Auto immune hepatitis in Libreville: epidemiological characteristics of 11 cases

doi:10.15406/ghoa.2020.11.00416

eISSN: 2373-6372

Gastroenterology & Hepatology: Open Access

Case Report Volume 11 Issue 2

Auto immune hepatitis in Libreville: epidemiological characteristics of 11 cases

Patrice Emery Itoudi Bignoumba,¹

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Maryam Saibou,¹ Agnes Angela Engoang,¹ Arthur Mackossot,¹ Ines Flore Maganga Moussavou,¹ Clausina Ahoui Apendi,¹ Josaphat Iba Ba,² Caroline Magne,³ Monique Mbounja Zue,¹ Soumana Sanou,⁴ Arnaud Georgio Eyi Nguema,¹ Patrick Nzouto,¹ Jean Baptiste Moussavou Kombila¹

¹Department of Hepatology, Gastroenterology and Digestive Endoscopy, University Hospital Center of Libreville, Gabon
²Department of Internal Medicine, Centre Hospitalier Universitaire De Libreville, Gabon
³Department of Internal Medicine, Military Training Hospital, Gabon
⁴Department of Internal Medicine, LA Polyclinique EL Rapha, Gabon

Correspondence: Patrice Emery Itoudi Bignoumba, Department of Hepatology, Gastroenterology and Digestive endoscopy of the University Hospital Center of Libreville, Gabon, Tel +241(66906639)

Received: February 22, 2020 | Published: March 31, 2020

Citation: Bignoumba PEI, Saibou M, Engoang AA, et al. Auto Immune hepatitis in Libreville: epidemiological characteristics of 11 cases. Gastroenterol Hepatol Open Access. 2020;11(2):69-71. DOI: 10.15406/ghoa.2020.11.00416

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Abstract

Introduction: Autoimmune hepatitis is a rare condition that is increasingly reported in the literature. The absence of Gabonese data prompted this study, and the objective was to determine the profile of autoimmune hepatitis in Libreville.

Patients and methods: This is a multicenter, cross-sectional study from January 2013 to December 2018. Foe this study we included patients followed for chronic liver disease in the hepato-gastroenterology and internal medicine departments of the CHU de Libreville, the internal medicine departments of Omar Bongo Ondimba Military Training Hospital, and the El Rapha Polyclinic, meeting the diagnostic criteria of the International Autoimmune Hepatitis Group simplified in 2008.

Results: Among the 252 patients followed for hepatopathy in these centers, 11 met the diagnostic criteria, which is 4.4% as hospital frequency. The median age was 26years. The sex ratio was 2 men for 9 women. Autoimmune hepatitis type 1, with 8 cases (72.7%), was the most common type. It was associated with another autoimmune disease in 6 patients (54.5%). Primary biliary cholangitis accounted for half of the associated autoimmune diseases. The treatment was based on the combination of corticosteroids and Azathioprine in 8 patients (72.7%) and on corticosteroids alone in 3 patients.

Conclusion: Autoimmune hepatitis is pathology, which is certainly rare but present in Libreville that affects young women. It is frequently associated with other autoimmune diseases.

Keywords: autoimmune hepatitis, autoimmune disease, primary biliary cholangitis

Introduction

Autoimmune hepatitis (AIH) is a rare inflammatory liver disease, characterized by the presence of serum autoantibodies, Polyclonal IgG-type hypergammaglobulinemia, periportal lymphocytic infiltration and strong necrotico activity-inflammatory in the absence of viral, medicinal or toxic etiologies.^1–5 In Europe, the prevalence of AIH is estimated between 10 and 17 cases per 100,000 inhabitants⁴ and its incidence is 0.8 to 3 cases per 100,000 inhabitants.^3,4 In Africa, there is no data in the general population, but case series are increasingly published.^5–8 In Gabon, the absence of a study on AIH led us to undertake this work, whose the purpose was to determine the presence of this affection in Libreville and to specify the characteristics of it.

Patients and method

This is a multicenter, cross-sectional study from January 2013 to December 2018. It included patients followed for liver disease in the hepato-gastroenterology and internal medicine departments of the CHU Libreville, the internal medicine departments of Omar Bongo Ondimba Military Training Hospital and the El Rapha polyclinic. We excluded patients with liver disease of infectious etiology (hepatitis A, B and C, hepatic amoebiasis); alcoholic (all patients with alcohol compliance greater than 20g/d); toxic (taking hepatotoxic medication, taking traditional decoctions); overload diseases (non-alcoholic fatty liver disease). The diagnosis was retained according to the diagnostic criteria of the International Autoimmune Hepatitis Group (IAHG) simplified in 2008. The epidemiological, diagnostic and therapeutic aspects were analyzed.

Results

Among the 252 patients followed for hepatopathy, 11 patients met the 2008 IAHG diagnostic criteria for AIH, representing a hospital frequency of 4.4% in a population of patients with hepatopathy. There were 9 women and 2 men. The median age was 26years (±4years) with extremes of 10 and 54 years of age. There were 8 students (72.7%), social assistance, an entrepreneur and one unemployed. The time between the onset of symptoms and the specialist consultation varied from 3months to 5years. The time between the specialist consultation and the diagnosis varied between 2months and 3years.

