Autoimmune liver disease - are there spectra that we do not know?

Autoimmune liver diseases (AILDs) are common leading causes for liver cirrhosis and terminal stage of liver disease. They have variable prevalence among patients with liver disease and have two major clinical and biochemical presentations. Autoimmune hepatitis (AIH) is the typical example of hepatocellular AILD, but it can also be presented under a cholestatic pattern. AIH has a scoring diagnostic system and respond in most cases to the treatment with prednisolone and azathioprine. Primary biliary cirrhosis (PBC) is the second most common AILD, with a cholestatic presentation and characterized by positive antimitochondrial antibody (AMA). It has an excellent response and long term outcome with the administration of ursodeoxycholic acid (UDCA). Another AILD that is thought to be a variant of PBC is the autoimmune cholangitis, being a disease that has biochemical and histological features similar to PBC; but the AMA is negative. Primary sclerosing cholangitis (PSC) is a rare entity of AILD that has a cholestatic presentation and respond poorly to the treatment, with the ultimate progression to advance liver cirrhosis in most patients. Other forms of AILD include the overlap syndromes (OS), which are diseases with mixed immunological and histological patterns of two AILD; the most commonly recognized one is AIH-PBC overlap (AIH-PSC overlap is less common). The treatment of OS involves the trial of UDCA and different immunosuppressants. Here we present three case reports of unusual forms of chronic liver diseases that most likely represent AILD. The first two patients had a cholestatic picture, whereas the third one had a hepatocellular picture at presentation. We discussed their biochemical, immunological and histological features as well as their response to treatment and their outcomes. Then, we compared them with other forms of AILD

Autoimmune Hepatitis as a Unique Form of an Autoimmune Liver Disease: Immunological Aspects and Clinical Overview

Autoimmune hepatitis (AIH) is a unique form of immune-mediated disease that attacks the liver through a variety of immune mechanisms. The outcomes of AIH are either acute liver disease, which can be fatal, or, more commonly, chronic progressive liver disease, which can lead to decompensated liver cirrhosis if left untreated. AIH has characteristic immunological, and pathological, features that are important for the establishment of the diagnosis. More importantly, most patients with AIH have a favorable response to treatment with prednisolone and azathioprine, although some patients with refractory AIH or more aggressive disease require more potent immune-suppressant agents, such as cyclosporine or Mycophenolate Mofetil. In this paper, we discuss the immunological, pathological and clinical features of AIH, as well as the standard and alternative treatments for AIH

Liver diseases in pregnancy and outcomes: A retrospective study from Saudi Arabia

Liver diseases unique to pregnancy are common causes of both maternal and fetal mortality and morbidity. We retrospectively studied liver diseases unique to pregnancy, including hyperemesis gravidarum (HG); intrahepatic cholestasis of pregnancy; eclampsia; preeclampsia; hemolysis, elevated liver enzymes, and a low platelets (HELLP) syndrome; and acute fatty liver of pregnancy. We collected data including maternal age, gestational weeks at presentation and at delivery, mode of delivery, number of parity, and laboratory markers at 0, 1 week, and within 24 hours after delivery; from 112 patients (mean age, 29.8 years) from April 2015 - March 2017. SPSS 22 was used for statistical analysis. We The commonest liver disease in pregnancy was pre-eclampsia followed by HG. HG patients were younger compared with those with eclampsia and preeclampsia (P=0.025). Gestational week at presentation and the week of delivery were significantly greater for preeclampsia/eclampsia and HELLP patients compared to HG. Primigravida represented 42.9% of our patients. Fetal complications were reported in 29 (26%) of cases. Of those, 17 had fetal or neonatal death. Fourteen mothers (12.5%) had ICU admission. Pregnancy related liver diseases are important causes for fetal mortality and morbidity. Maternal age and gestational weeks are important predictors of fetal and maternal outcomes.   Les maladies du foie propres à la grossesse sont des causes courantes de mortalité et de morbidité maternelles et foetales. Nous avons étudié rétrospectivement les maladies du foie propres à la grossesse, y compris l'hyperemesis gravidarum (HG); cholestase intrahépatique de la grossesse; éclampsie; prééclampsie; hémolyse, élévation des enzymes hépatiques et syndrome de bas taux de plaquettes (HELLP); et stéatose hépatique aiguë de la grossesse. Nous avons recueilli des données comprenant l'âge maternel, les semaines de gestation à la présentation et à l'accouchement, le mode d'accouchement, le nombre de parité et les marqueurs de laboratoire à 0, 1 semaine et dans les 24 heures suivant l'accouchement; de 112 patients (âge moyen, 29,8 ans) d'avril 2015 à mars 2017. SPSS 22 a été utilisé pour l'analyse statistique. Nous La maladie hépatique la plus courante pendant la grossesse était la pré-éclampsie suivie de l'HG. Les patients atteints de HG étaient plus jeunes que ceux atteints d'éclampsie et de prééclampsie (P = 0,025). La semaine gestationnelle lors de la présentation et la semaine de l'accouchement étaient significativement plus importantes pour les patients prééclampsie / éclampsie et HELLP par rapport à HG. Primigravida représentait 42,9% de nos patients. Des complications foetales ont été rapportées dans 29 (26%) des cas. Parmi ceux-ci, 17 ont eu un décès foetal ou néonatal. Quatorze mères (12,5%) ont été admises à l'USI. Les maladies hépatiques liées à la grossesse sont des causes importantes de mortalité et de morbidité foetales. L'âge maternel et les semaines de gestation sont des prédicteurs importants des issues foetales et maternelles. &nbsp

