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Autoimmune Liver Diseases (AILD) are spectrum of autoimmune disease primarily involving the liver although other systems may also be involved. It mainly includes Autoimmune Hepatitis (AIH), Primary Biliary Cirrhosis (PBC) & Primary Sclerosing Cholangitis (PSC). These diseases usually occur in isolation but sometimes their features overlap which is a distinct entity known as overlap syndrome, this distinction is important as both the treatment and prognosis differs1. Here we present an interesting case of autoimmune liver disease which has typical features of PBC along with certain features of AIH although not sufficient to be categorized in overlap syndrome.
Our patient is a 60 year old female resident of Uttar Pradesh a known case of T2 DM and hypothyroidism presented to medicine OPD with complaints of easy fatigability for two months and generalized itching for one month along with right hypochondrial pain for fifteen days. There is no history of any fever, jaundice or clay colored stools. No h/o breathlessnesss, palpitation or chronic cough. On Examination patient was conscious cooperative with stable vital parameters. GPE showed mild pallor, no icterus, clubbing or lymphadenopathy. On systemic examination there is evidence of hepatomegaly 6cm BCM which was nontender firm in consistiency with sharp regular margins, spleen was also palpable 2cm BCM. No evidence of free fluid or dilated veins over abdomen. Rest of the systemic examination was normal. Investigations showed Hb- 8.2 g% TLC 8,400 Plt.- 2lacs. LFT Bil-0.8 AST-186 u/ml ALT-164u/ml ALP-760 iu/ml TProtein-7.5g% Alb-2.5g% PT-13/13 Ultrasound showed Liver – 17 cm heterogenous echotexture, No SOL GB – Normal PV – 12mm with hepatopetal flow, CBD – Normal Spleen – 14 cm enlarged, normal echo, no SOL. Upper GI Endoscopy showed single column of grade 1 esophageal varix. Hbs Ag, Anti HCV and HIV were negative TSH -60(0.5-5.5)iu/ml Anti TPO 112.9 U/ml (1-10) AMA – POSITIVE (4+) ANA – Neg (Cytoplasmic granularity 3+) ASMA – Neg ASLA – Neg Anti LKM – Positive (1+) Anti dsDNA – Neg Anti SSA/SSB – Neg Anti Centromere – Neg Anti Topoisomerase ( Scl-70) – Neg Anti U1 RNP – Neg IgM levels – 7.58 g/l (0.4-2.4) IgG levels - 26.8 g/l (7-16) MRCP did not show any evidence of intrahepatic biliary radical dilation. Considering above findings liver biopsy was done which showed lobular architecture disarray with portal to portal bridging fibrosis with focal ballooning of hepatocytes. Portal tracts show edematous expansion with moderate chronic inflammation and interface hepatitis. Bile duct injury and bile ductuler proliferation is present. Copper associated protein is present indicative of chronic cholangiopathy. (Fig1)
PBC is an autoimmne disease of the liver with predisposition to intrahepatic ducts, leading to choleststic manifestations, seen mainly in middle aged to elderly females. Apart from historical pointers such as pruritis, presence of easy fatigability is also a common feature (60-80%)2. Biochemically ALP is usually raised although AST/ALT may be slightly raised. Bilirubin generally rises in advanced disease or after development of cirrhosis2. The serological hallmark is the presence of AMA which is seen in 95% of cases, in AMA negative cases antibody against PDCE2 or 2-oxo-glutaric acid dehydrogenase complex is usually detected. Liver biopsy may not be necessary for diagnosis but is important in assessing severity of disease or when there are certain atypical manifestations such as AMA-ve cases or raised AST/ALT3. Our patient is an elderly female with typical clinical features of PBC. But on biochemical analysis apart from ALP, AST& ALT are also significantly raised. On serological assessment both AMA and anti LKM 1 were positive and liver biopsy showed evidence of both cholestatic and interface hepatitis. These atypical features made us think are we dealing with PBC/AIH overlap syndrome? As this distinction is important because overlap syndrome has poorer prognosis and need long term immunosupressants as an additional therapy. To make a diagnosis of AIH modified diagnostic criteria of the International Autoimmune Hepatitis Group should be fulfilled which was being fulfilled in our case. But if we use the extended criterion i.e Revised Original Scoring System of the International Autoimmune Hepatitis Group which gives points to various parameters our patient falls in the probable diagnosis (10-15), for definite diagnosis(>15) score is needed4. Also the “Paris Criterion” (Table1) which was first developed by Chazouillères etal5 and later used in various studies of overlap syndrome was not fulfilled by our case. Hence considering the above features we made a final diagnosis of PBC and patient was started on 15mg/kg UDCA and cholestyramine to which patient responded both symptomatically and biochemically.
PBC has been extensively reported in western literature, its incidence ranged from 0.7–49 cases per million per year in most recent studies and is found to be increasing6. Role of environmental toxins and certain infectious agents have been highlighted as potential risk factors. However not much indian data is available. Case series by Sarinetal have analysed 15 cases of PBC and followed for 10 years have shown it to be a rare cause of liver disease unlike west, and observed that patient usually present late with features of cirrhosis and decompensation7. Overlap syndrome have been described as a separate entity and its correct and timely diagnosis is important as prognosis & treatment differs.
PBC is now a well established cause of chronic liver disease, its early diagnosis and treatment is of utmost importance to prevent decompensation. This report highlights a case of PBC with certain atypical features, making its distinction from overlap syndrome difficult. But by applying the above mentioned criterions we are able to rule out simultaneous presence of AIH.
Table 1: Paris criteria.
|PBC criteria:||AIH criteria:|
1) serum ALP levels at least two times the upper limit of normal values or serum GGT levels as least five times the upper limit of normal values
2) a positive test for AMA
3) a liver biopsy specimen showing florid bile duct lesions
1) serum ALT levels at least five times the upper limit of normal values
2) serum IgG levels at least two times the upper limit of normal values or a positive test for SMA3) a liver biopsy showing moderate or severe periportal or periseptal lymphocytic piecemeal necrosis
PBC-AIH overlap syndrome: 2 out of 3 criteria in each group are met either simultaneously or consecutively
- Wagle SD, Jagtap SP, Wadia F: Primary biliary cirrhosis and autoimmune overlap syndrome. Indian J Gastroentrology 2004,23:70-71.
- Amarapurkar DN, Patel ND: Spectrum of autoimmune liver diseases in Western India. J of Gastroentrol and Hepatol 2007,22:2112-2117.
- Samanta AKS, Bhagwat AG, Mukherjee M etal: Primary biliary cirrhosis in India. Gut 1973, 14:448-450.
- Alvarez F, Berg PA, Binachi FB, et al.: International autoimmune hepatitis group report: review of criteria for diagnosis of Autoimmune hepatitis. J Hepatol 1993, 31:929-938.
- Chazouillères O1, Wendum D, Serfaty Letal Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome: clinical features and response to therapy. Hepatology.1998 Aug;28(2):296-301.
- Nadya Al-Harthy and TeruKumagi Natural history and management of primary biliary cirrhosis. Hepat Med. 2012; 4: 61–71.
- Sarin SK, Monga R, Sandhu BS, Sharma BC, Sakhuja P, Malhotra V: Primary biliary cirrhosis in India. Hepatobilliary Pancreat Dis Int 2006, 5:105-109.