Table 1 shows the clinical characteristics of the patients. Cholestatic jaundice, portal hypertension, deterioration of general condition and fever were the main clinical manifestations. Biologically, there was biological cholestasis syndrome and hepatic cytolysis syndrome in the 11 patients. The prothrombin level was less than 70% in the 11 patients. Hypoalbuminemia was present in 9 patients (81.8%). Polyclonal gammopathy greater than 25g/L was present in 10 patients (90.9%). The autoimmune workup was positive in 10 patients (90.9%). Table 2 shows the distribution of the autoantibodies found.

Clinical data	Numbers (n)	Percentage (%)
Icterus	11	100
Itching	10	90.9
Alteration of the general state	8	72.7
Splenomegaly	8	72.7
Ascites	8	72.7
Fever	7	63.6
Hepatomegaly	5	44.5
Polyarthralgia	4	36.4
Upper digestive haemorrhage	3	27.3

Table 1 Clinical characteristics of patients

Autoantibodies	Numbers (n)	Percentage (%)
Anti nuclear antibodies	6	54.5
Smooth muscle antibodies	8	54.5
Anti-actin antibodies	2	18.2
Anti-mitochondria antibodies	3	27.3
Anti LKM Antibodies	2	18.2
Anti-SSA antibodies	1	9.1

Table 2 Distribution of the auto antibodies found

The Liver biopsy was performed in 7 patients. It was characteristic of AIH in the 7 patients. It revealed interface hepatitis in 2 patients and a lymphoplasmacytic infiltrate in 5 patients, 3 of them had advanced fibrosis with regenerative nodules. The abdominal ultrasound found a dysmorphic liver in 5 patients (44.5%), homogeneous hepatomegaly in 4 patients (36.4%), ascites with signs of portal ultrasound hypertension in 9 patients (81.8%). The Esogastroduodenal endoscopy found endoscopic signs of portal hypertension in 6 patients (54.5%). The diagnosis of AIH according to the 2008 IAHG diagnostic criteria was certain in 7 patients and probable in 4 patients. There were 8 cases (72.7%) of autoimmune hepatitis type 1.

The association with other autoimmune diseases was found in 6 patients (54.5%). There were 3 cases of primary bile cholangitis (27.3%), 1 case of IgG4 pancreatitis, 1 case of autoimmune thrombocytopenia and 1 case of autoimmune thyroiditis. Therapeutically, the 11 patients had been treated with corticosteroid therapy, 8 had the corticosteroid and Azathioprine combination. Other therapies such as diuretics were used in 8 patients, ursodeoxycholic acid in 3 patients and ligation of esophageal varices in 3 patients. The average follow-up was 2years. The outcome was favorable under treatment in all patients.

Discussion

The Autoimmune hepatitis with a 4.4% hospital frequency of patients with hepatopathy is a rare condition in Libreville.³ Although rare, this series of 11 patients in 6years joins the series of cases published in Africa, especially in Senegal 12 cases in 8 years,⁶ in Tunisia 83 cases in 8years,⁹ in Egypt 34 cases in 5years.⁷ The female predominance found in our study with a sex ratio of 2 men for 9 women is constant in the literature.^–10 The AutoImmune Hepatitis type 1 was the most common form with 8 of 11 patients. This observation is consistent with data from the literature, which estimates its frequency at 80% of AutoImmune Hepatitis.^2–5 The time between the onset of symptoms and the specialist consultation varied between 3months and 5years. This delay, often long, is variable in the literature. It is linked both to the polymorphic and non-specific nature of the symptomatology and to the ignorance of this affection.^5–9

The clinical presentation of the patients, although not specific, dominated by cholestatic jaundice, portal hypertension, deterioration in general condition, fever and joint pain, is consistent with the data in the literature. However, the patients appeared to have been seen late in our series because 8 out of 11 patients had decompensated cirrhosis.^1–10

The diagnosis of AutoImmune Hepatitis is based on a body of arguments that integrate the absence of other etiologies of hepatopathy, the presence of autoantibodies, histological abnormalities and an elevation of IgG type immunoglobulins.^2–4 In our series, the liver biopsy was not performed in 4 patients due to either abundant ascites or blood crase disorders and the impossibility of performing this liver biopsy by transjugular route. This difficulty is also found in some series by Diallo et al in Senegal.⁶ The Liver biopsy is a key element in the management of AIH as essential for the diagnosis, prognosis and monitoring of patients.^2–4 The association with other AutoImmune diseases (6 patients) is a constant also found in our series.^1–10 Primary biliary cholangitis (3 out of 6 patients) was the most common AutoImmune disease associated with AutoImmune Hepatitis.^2,3,9,10

Therapeutically, the treatment of AIH is based on corticosteroid therapy and immunosuppressants.^2–4 Eight patients benefited from a corticosteroid and Azathioprine combination in accordance with the protocol of the European association for the study of the liver (EASL) 2015.¹¹

Conclusion

Autoimmune hepatitis is a rare condition but present in Libreville and affects young women. It manifests as cholestatic jaundice, portal hypertension syndrome, and deterioration of the general condition or fever. The Type 1 AIH is the most common and diagnosis is often late. It is often associated with other autoimmune diseases including primary biliary cholangitis. The evolution was favorable under corticosteroid and Azathioprine.