Behaviour of Crohn's disease according to the Vienna classification: changing pattern over the course of the disease.

BACKGROUND: Crohn's disease is a heterogeneous disorder with both a genetic and environmental aetiology. Clinical classifications of the disease, such as the newly proposed Vienna classification, may help to define subgroups of patients suitable for studying the influence of specific genetic or environmental factors. AIM: To assess the stability over the course of the disease of its location and behaviour, as determined according to the Vienna classification. PATIENTS AND METHODS: The notes of 297 Crohn's disease patients regularly followed up at our institution were carefully reviewed retrospectively. The behaviour and location of the disease according to the Vienna classification were determined at diagnosis and after 1, 3, 5, 10, 15, 20, and 25 years of follow up. The proportions of the different behaviours and locations of the disease were calculated at these time points. A statistical analysis of the evolution of these characteristics over 10 years was performed on a subgroup of 125 patients with at least 10 years of follow up. The influence of age at diagnosis on location and behaviour of the disease was assessed as well as the influence of location on the behaviour of the disease. RESULTS: The location of the disease remained relatively stable over the course of the disease. Although the proportion of patients who had a change in disease location became statistically significant after five years (p=0.01), over 10 years only 15.9% of patients had a change in location (p<0.001). We observed a more rapid and prominent change in disease behaviour, which was already statistically significant after one year (p=0.04). Over 10 years, 45.9% of patients had a change in disease behaviour (p<0.0001). The most prominent change was from non-stricturing non-penetrating disease to either stricturing (27.1%; p<0.0001) or penetrating (29.4%; p<0.0001) disease. Age at diagnosis had no influence on either location or behaviour of disease. Ileal Crohn's disease was more often stricturing, and colonic or ileocolonic Crohn's disease was more often penetrating: this was already the case at diagnosis and became more prominent after 10 years (p<0.05). CONCLUSIONS: Location of Crohn's disease, as defined by the Vienna classification, is a relatively stable phenotype which seems suitable for phenotype-genotype analyses. Behaviour of Crohn's disease according to the Vienna classification varies dramatically over the course of the disease and cannot be used in phenotype-genotype analyses. The potential influence of genes on the behaviour of Crohn's disease should be studied in subgroups of patients defined by their disease behaviour after a fixed duration of disease.     

Multilevel disease of the conduction system

We found that the incidence and distribution of conduction system disease simultaneously affecting several levels of the system had been incompletely described. We therefore analysed all patients in whom conduction disease had been diagnosed and a complete electrophysiological study of the conduction system had been made, during a defined period. Patients were classified as to the presence or absence of sinus node disease, and proximal and distal atrioventricular disease. Since our hospital serves a defined population and a large proportion of all patients with conduction disease seen during the period was included, the series probably is representative of conduction disease in general. Twenty-four of the 59 patients had defects at more than one of the three levels (41%). Fifty-five per cent of the patients with sinus node disease had some kind of atrioventricular disease, while 42% of the latter had evidence of sinus node failure. The patients with multilevel disease were characterized by more severe symptoms, higher age and a higher incidence of generalized heart disease than those with single-level disease. The observations fit the concept that conduction system disease in most patients is part of a diffuse disease process progressively involving various parts of the system.     

Global revascularization: the role of the cardiologist

There are compelling reasons for cardiologists to undertake a more global approach to patients with peripheral vascular diseases: atherosclerosis is a 'systemic' disease frequently causing both coronary and peripheral vascular problems in the same patient; coronary artery disease is the most common cause of morbidity and mortality in patients with peripheral vascular disease; and peripheral vascular disease negatively impacts the management of angina pectoris and congestive heart failure. There are four major areas of special interest to the cardiologist: (1) iliac arteries (vascular access), (2) renal arteries (hypertension and volume overload), (3) subclavian arteries (coronary steal with a left internal mammary artery [LIMA] graft), and (4) carotid arteries (stroke). coronary angioplasty are transferable to the peripheral vasculature. However, an understanding of the natural history of peripheral disease, patient and lesion selection criteria, and knowledge of other treatment alternatives are essential to performing these procedures safely and effectively. Appropriate preparation and training, and a team approach, including an experienced vascular surgeon, are both desirable and necessary before interventional cardiologists who are inexperienced in the treatment of peripheral vascular disease attempt percutaneous peripheral angioplasty. There are inherent advantages for patients when the cardiologist performing the procedure is also a clinician. Judgments regarding the indications, timing, and risk/benefit ratio of procedures are enhanced by a long-term relationship between physician and patient. Finally, in view of the increased incidence of coronary artery disease in patients with atherosclerotic peripheral Technical skills necessary to perform vascular disease, the participation of a cardiologist in their care seems appropriate. (Int J Cardiovasc Intervent 2000; 3: 71 79)     