Intestinal tuberculosis and Crohn’s disease: the dilemma of similarities and misdiagnosis

Differentiating intestinal tuberculosis from Crohn’s disease is one of the most difficult and challenging issues for the gastroenterologist, radiologist and pathologist. The final diagnosis of such cases may need the gathering of all clinical, endoscopic, radiological and pathological data. In the following report three cases of intestinal tuberculosis are described. Two of these were initially misdiagnosed as Crohn’s disease and the third was thought to be ovarian or colonic cancer; a fourth patient was diagnosed as having Crohn’s disease but on presentation was thought to have intestinal tuberculosis. Their clinical presentations, colonoscopic findings, radiological features and the pathological diagnosis are described and the similarities between intestinal tuberculosis and Crohn’s disease as compared to previous reports are discussed

Effect of Pegylated Interferon on Non-Responders and Relapsers with Interferon

Objectives: To assess whether a combination of pegylated interferon (interferon conjugated with polyethylene glycol) and ribavirin can improve the response rate in patients with chronic hepatitis C who either did not respond to (Non-responders), or had relapsed after responding to (Relapsers) standard interferon and ribavirin combination therapy. Patients and methods: In this prospective study, 20 chronic hepatitis C patients (comprising 16 Non-responders and 4 Relapsers to previous treatment with alpha interferon and ribavirin), were treated with pegylated interferon-2b weekly and ribavirin daily for one year. Eleven patients had genotype 4, eight were of genotype 1 and one patient had genotype 3. Response to treatment was determined based on normalisation of liver enzymes and negative viral load (assessed using qualitative HCV RNA PCR) at end of treatment (ETR) and 6 months off treatment (SVR). Results: Seven patients (35%) achieved normalisation of liver enzymes and negative viral load at the end of treatment. However, only 2 patients (10%) managed to retain these levels after six months off treatment. The latter two patients had been previous Relapsers. Conclusion: Combination of pegylated interferon and ribavirin may be beneficial in previous relapsers with standard interferon-ribavirin combination therapy, but is unlikely to achieve sustained virological response in non-responders.

Prevalence and molecular characterization of hepatitis D virus in Saudi Arabia: A single-center study

Background/Aims: Hepatitis D virus (HDV) is a defective RNA virus that is dependent on hepatitis B surface antigen (HBsAg) for transmission and replication. HDV significance arises from the possibility of poor prognosis of hepatitis B virus (HBV) infection. In Saudi Arabia, HDV prevalence varied from 8 to 32% before the HBV vaccination program and ranged from 0 to 14.7% after the vaccination program was started. The last study, performed in 2004, showed a prevalence of 8.6% in hospital-based HBV cases and 3.3% in healthy donors. The aim of this study was to investigate the prevalence and molecular characterization of HDV in chronic hepatitis B (CHB) patients at the King Abdulaziz University Hospital in Jeddah, Saudi Arabia by molecular and serological techniques. To the best of our knowledge, this is the first study to detect HDV at the molecular level in Saudi Arabia. Patients and Methods: The study included samples from 182 CHB patients from Jeddah; 13 samples with HBsAg negative were excluded. Samples were tested for HDV-Ab, viral RNA by reverse transcriptase–polymerase chain reaction (RT-PCR) in the HDV L-Ag region and sequence analysis. Results: The mean age of the participants was 44.36 years; 75.1% of the participants were Saudi nationals, 58% were males. Nine samples were positive for HDV-Ab and four were borderline; all were subjected to RT-PCR amplification. Three of the positive HDV-Ab cases and 1 borderline case were positive by RT-PCR. All the positive cases had HBV genotype D, and the positive RT-PCR cases were positive for HBV DNA. One of the HDV viremic samples was of genotype 1 by sequencing. The prevalence of HDV in the study was 7.7%, which was lower in Saudis (6.3%) than in non-Saudis (11.9%). Conclusion: HDV coinfection does not seem to have an effect on the clinical status of the recruited CHB cases in this study. More studies are needed to investigate the genetic diversity in other areas such as the southern parts of the Kingdom