Autoimmune Hepatitis: Single-center Experience of Clinical Presentation, Response to Treatment and Prognosis in Saudi Arabia

Background /Aim:

Autoimmune hepatitis (AIH) is a common cause of end-stage liver disease worldwide. It is a disease prevalent in children and adults, with female predominance and variable clinical presentations. AIH has favorable responses to steroids and immunomodulators. Diagnosis of AIH is based on clinical and laboratory criteria, as suggested by the International Autoimmune Hepatitis Group. Data on the disease pattern of AIH from the Middle East countries is scarce.

Materials and Methods:

In this retrospective analysis, we studied clinical and laboratory features, immunological data, radiological findings, liver biopsy findings and response to therapy in patients with AIH from the hepatology clinics of King Abdul Aziz, University Hospital, Jeddah, from 1994 to 2008.

Results:

We diagnosed 41 patients with AIH, and 33 were included in the analysis. The mean age was 32.3 years, with female predominance of 75.7%. De-compensated cirrhosis at presentation was found in 45.5% of the patients. Acute hepatitis was associated with significantly higher levels of the serum ALT and bilirubin (P=0.001 and P=0.03, respectively). All our patients had type 1 AIH. Treatment with prednisolone and azathioprine resulted in complete or partial remission in majority of the patients (54.8%). However, patients with advanced disease showed a poorer response to treatment (P=0.016). Six patients with poor compliance had relapse of AIH. Two patients had a flare of the disease during pregnancy, and they responded well with prednisolone. The longest follow-up was 14 years and the shortest was 2 months. Four patients died from liver disease.

Conclusion:

AIH patients in Saudi Arabia are likely to present with advanced disease at a young age and would have a poorer response to therapy as compared with patients in other countries worldwide.     

Complex regional pain syndrome: a review

Complex regional pain syndrome (CRPS), formerly known as "reflex sympathetic dystrophy," is a chronic neurological disorder characterized by disabling pain, swelling, vasomotor instability, sudomotor abnormality, and impairment of motor function. The disorder usually develops after minor trauma or surgery. No specific diagnostic test is available and, hence, diagnosis is based mainly on history, clinical examination, and supportive laboratory findings. This review gives a synopsis of CRPS and discusses the principles of management based on the limited available literature in the area. A literature search was conducted using electronic bibliographic databases (Medline, Embase, Pubmed, CENTRAL) from 1970 to 2006. Keywords complex regional pain syndrome, reflex sympathetic dystrophy, neuropathic pain, and causalgia were used for the search. Relevant articles from the reference lists in retrieved articles were also studied. There were 3,771 articles published in the area. Seventy-six randomized controlled trials were identified. Most studies were on the role of sympathetic blockade in the treatment of CRPS (n = 13). The role of sympathectomy is unclear, with some studies showing transient benefit and others showing no beneficial effects, with most studies containing only a small number of patients. Nine studies were on bisphosphonates or calcitonin. Studies involving bisphosphonates showed benefit, but studies involving calcitonin showed no definite benefit. Four studies were on cognitive behavioral therapy, physiotherapy, or occupational therapy, all of which demonstrated a potential beneficial effect. Three studies on spinal cord stimulation and two studies each on acupuncture, vitamin C, and steroid all showed a potential beneficial effect in pain reduction. The remaining studies were on miscellanous therapy or combination therapy, making it difficult to draw any conclusions on the effect of treatment. There is very little good evidence in the literature to guide treatment of CRPS. Early recognition and a multidisciplinary approach to management seems important in obtaining a good outcome. Treatments aimed at pain reduction and rehabilitation of limb function form the mainstay of therapy. Comorbidities, such as depression and anxiety, should be treated concurrently.     