Positive non-invasive tests in the chest pain unit: importance of the clinical profile for estimating the probability of coronary artery disease

We studied 100 consecutive patients with coronary angiography performed after a positive result of a non-invasive test in the chest pain unit. Mean age was 66 years. The percentage of patients without significant coronary artery disease, 1-vessel disease and multivessel disease was 35, 33, and 32%, respectively. The CPU-65 index that combines four clinical variables (comorbidity: diabetes, pain: typical, use of: aspirin, 65 years old or more) was associated with the presence of coronary artery disease, multivessel disease, and coronary revascularization. We conclude that in the chest pain unit, patients with a positive non-invasive test the rate of false positive results, one-vessel disease and multivessel disease is similar. However, important changes in these percentages are seen, according to the clinical profile. The CPU-65 index is useful to determine the pre-test probability of significant coronary artery disease and patients with intermediate scores are the ones that benefit most of non-invasive tests.     

Precipitation and the occurrence of lyme disease in the northeastern United States

The occurrence of Lyme disease is a growing concern in the United States, and various studies have been performed to understand the factors related to Lyme disease occurrence. In the United States, Lyme disease has occurred most frequently in the northeastern United States. Positive correlations between the number of cases of Lyme disease reported in the northeastern United States during the 1992-2002 period indicate that late spring/early summer precipitation was a significant climate factor affecting the occurrence of Lyme disease. When late spring/early summer precipitation was greater than average, the occurrence of Lyme disease was above average, possibly due to increased tick activity and survival rate during wet conditions. Temperature did not seem to explain the variability in Lyme disease reports for the northeastern United States.     

糖尿病与牙周病关系的临床研究

目 的 探讨糖尿病与牙周病的相关性。方法 调查对比分析DM组与非糖尿病（NDM）组相关指标。再将DM组中的牙周病患者随机分为4组,并在控制血糖的基础上给予不同治疗。A组仅保持口腔清洁,不针对牙周病治疗;B组接受口腔常规治疗;C组接受口腔常规治疗联合甲硝唑控释药膜治疗;D组在C组基础上加用盐酸米诺环素。分析各组治疗效果。 结果 DM组牙周病发病率高于NDM组（P〈0.05）。牙周病病症指标与FPG呈正相关（r≥0.136, P〈0.05）。D组的治疗效果最佳,好转率和有效率分别为61.90%和73.81%,均高于其余3组（P〈0.05）。结论 糖尿病患者牙周病发病率高,控制血糖治疗并给予积极治疗可有助于降低牙周病发病率。     

Review: Current recommendations on the role of diet in the aetiology and management of IBD

Diet is a key modifier of risk of inflammatory bowel disease development and potentially a treatment option in patients with established disease. International organisations in gastroenterology and inflammatory bowel disease have published guidelines for the role of diet in disease onset and its management. Here, we discuss the major overarching themes arising from these guidelines and appraise recent literature on the role of diet for inflammatory bowel disease prevention, treatment of active disease and maintenance of remission, considering these themes. Except for exclusive enteral nutrition in active Crohn’s disease, we currently possess very little evidence to make any further dietary recommendations for the management of inflammatory bowel disease. There is also currently uncertainty on the extrapolation of epidemiological dietary signals on risk of disease development and preclinical experiments in animal models to management, once disease is established. Until high-quality evidence from clinical research becomes available, the only specific recommendations for inflammatory bowel disease we might safely give are those of healthy eating which apply for the general population for overall health and well-being.     

莱姆病治疗进展

莱姆病是一种由伯氏疏螺旋体所引起的,以硬蜱为主要传播媒介的自然疫源性疾病。对莱姆病的治疗主要是早期使用抗生素。莱姆病是一种全身性慢性传染病,临床表现复杂多样,侵犯多系统多器官引起损伤,根据其病程发展可分为早中晚三期,而针对不同分期、不同表现及不同发病人群的莱姆病其治疗方法又各不相同,本文将对此进行详细综述。     

Antihypertensive drugs and the kidney

In the United States, 50 million Americans are estimated to have hypertension. Over the past several decades, it has become clear that hypertension is both a cause and a consequence of kidney disease. In contrast to the striking decline in mortality rates from both stroke and coronary heart disease, the prevalence of hypertension as a cause of end-stage renal disease (ESRD) has increased such that it is now the second most common cause of ESRD in the United States. Hypertension and proteinuria occur in most patients with chronic kidney disease and are risk factors for faster progression of kidney disease. Antihypertensive agents reduce blood pressure and urine protein excretion and slow the progression of kidney disease. The level of blood pressure achieved and use of renin-angiotensinaldosterone system-blocking agents is critical for delaying progression of renal disease in all ethnic groups.     

