Real-time PCR detection of Helicobacter pylori clarithromycin resistance in Thrace, Greece.

BACKGROUND: Growing resistance to clarithromycin is a major concern regarding treating Helicobacter pylori (H. pylori) infection. Resistance rates have a great variation even in different geographic areas within the same country and are associated with point mutations of the microbial 23S rRNA (A2142C, A2142G, and A2143G). Given the absence of available data in Thrace, the objective of this study was to estimate the resistance of H. pylori to clarithromycin and identify specific mutations that contribute to clarithromycin resistance. METHODS: In this prospective study, we enrolled consecutive patients referred for dyspeptic complaints who underwent upper gastrointestinal endoscopy over two years. Gastric biopsies from corpus and antrum were initially tested for the presence of urease by a rapid urease test. Urease positive samples were followed by real-time PCR to confirm the presence of H. pylori and to detect point mutations. RESULTS: A total of one hundred and thirty patients were included in the study (72 women and 58 men). Resistance to clarithromycin was detected at 23.2 %. Neither gender nor age was independently correlated with resistance rate in our patient group. The most common mutations were A2142G and A2143G. CONCLUSIONS: A high rate of H. pylori resistance to clarithromycin was observed in our region, implicating that it should be addressed in accordance with the recommendations provided by national and international guidelines. Molecular testing should be considered an integral tool for effective monitoring in case of suspected antibiotic resistance. HIPPOKRATIA 2021, 25 (2):51-55.

Smoking habits and gallbladder disease: a systematic review and meta-analysis study.

BACKGROUND: It has been claimed that smoking is linked with an increased risk for gallbladder disease (GBD); however, related issues need further consolidation and clarification. The present systematic review and meta-analysis aimed to further investigate the potent correlation between GBD and smoking. METHODS: We conducted a comprehensive literature review to identify every study published from January 1989 to December 2019, reporting risk estimates regarding GBD and smoking. The random-effect, generic inverse variance method, according to description by DerSimonian and Laird, was used to compute pooled estimates. We used the Newcastle-Ottawa quality assessment scale to appraise the included studies' quality. RESULTS: Thirty published case-control, cross-sectional, and cohort studies including 4,623,435 individuals met the eligibility criteria and were considered for data synthesis. Compared to the non-smokers, ever smokers had 1.25 times higher odds of developing GBD [95 % confidence interval (CI): 1.09-1.44]; however, increased heterogeneity was observed (I2 =96 %, 95 % CI: 62-100 %, p <0.001). Publication bias was non-significant (Eggers' regression p =0.072). The main sources of heterogeneity, as detected by meta-regression analyzing study characteristics, biases and confounders, were non-adjustment for family history (p =0.007) and alcohol (p =0.020), respectively. Subgroup analysis indicated a comparable risk for GBD as far as current, former and ever smokers are concerned (p =0.520). Quantitative analysis suggested a dose-effect for current smoking and GBD (p =0.010). CONCLUSIONS: Non-smokers were demonstrated to be at a lower risk of presenting GBD when compared with ever smokers; all relevant risk estimates necessitate adjustment for family history and alcohol intake. HIPPOKRATIA 2020, 24(4): 147-156.

Factors predicting a positive capsule endoscopy in past overt obscure gastrointestinal bleeding: a multicenter retrospective study.

OBJECTIVES: Capsule endoscopy (CE) remains the examination of choice for the investigation of obscure gastrointestinal bleeding. Although the factors predicting positive CE findings in the overall obscure gastrointestinal bleeding have been investigated, the clinical characteristics that predict a positive CE in patients with past overt obscure gastrointestinal bleeding (OOGIB) have not been systematically studied. METHODS: Between September 2004 and December 2013, 262 patients underwent CE for evaluation of past OOGIB after negative upper and lower endoscopy, and other diagnostic modalities. Patients' records were retrospectively reviewed to assess the factors that could possibly predict positive CE findings. RESULTS: Two hundred and twenty four patients with a median age of 70 years (range: 17-87) were enrolled in the final analysis and were divided into two groups; those who had positive (group A: 118 patients) and those who had negative CE findings (group B: 106 patients). The overall diagnostic yield of CE was 52.68 %. Multivariate analysis demonstrated that age >65 years, anticoagulant use, antiplatelet use, and non-steroidal anti-inflammatory drugs use were independent predictive factors for positive findings on CE. Of the 118 patients with positive CE, therapeutic interventions were performed in 56 patients (47.46 %). Recurrence of bleeding presented in nine patients of group B compared with 39 patients of group A (p <0.001). CONCLUSIONS: Certain clinical characteristics predict a positive CE in patients with past OOGIB. Patients with OOGIB and negative CE had a considerably lower rebleeding rate, and further invasive investigational procedures may be adjourned or may not be required, though such recommendation warrants further validation. Hippokratia 2016, 20(2): 127-132.

