin ratio [Table 1]. An initial diagnosis of CLD cause was made and the following investigations were ordered including repeat LFTs, abdominal ultrasound scanning and hepatitis B and C viral screening.
Initial abdominal ultrasound scanning showed enlarged liver with increased parenchymal echogenicity but no focal mass lesion. The intra-hepatic biliary channels and vasculature were within normal limit while the repeated scanning about a year later showed a liver of 10cm span with coarsened echo pattern and slightly irregular outline. There were attenuated hepatic veins with no focal mass lesions and normal intrahepatic biliary radicles. HBsAg, anti-HBc, and HBsAb, as well as anti-HCV were all non-reactive. Fasting blood glucose was 4.2mmol/L. Assay for α-Feto-protein was 10ng/ml. This initial evaluation further confirmed the diagnosis of CLD and with the patient having a background thyroid disease and RA and with no history of alcohol ingestion and negative hepatitis B and C serology, we made a provisional diagnosis of AIH likely type 2.
Tests to confirm type 2 AIH were then carried out including antinuclear antibody (ANA), anti-smooth muscle antibody (ASMA), serum immunoglobulin (IgG) concentration and liver biopsy. The results were as follows: ANA titre of 1:80, total serum IgG of 4320g/L (7-16), ASMA titre of 1:160. Liver histology showed severe necroinflammatory activity indicated by periportal inflammation with bridging and lobular hepatitis [Figure 1]a. The predominant inflammatory cells infiltrates were plasma cells and lymphocytes [Figure 1]b and the hepatocytes formed rosettes focally with feathery and balloon degenerations in areas [Figure 1]c.
|Figure 1: H&E (a) x100. Photomicrograph of the liver showing periportal mononuclear inflammatory infiltrates with bridging necrosis and lobular hepatitis. (b) x400: section showing extensive infiltration by mononuclear cells mainly plasma cells and polymorphonuclear cells. some hepatocytes exhibit degenerative changes. (c) x400: section showing hepatic rosette and ground glass appearance.|
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A final diagnosis of CLD secondary to Type I AIH was made and she was commenced on Oral Prednisolone at 60mg/day and tapered down. The patient was subsequently commenced on Azathioprine 50mg daily after 12 weeks of steroid therapy. Repeat liver function test after 6 weeks of steroid therapy showed normalization of liver enzymes and serum bilirubin [Table 1].
| Discussion|| |
AIH is a progressive chronic hepatitis of unknown etiology. It is thought to result from loss of immune tolerance towards the hepatocytes that leads to progressive liver damage. Diagnosis of AIH is based on a combination of biochemical, immunological, histological characteristics and exclusion of other causes of liver disease. AIH is predominantly a disease of females that affect children and young adults. The clinical manifestation follows a fluctuating course and the presentation ranges from asymptomatic to markedly symptomatic cases and rarely fulminant hepatic failure., Diagnosis of AIH is simplified using the 2008 revised criteria of International AIH Group (IAIHG) which has 4 key elements: increased serum IgG levels, presence of serum autoantibodies, liver histology and absence of viral hepatitis markers.
The index case is a 56year old woman with a 3 months history of jaundice which has cleared before presentation and fatigue. On examination there was no jaundice and no stigmata of chronic liver disease. Abdominal examination was essentially not remarkable with no palpable liver and normal liver span. No ascites was demonstrable. Initial Liver function tests at onset of symptoms showed abnormal liver enzymes. ([Table 1]). This indicates the insidious onset of the disease in this patient. Fatigue which the patient has is a common symptom of AIH and up to half of patients will have history of jaundice even with insidious onset of the illness. This patient has thyroid disease and rheumatoid arthritis which also supports the diagnosis of AIH. Up to 50% of AIH patients will have other autoimmune diseases. In total, 10% to 23% of AIH patients will have thyroiditis and 2% to 5% will have rheumatoid arthritis.
The liver function test of the patient showed features of chronic hepatitis as shown by moderately elevated liver enzymes and low albumin with high total protein. The elevated aminotransferases were more marked than the rise in alkaline phosphate and bilirubin which is consistent with AIH. Likewise the finding of elevated globulin fraction of the serum is characteristic of AIH. The markedly high serum IgG seen in this patient is one of the criteria for diagnosis of AIH according to 2008 revised IAIHG diagnostic criteria. Presence of autoantibodies are the hallmark of AIH. In type 1 AIH, presence of ANA and/or ASMA in the patient’s serum is one of the diagnostic criteria. Both the ANA and ASMA were above the cut off for diagnosis in our patient which showed that she most likely has type I AIH.
Liver histology is an important component in the diagnostic work-up and follow-up of patients with AIH. The histology of AIH is that of chronic hepatitis with characteristic but non-specific features such as interface hepatitis, rosetting and emperipolesis., Liver histology of our patient showed features of chronic hepatitis characterized by periportal inflammation with bridging and lobular hepatitis. The predominant inflammatory cells were plasma cells and lymphocytes. Other findings noted were areas of hepatic rosette and feathery and balloon degenerations [Figure 1]a-c. These histological changes are consistent with AIH though not entirely characteristic. Diagnosis of AIH can still be made without typical histological features.
The IAIHG decidedly devised the simplified scoring system, with very high sensitivity and specificity, for wider applicability in routine clinical practice which thus proposed the diagnosis of probable AIH and definite AIH on a candidate-criteria that included sex, age, autoantibodies, immunoglobulins, absence of viral hepatitis and histology. Using this clinical evaluation tool, the index case squarely fits definite type entity. It is noteworthy also, that, without going into the complex standardized scoring system reported by Alvarez and coworkers, the application of the revised descriptive criteria of the earlier IAIHG system will similarly reaffirm the diagnosis as definite AIH.
Our patient was commenced on oral prednisolone, initially 60mg once daily and tapered gradually. Subsequently azathioprine 50mg once daily was added after 12 weeks of commencement of the steroid. The decision to commence therapy was based on the presence of fatigue, ongoing hepatic necro-inflammation and the liver cirrhosis which are all indications of treatment in patients with AIH, The two widely accepted treatment regimen are use of steroid (prednisolone or Prednisone) alone or combination of steroid (at reduced dose) with azathioprine., We decided to use the combination regimen so as to avoid steroid-related complications such as diabetes, emotional instability and osteoporosis. In moderate/severe AIH, serum ALT usually falls within two weeks of treatment with prednisolone with or without azathioprine similar to what was obtained in our patient after 6 weeks of prednisolone therapy when LFTs was repeated. Histological remission lags behind biochemical remission and it is important for the patient to be on follow-up including a follow-up of liver biopsy because the disease can progress despite treatment or relapse.
In conclusion, AIH is a relapsing and remitting CLD that can progress to liver cirrhosis. It should be suspected in any patient with liver disease and absence of hepatitis B and C.
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Conflicts of interest
There are no conflicts of interest.
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