Extended use of serum free light chain as a biomarker in lymphoproliferative disorders: a comprehensive review

Serum free light chain (sFLC) assays were shown to improve detection, management, and prognostication in plasma cell disorders. Recently, sFLC assays improved detection of M proteins when combined with standard methods of protein electrophoresis/immunofixation in patients with non-Hodgkin lymphoma/chronic lymphocytic leukemia (NHL/CLL). Incidence of abnormal sFLC ratio (sFLCr) varied from 0% to 36% and 29.7% to 59% in NHL and CLL, respectively. Increased sFLC levels or abnormal sFLCr predict shorter overall survival in early-stage CLL. Furthermore, abnormal sFLCr correlated with advanced disease stage and poorer outcome. In diffuse large B-cell lymphomas, increased sFLC was demonstrated as an independent, adverse prognostic factor for overall/event-free survival. Moreover, abnormal sFLCr can be a diagnostic tool in central nervous system lymphomas. Finally, the quantitative FLC assay has the potential to become a new, easily measured biomarker for predicting prognosis and enhanced detection in NHL/CLL. It may be used serially at follow-up evaluations to provide clues to relapse.     

Network meta-analysis of nine large cardiovascular outcome trials of new antidiabetic drugs

The aim of this network meta-analysis (NMA) was to indirectly compare the cardiovascular (CV) safety of new antidiabetic medications in patients with type 2 diabetes mellitus (T2DM).Data synthesisA search of the Embase and MEDLINE databases was conducted systematically to identify cardiovascular outcome trials (CVOTs) of new antidiabetic medications (DPP-4 inhibitors, GLP-1 agonists and SGLT-2 inhibitors) in patients with T2DM. The primary outcomes were the composite endpoint of CV death, nonfatal MI, and nonfatal stroke (MACE), death from CV causes, nonfatal MI, nonfatal stroke and death from any cause. Hospitalization for HF and unstable angina were evaluated as secondary endpoints. A total of 9 trials, including 87,162 patients, met the eligibility criteria and were retained for the analysis.The NMA results showed no significant differences among the DPP-4 inhibitors (sitagliptin, alogliptin, and saxagliptin) in any of the CV endpoints. Similarly, no significant changes were seen in the NMA among the GLP-1 receptor agonists nor the SGLT-2 inhibitors. The pairwise meta-analysis showed that DPP-4 inhibitors have a CV safety profiled comparable to placebo. GLP-1 agonists on the other hand, showed significant reduction in MACE (RR 0.92; 95% CI 0.87–0.97), death from CV causes (RR = 0.88; 95% CI 0.80–0.97), and death from any cause (RR = 0.89; 95% CI 0.82–0.96). SGLT-2 inhibitors showed significant reduction in hospitalization for heart failure events (RR 0.72; 95% CI 0.6–0.86) compared to placebo.ConclusionThis meta-analysis has shown that new antidiabetic medications do not impose any additional CV risk. The indirect comparison among the medications of each class resulted in no significant changes regarding CV endpoints and death from any cause.     

Endovascular repair of severe aortic coarctation,transcatheter aortic valve replacement for severe aortic stenosis,and percutaneous coronary intervention in an elderly patient with long term follow-up

To the best of our knowledge, there have not been any reports of total transcatheter approach including stenting of severe coarctation of the aorta (CoA), transcatheter aortic valve replacement (TAVR) for concomitant severe aortic valve stenosis, and percutaneous coronary intervention (PCI) to treat significant coronary artery disease in a single patient. We report a 70-year-old female, who presented with uncontrolled hypertension and acute decompensated heart failure (ADHF) and was found to have severe CoA, severe bicuspid aortic valve (BAV) stenosis, and significant proximal left anterior descending (LAD) coronary artery disease. In a multidisciplinary heart team meeting, we decided to perform an endovascular repair of both cardiac and vascular pathologies using a two-stage approach due to the significant comorbidities; mainly uncontrolled hypertension, type 2 diabetes mellitus, chronic obstructive pulmonary disease, and severe calcifications of the ascending aorta. The procedures were successfully performed and the patient was asymptomatic 30?months later at follow-up and was without any significant gradients across the coarctation or the aortic valve.