Association of antiviral therapy with reduced disease progression in chronic Hepatitis B patients: Results from a nation-wide cohort study.

BACKGROUND AND AIMS: Although effective treatment in terms of inducing virological and biochemical response for chronic hepatitis B (CHB) is available, its effect on the clinical course of the disease has not yet been accurately estimated. Objective of this study was to evaluate the effect of antiviral therapy and its type [interferon +/- nucleos(t)ide analogs (NAs) vs. NAs] on the occurrence of a clinical event (liver decompensation, liver transplant, hepatocellular carcinoma and death from a liver-related cause) in CHB patients. METHODS: The study population was derived from the HEPNET-Greece, a nationwide cohort study aimed to evaluate the current epidemiological course of viral hepatitis. To account for time-dependent confounding, Cox marginal structural models were used to analyze data. RESULTS: Thirty out of 2,125 eligible patients experienced a clinical event during their follow-up. When comparing treated to untreated individuals, the hazard ratio (HR) for a clinical event was 0.39 (95% CI: 0.16-0.98; p =0.044) in the whole sample, whereas there were indications of a more intense effect in the subgroup of patients with cirrhosis at presentation (HR =0.16, 95% CI: 0.02-1.21; p =0.075). The effect of Interferon initiated treatment was not significantly different of that of NAs. There was some evidence, albeit not statistically significant, of a protective treatment effect on hepatocellular carcinoma development (HCC). CONCLUSIONS: Data from observational studies can provide useful inference, provided they are analyzed appropriately. The current study has shown that the available treatment options for CHB offer a significant clinical benefit to CHB infected individuals. Hippokratia 2016, 20(3): 214-221.

Hepatocellular carcinoma risk in HBeAg-negative chronic hepatitis B patients with or without cirrhosis treated with entecavir: HepNet.Greece cohort.

Hepatocellular carcinoma (HCC) may still develop in chronic hepatitis B (CHB) patients treated with lamivudine. Whether HCC rates are comparable in patients treated with the current first-line antivirals remains uncertain. We estimated the incidence and evaluated predictors of HCC in a large nationwide prospective cohort (HepNet.Greece) of HBeAg-negative CHB patients treated with entecavir. HBeAg-negative CHB patients from the same cohort who were initially treated with lamivudine were used as controls. We included 321 patients treated with entecavir for a median of 40 months and 818 patients treated initially with lamivudine for a median of 60 months. In the entecavir group, HCC developed in 4 of 321 (1.2%) patients at a median of 1.5 (range: 1.0-4.5) years, while the cumulative HCC incidence was significantly higher in cirrhotics than noncirrhotics (1, 3, 5 years: 0%, 3%, 9% vs 1%, 1%, 1%; P = 0.024) and in older patients (P = 0.026). Entecavir compared with lamivudine group patients had lower HCC incidence (1, 3, 5 years: 0.3%, 1.2%, 2.8% vs 0.7%, 3.8%, 5.6%; P = 0.024). However, in multivariable Cox regression analysis, the HCC risk was independently associated with older age (P < 0.001), male gender (P = 0.011) and cirrhosis (P = 0.025), but not with the initial agent. In conclusion, our large nationwide study indicates that the HCC risk remains increased in entecavir-treated HBeAg-negative CHB patients with cirrhosis, particularly of older age, at least for the first 5 years. The HCC risk does not seem to be significantly reduced with entecavir compared with antiviral therapy starting with lamivudine.

Can capsule endoscopy be used as a diagnostic tool in the evaluation of nonbleeding indications in daily clinical practice? A prospective study.

