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Autoimmune hepatitis

Autoimmune hepatitis

Autoimmune hepatitis is chronic hepatitis associated with hypergammaglobulinaemia and the presence of circulating autoantibodies. In most cases it responds favorably to immunosuppressive therapy. Diagnosis is challenging, in general, all other possible causes of chronic hepatitis should be excluded. The case report describes a case of type 1 autoimmune hepatitis in a 69-year-old patient.

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Anamnesis:

The 69-year-old patient was followed up by his GP because of long-term cholestatic hepatic enzymes (GMT, ALP) elevation. He was admitted to 2nd surgical clinic because of an acute deterioration of the condition (jaundice, pruritus and significant progression of hepatic enzymes). Obstruction of the bile duct was not confirmed by MRCP and abdominal CT, so he was subsequently transferred to 4th internal clinic for the purpose of further differential diagnosis of hepatopathy. The patient reported that he noticed his skin was yellow and itchy 4 days before he came to the hospital and his urine was dark and stool was lighter. He did not have dyspeptic difficulties. He felt weak for about half a year. He had vertebrogenic problems, so used intermittently Dorsiflex and Aflamil for about half a year, but last time 2 months ago. A few weeks ago, he also used antibiotics and Legalon for the liver. He did not drink herbal teas, did not eat mushrooms, did not have temperature, was not in contact with infectious disease, alcohol consumption only occasionally.

Physical examination: BP 115/80 mmHg, TT: 36,2 °C
Patient conscious, oriented in place, time and person, active position, normostenic habitus, excessive nourishment, jaundice, skin without cyanosis, eupnoic, without lateralisation, teleangiectases on cheeks. Pupils isocoric, bilateral fotoreaction, subicteric eyeballs, pink conjunctivas, tongue in a mid position, wet, uncovered, normal and symmetrical jugular vein filling, non-palpable lymphatic nodes and thyroid gland, symmetrically palpable pulsation of carotid arteries. Vesicular breathing, without additional breathing phenomenas, regular heart rate, fr.92 / min, abdomen without pain and resistance during palpation, non-palpable liver and spleen, tapottement bilaterally negative, lower extremities without oedemas, pulsations on a. dorsalis pedis, a. tibialis posterior bilaterally palpable.

ECG: sinus rhythm, fr. 91/min., PQ 140 ms, QRS 80 ms, ST without elevation/depression, T posit., without ectopy.

Laboratory results:

Lab. tests (serum): Bil-T 319,3 umol/l*, Bil-D 163,7 umol/l*, AST 12,03 ukat/l*, ALT 12,54 ukat/l*, GMT 4,58 ukat/l*, ALP 2,93 ukat/l*, CRP 25,10 mg/l*, NH3 61,0 umol/l*, feritín 1500 ug/l*, total proteins 58,8 g/l*, albumin 31,2 mg/l*, D-dimer 0,73 mg/l*, f-kappa 42,96 mg/l*, f-lambda 33,13 mg/l*, SLA more than 600*, APCA posit.*, Cu 25,86 umol/l*, the rest of lab. tests normal.
IgA. IgM, IgG, IgD, iron, folate, haptoglobin, transferin, B12, ceruloplasmin, C3, C4, AFP, CA 19-9, CEA, gamaglobulinaem normal. AMA, ASMA, LKM negat.
HBsAg negat., anti HCV negat., anti HIV 1,2 negat., anti HAV IgM negat, anti HEV IgM negat., P. Bunnel negat., EBV IgM negat.

Differential diagnosis:

- Autoimmune hepatitis
- Chronic hepatitis B
- Chronic hepatitis C
- Drug-induced liver disease
- Alcoholic liver disease
- Primary biliary cirrhosis
- Primary sclerosing cholangitis
- Wilson´s disease
- Haemochromatosis

Imaging methods:

Abdominal ultrasound: liver steatosis, cholecystolithiasis, thickened wall of the gallbladder, splenomegaly, hydroureteronephrosis l.sin.
CT angiography of pulmonary artery: Drop in contrast medium in nearly all branches of pulmonary arteries for right lower lung lobe with over 50% obliteration.
Ophthalmological examination: Kayser-Fleischer´s ring not present.
Abdominal CT: ureterolithiasis l.sin., nephrolithiasis l.sin., parapelvic cysts of left kidney, cysts of right kidney.
Echocardiography: without dilatation of heart cavities, without cor pulmonale acutum, without systolic dysfunction, without regional kinetic disorders, without pulmonary hypertension, diastolic dysfunction type I, without valvular disorders.
Gastrofibroscopy: No pathological finding on the mucous membrane of the oesophagus, stomach and duodenum.
Elastography of liver: Liver cirrhosis

Diagnosis:

Type 1 autoimmune hepatitis (positivity of autoantibodies APCA, SLA)

Therapy:

The drug of choice is corticosteroid alone or in combination with azathioprine. Immunosuppressive treatment is life-long in most cases. Alternative treatment is required in corticosteroid resistant patients (cyclosporin A, mycophenolate mofetil, cyclophosphamide, tacrolimus, 6-mercaptopurine or methotrexate). Patients with complications of corticosteroid therapy (osteoporosis) are treated with 2nd generation corticosteroid (budesonide) with less systemic effects. The addition of ursodeoxycholic acid makes it possible to reduce doses of immunosuppressants. Liver transplantation is the method of choice in the final stages of the disease (liver cirrhosis).
Our patient was treated by corticosteroid in combination with ursodeoxycholic acid and PPI as ulcerogenic prevention.

Discussion:

In approximately 25% of patients, the onset of autoimmune hepatitis is acute, sometimes fulminant. In most cases, the disease is diagnosed in chronic stage, often accidentally, based on the finding of increased hepatic enzyme activity. There are 3 types of autoimmune hepatitis. The most common is type 1, which is often associated with other autoimmune diseases, and about 25% of patients already have liver cirrhosis at the time of diagnosis. The response to immunosuppressive therapy is usually good. Clinically and even in response to immunosuppressive treatment, there is no difference between types 1 and 3, so some authors do not recognize type 3 and include these patients in type 1.

Authors declare the case report will not be published in any national or international publications.

Classification ICD-10: K75.4 Autoimmune hepatitis

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Creative Commons LicenseContribution content is subject to licence Creative Commons Uveďte autora-Neužívejte dílo komerčně-Nezasahujte do díla Attribution 3.0 Czech Republic
author: Katarína Demková | organization: 4th Department of Internal Medicine | published on: 2.1.2018 | last modified on: 16.4.2018
citation: Demková Katarína: Autoimmune hepatitis. Multimedia support in the education of clinical and health care disciplines :: Portal of Faculty of Medicine [online] 2018-01-02, last modif. 2018-04-16 [cit. 2018-11-15] Available from WWW: <http://portal.lf.upjs.sk/articles.php?aid=348>. ISSN 1337-7000.
 

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