OBJECTIVE: To evaluate the diagnostic yield of capsule endoscopy (CE) and its impact on treatment and outcome in patients without bleeding indications. SUBJECTS AND METHODS: One hundred and sixty-five nonbleeding patients were enrolled in the study. The most common indications for CE were chronic abdominal pain alone (33 patients) or combined with chronic diarrhea (31 patients) and chronic diarrhea alone (30 patients). Among the 165 patients, 129 underwent CE for evaluation of gastrointestinal symptoms and 36 for surveillance or disease staging. RESULTS: CE findings were positive, suspicious and negative in 73 (44.2%), 13 (7.9%) and 79 (47.9%) of cases, respectively. The diagnostic yield was highest in patients with refractory celiac disease (10/10, 100%) and suspected Crohn's disease (5/6, 83.3%), followed by patients with chronic abdominal pain and chronic diarrhea (13/31, 41.9%), established Crohn's disease (2/6, 33.3%), chronic diarrhea alone (8/30, 26.7%), chronic abdominal pain alone (8/33, 24.2%) and other indications (3/13, 23.1%) (p < 0.005). The CE findings led to a change of medication in 74 (47.7%) patients, surgery in 15 (9.7%), administration of a strict gluten-free or other special diet in 13 (8.4%) and had other consequences in 11 (6.7%). Management was not modified in 42 (27.1%) patients. Among symptomatic patients (n = 129), 29 (22.5%) were lost to follow-up. The remaining 100 patients were followed up for 8.7 ± 4.0 months (range 2-19). Among the latter, resolution or improvement of symptoms was observed in 86 (86%) patients, no change in 11 (11%) and 3 (3%) died. All 86 patients who experienced resolution or improvement of their symptoms had a modification of their management after CE; only 7/11 patients whose symptoms did not change (63.6%) and 2/3 patients who died (66.7%) had a modification of management (p < 0.001). CONCLUSIONS: CE appears to be a useful tool in the evaluation of patients with nonbleeding indications. The outcome of most patients with negative findings was excellent.

Influence of ghrelin on the gastric accommodation reflex and on meal-induced satiety in man.

Ghrelin increases gastric tone in the fasting state and enhances gastric emptying in gastroparesis. The aims of the study were to evaluate the effect of ghrelin on postprandial gastric tone and on meal-induced satiety in health. Ten healthy volunteers underwent a barostat study on two occasions. After determination of intra-abdominal pressure (minimal distending pressure, MDP), isobaric volume measurement was performed for 90 min at MDP + 2 mmHg. After 20 min, ghrelin (40 microg) or saline was administered i.v. over 30 min in a double-blind-randomized cross-over design, followed 10 min later by a liquid meal (200 mL, 300 kcal). Stepwise isobaric distentions (+2 mmHg per 2 min) were performed 60 min after the meal. Data (mean +/- SEM) were compared using paired Student's t-test and ANOVA. Separately, a satiety drinking test (15 mL min(-1) until satiety score 5) was performed on 10 subjects twice, after treatment with placebo or ghrelin. Ghrelin infusion significantly inhibited gastric accommodation (mean volume increase adjusted means 108.0 +/- 50 vs 23.0 +/- 49 mL, P = 0.03, ANCOVA with the premeal postinfusion volume as covariate) and reduced postprandial gastric volumes (197.2 +/- 24.6 vs 353.5 +/- 50.0 mL, P = 0.01). Pressures inducing perception or discomfort during postprandial gastric distentions were not altered. During satiety testing, ghrelin did not alter nutrient volume ingested till maximal satiety (637.5 +/- 70.9 vs 637.5 +/- 56.2 mL, ns). Ghrelin administered during the meal significantly inhibits gastric accommodation in health, but this is not associated with early satiation.

Recurrence of HCV infection in a sustained responder after chemotherapy for non-Hodgkin's lymphoma: successful retreatment.

It is known that sustained virological response (SVR) in patients with chronic hepatitis C is associated with sustained elimination of hepatitis C virus (HCV) and that late relapse after SVR in HCV patients is doubtful. A 47-year-old man with chronic hepatitis C genotype 3, achieved SVR after combination treatment with pegylated interferon and ribavirine for 6 months. Sixteen months later non-Hodgkin's lymphoma was diagnosed. After successful completion of chemotherapy for non-Hodgkin's lymphoma, he presented with HCV infection recurrence of the same genotype. Retreatment with the same schedule resulted in normalization of aminotransferases and disappearance of HCVRNA from the serum. This case suggests that recurrence of HCV infection in a sustained responder may be probable after immunosuppressive therapy. Prevention is currently impossible but retreatment may be successful.

Role of endogenous opioids in the control of gastric sensorimotor function.

Endogenous opioids have been implicated not only in the process of feeding but also in the control of gastric sensitivity and gastric motor responses, and impairment of antinociceptive opioid pathways has been hypothesized to contribute to the pathogenesis of functional dyspepsia. Our aim was to study the effect of suppression of endogenous opioid action by naloxone on gastric sensorimotor function in healthy volunteers. During intravenous administration of saline or naloxone (0.4 mg intravenous bolus followed by continuous infusion 20 microg kg(-1) h(-1)), sensitivity to gastric distension, gastric accommodation and fundic phasic contractility were evaluated by barostat in 15 subjects. Nutrient tolerance and meal-related symptoms were assessed using a satiety drinking test (n = 13), and solid and liquid gastric emptying were evaluated by breath test (n = 14). Naloxone did not influence gastric compliance and sensitivity. No effect on preprandial gastric tone was found but meal-induced accommodation was significantly inhibited by naloxone (P = 0.031). Subjects receiving naloxone demonstrated a higher motility index before (20.8 +/- 2.4 vs 28.0 +/- 1.9 mL s(-1), P = 0.007) and after (15.2 +/- 2.0 vs 22.7 +/- 1.5 mL s(-1), P = 0.0006) the meal. Naloxone significantly decreased the amount of food ingested at maximum satiety (715.4 +/- 77.7 vs 617.3 +/- 61.3 mL, P = 0.03). No effect of naloxone on gastric emptying was observed and intensity of postprandial symptoms was unchanged. These observations suggest that endogenous opioids are involved in the control of gastric accommodation and phasic contractility but not in the control of sensitivity to gastric distension or gastric emptying in healthy volunteers.

Fatal acute acalculous cholecystitis as an early complication after radiation therapy..

Acute acalculous cholecystitis (AAC) is a life-threatening condition whose incidence is steadily increasing, although it is still very much lower than that of the corresponding calculus form. The severity of the disease is due to the rapid course towards gallbladder necrosis and biliary peritonitis. Traditionally, it has been thought that AAC is associated with recent trauma, overeating or major surgical procedures. We describe a patient who presented acute cholecystitis, two days after completion of radiation therapy for metastatic lymphadenopathy along the hepatoduodenal ligament and distal common bile duct. He underwent exploratory laparotomy but he died from uncontrolled sepsis three days later. Histological study of the resected gallbladder showed findings of acute acalculous cholecystitis.

Subcutaneous metastasis from a gastrointestinal stromal tumor of the stomach: a case report.

Gastrointestinal stromal tumors (GISTs) are rare mesenchymal neoplasms of the stomach, which account for approximately 3.6% of all gastric tumors. They may or may not be malignant. Malignant GIST rarely metastasizes to distant organs. We report a case of a gastric GIST diagnosed in a 69- year-old woman presented with a synchronous subcutaneous paraumbilical metastasis. Computed tomography (CT) scan demonstrated a space-occupying lesion arising from the gastric wall with a second well-circumscribed lesion in the subcutaneous tissue which infiltrated the aponeurosis of the right rectus abdominis. The patient underwent total gastrectomy and resection of the subcutaneous mass. Pathologic examination of the gastric tumor and subcutaneous mass showed histological and immunohistochemical characteristics of a GIST. The patient succumbed on the 4th postoperative day. Gastric stromal tumor metastasis must be taken into consideration in the differential diagnosis of a palpable paraumbilical mass in a patient diagnosed with malignant GIST.

The rumination syndrome in adults: a review of the pathophysiology, diagnosis and treatment.

Rumination in adults is considered to be the effortless regurgitation of recently ingested food into the mouth, followed by either rechewing and reswallowing or expulsion of the regurgitate. On the basis of the definition of rumination as a unique category of functional gastroduodenal disorders, according to the newly established Rome III classification, a review of the pathophysiology, diagnosis and treatment of the rumination syndrome in adults is presented after systematic and critical approach of all articles that could be retrieved through PubMed using the term "rumination".

Analytical and clinical sensitivity of the Procleix Ultrio HIV-1/HCV/HBV assay in samples with a low viral load.

BACKGROUND AND OBJECTIVES: The Procleix Ultrio human immunodeficiency virus type 1 (HIV-1)/hepatitis C virus (HCV)/hepatitis B virus (HBV) (Ultrio) assay simultaneously detects HIV-1 RNA, HCV RNA and HBV DNA in individual blood donations. The main objective of the study was to assess the analytical and clinical sensitivity of the multiplex and discriminatory probe assays in samples with a low viral load. MATERIAL AND METHODS: The VQC HIV RNA genotype B, HCV RNA genotype 1 and HBV DNA genotype A standard dilutions were tested in 26 repeats. The probability of detection by Ultrio was compared with previously obtained data of the Procleix Duplex HIV-1/HCV assay on the same reference panels. A selection of 121 anti-HIV-1, 138 anti-HCV and 190 HBsAg positive samples from patients receiving antiviral therapy were tested. The majority of patient samples had a viral load below the detection limit of the diagnostic nucleic acid test assays, which made them suitable to evaluate the performance of the multiplex and discriminatory assays on yield cases with a similar low viral load. RESULTS: The 95% and 50% detection end-points of the Ultrio assay along with the corresponding 95% confidence intervals are 53.7 (32.9-117.2) and 8.6 (6.2-12.1) geq/ml for HIV-1 RNA, 30.3 (19.0-62.4) and 5.2 (3.7-7.2) geq/ml for HCV RNA and 393.7 (147.9-6978) and 54.5 (22.4-143.8) geq/ml for HBV DNA. The analytical sensitivity of Ultrio expressed as a potency factor relative to previously obtained Duplex results on the same HIV-1 RNA and HCV-RNA standard dilutions was 1.09 (0.20-6.10) and 1.11 (0.21-5.89), respectively. The assay detected all 22 HIV-1 infected patients with viral load > 50 copies/ml, and 41 of 99 patients (41%) with viral load < 50 copies/ml, of which 23 (56%) were detected by the discriminatory assay. All 47 patients with HCV RNA load > 521 IU/ml and 10/91 polymerase chain reaction-negative patients with viral load < 50 IU/ml tested positive in Ultrio assay of which five were missed in the discriminatory test. The assay detected 53/55 HBV infected patients (96%) with viral load > 250 copies/ml and 108/135 patients (80%) with viral load < 250 copies/ml of which 17 (16%) were missed by the discriminatory test. CONCLUSIONS: The new Procleix Ultrio assay is as sensitive as the Procleix Duplex assay for HIV-1 and HCV detection meeting the requirements of universal guidelines. The ability of the assay to detect HBV DNA in low viral load samples could be useful for screening blood. Inevitable negative results of discriminatory probe assays caused by stochastic sample variation will reduce the chance of recognizing low viraemic blood donors detected by individual donation nucleic acid test.

Improved survival of patients presenting with acute variceal bleeding. Prognostic indicators of short- and long-term mortality.

BACKGROUND AND AIM: Variceal bleeding is a severe complication of portal hypertension with a mortality rate between 30% and 60% in previous studies. During the last two decades the treatment of these patients has been improved. The aim of this study was to investigate the clinical outcome of patients after an episode of acute variceal bleeding and to identify risk factors for early and late mortality in these patients. MATERIALS AND METHODS: All patients with acute variceal bleeding hospitalised at two large hospitals between January 1, 1999 and June 30, 2004, were retrospectively enrolled in this study. After discharge, patients were followed until death or study closure date, on June 30, 2005. Bleeding related mortality, 6-week, 1-year and overall mortality were evaluated as well as factors related to early and late mortality. RESULTS: One hundred and forty one patients were included (114 men, 27 women) with a mean age of 60.5+/-13.5 years. In hospital, 6-week, 1-year and overall mortality were 12.1%, 18.4%, 32.6% and 48.2%, respectively. The mean length of hospitalisation was 11.4+/-9 days (1-55) and the mean packed red blood cell requirement was 3.9+/-3.7 (0-25). The rate of recurrent bleeding was 10.7% during initial hospitalisation. Being Child-Pugh C (p=0.003) and shock on admission (p=0.037) were independent predictors of 6-week mortality, while being Child-Pugh C (p=0.028), presence of hepatocellular carcinoma or other neoplasia (p=0.04) and partial thromboplastin time (p=0.021) during the initial admission were independent predictors for 1-year mortality. Mortality was not affected by the presence of active bleeding and/or white nipple at emergency endoscopy. Also presence of infection was not an adverse factor of clinical outcome in our patients. CONCLUSIONS: In conclusion, the clinical outcome of patients with acute variceal bleeding is better in comparison with previous studies. The severity of liver failure as well as the presence of neoplasia mainly affects the survival.

Safety and long-term follow-up of endoscopic snare excision of ampullary adenomas.

BACKGROUND: Adenomas of the duodenal papilla are rare. Because of their malignant potential, resection is mandatory. Options for resection include endoscopic resection techniques, transduodenal local excision, and pancreaticoduodenectomy. The aim of this retrospective study was to evaluate the safety and outcome of endoscopic snare resection of papillary adenomas in a Greek cohort of patients. METHODS: Fourteen patients (six women and eight men; age range, 42-76 years) were referred for endoscopic management of ampullary adenomas. A questionnaire was completed for each patient, which included preoperative and postoperative data points. Presenting symptoms were jaundice (n = 4), cholangitis (n = 1), and pain (n = 2). Seven patients were asymptomatic. If there was no common bile and main pancreatic duct invasion and the appearance suggested a benign lesion, biductal sphincterotomy onto normal duodenal tissue was performed. The adenomas were resected via a diathermy snare, along with the major papilla, after elevation of the lesion by epinephrine plus dextrose 50% (1:10,000) solution. At the discretion of the endoscopist, a biliary or pancreatic stent was inserted as a prophylactic procedure immediately after excision. RESULTS: Histopathologically, resected tissue included 11 adenomas and three adenomas with focal malignancy, referred for pancreaticoduodenectomy. Immediate complications were moderate bleeding (n = 1) and mild pancreatitis (n = 1). No procedure-related death occurred. Follow-up was available for 11 patients (mean, 28.36 months; range, 6-72). Pancreatic and biliary stents were placed in four and nine patients, respectively. Follow-up endoscopy revealed recurrent/residual adenomatous tissue in two patients (18%), which was resected endoscopically. CONCLUSION: Endoscopic snare resection of adenomas of the major duodenal papilla is a safe, well-tolerated alternative to surgical therapy. In expert hands, complications are mild and may be avoided by pre-resection biductal sphincterotomy, stent placement, and elevation of the lesion by epinephrine plus dextrose 50% solution injection.

Lipohyperplasia of the ileocecal valve as a cause of intussusception.

We present a case of lipohyperplasia of the ileocecal valve causing episodes of intussusception, and visualized by abdominal ultrasound and CT as a "target-like" appearance. At surgery, a large yellow soft mass was in the region of the ileocecal valve, raising the suspicion of lipohyperplasia. Resection of the fatty tissue from the ileocecal valve was performed at operation, after histologic diagnosis on frozen section. Our case suggests that limited resection removing the terminal ileum is effective and preferable to more extensive resection.

Predisposing factors and clinical symptoms in HIV-negative patients with Candida oesophagitis: are they always present?

Candida oesophagitis (CO) is scarce among immunocompetent patients. This study aimed at evaluating predisposing factors, clinical symptoms and endoscopic findings in this group. We retrospectively reviewed 55 patients diagnosed as CO endoscopically (whitish plaques) and cytologically (fungal mycelia on brush cytology). Carcinoma, diabetes, acid suppression, steroids, gastric surgery and oesophageal motility disorders were considered as predisposing factors. Twenty of 55 patients lacked any predisposing factor for CO. These patients were more frequently asymptomatic (8/20) when compared with those with known predisposing factors (5/35) (p = 0.031). Moreover, dysphagia was more prevalent in the latter group (24/35 vs. 8/20; p = 0.039). Endoscopic findings correlated with the presence of neither predisposing factors nor symptoms (Wilcoxon p > 0.05). Thus, CO can be discovered in patients without apparent predisposing risk factors and clinical symptoms. Further studies are needed to elucidate the mechanisms of transition from colonisation to infection.

Primary biliary cirrhosis complicated by transverse myelitis in a patient without Sjögren's syndrome.

Transverse myelitis is an acute inflammatory process, affecting one or more segments of the spinal cord. Its association with primary biliary cirrhosis has been documented in only four cases--all along with Sjögren's syndrome. Herein, we report for the first time, a patient who developed recurrent acute transverse myelitis in association with primary biliary cirrhosis without any clinical or histological indication of Sjögren's syndrome. A 42-year-old woman with primary biliary cirrhosis developed acute onset quadriparesis and urinary retention. Diagnostic evaluation excluded the presence of Sjögren's syndrome, other autoimmune syndromes, infections and multiple sclerosis. Magnetic resonance imaging of the spinal cord disclosed signal intensity abnormalities from C1 to T2 after gadolinium enhancement. As diagnosis of acute transverse myelitis was prominent, the patient was treated with intravenous methylprednisolone. The patient had a fair outcome despite an early recurrence of the symptoms after treatment withdrawal.