Chagas' cardioneuropathy: Cardiovascular autonomic dysfunction as the first manifestation of the disease

Chagas' disease is a leading cause of cardiac arrhythmias, congestive heart failure, autonomic nervous system dysfunction, and sudden death in endemic areas of the American continent. It is also a major cause of chronic myocarditis, and the apical aneurysm is a distinctive pathological marker of the disease. Wall motion abnormalities, either segmental or diffuse, are another important clinical feature of the disease. Information regarding geographical distribution, frequency, and appearance of the autonomic dysfunction at the different stages of the disease is still lacking. A group of 181 patients with Chagas' disease were studied in Venezuela. Clinical and physiological tests showed abnormalities in cardiovascular reflexes, making it possible to confirm the presence of autonomic nervous system dysfunction in Venezuela, as well as in other patients with Chagas' disease previously described in other countries. Chagas' disease patients showed a high incidence of autonomic nervous system dysfunction (54%) which occurred in the three stages of the evolution of this disease, being frequently found during the initial stage in young, asymptomatic, cardiovascular patients. Autonomic dysfunction may be the first and only sign of the disease in this subgroup, and it cannot be related to age or heart failure.Presented at The 38th Annual World Congress, International College of Angiology, Köln, Germany, June 1996     

alpha-1-antitrypsin deficiency in liver disease: the extent of the problem.

A prospective screening program was undertaken at the Royal Free Hospital, London, to ascertain the incidence of alpha-1-antitrypsin (AAT) deficiency in patients with liver disease. Quantitative determinations of serum alpha-1-antitrypsin were performed on 469 patients with hepatobiliary disease and 98 subjects with no known liver disease. Sera with low values of AAT were phenotyped. The homozygous state was rare and comprised only 1% of the patients with liver disease. All of the 5 homozygous deficient (ZZ phenotype) patients had a history of neonatal liver disease. Other phenotypes (partial deficiency) were found in 4.7% of patients with liver disease and 6.1% of subjects with normal liver function. Types of liver disease in the patients with other phenotypes were widely varied. Routine determination of serum AAT level and phenotype and special staining for AAT in liver biopsies in all adults with liver disease appears unnecessary. Investigation of possible AAT deficiency should be carried out, however, in children and young adults, in those with a history of neonatal liver disease, and possibly in all patients with liver disease of unknown aetiology.     

Involvement of the immune system in the pathogenesis of Crohn's disease. Expression of the T9 antigen on peripheral immunocytes correlates with the severity of the disease

Peripheral lymphocyte cells from patients suffering from Crohn's disease were analyzed for the expression of the "activation" antigens T9 and HLA-DR on their cell surface. It was found that high numbers of "activated" lymphocytes, the majority of which have proven to be T cells, could be detected in patients with active Crohn's disease, whereas in healthy controls and inactive disease only a small subfraction of lymphocytes was positive for these antigens. This difference was highly significant (p = 0.0001). Within the subpopulation of T9-positive cells the ratio between T4- and T8-positive cells is about 1.8 (compared with 2.0 in the total T-cell subset). All HLA-DR-positive, non-B and non-glass-adherent cells could be detected in the T9-positive cell fraction. The presence of T9 antigens was found to correlate with the grade of severity of the disease as assessed by a Crohn's disease activity index. The presence of high amounts of T cells exhibiting this antigen is not restricted to Crohn's disease but is thought to be of importance as a marker for the involvement of the immune system in other maladies as well. Nevertheless, the determination of T9 antigen is expected to provide objective data reflecting the severity of Crohn's disease.     

Cavernous transformation of the portal vein: etiology determines the outcome

BACKGROUND/AIMS: Cavernous transformation of the portal vein is a rare condition with various etiologies and diverse clinical presentations. We aim to assess the disease outcome of different etiologic groups. METHODOLOGY: We retrospectively scrutinized the clinicopathologic characteristics of 35 patients with cavernous transformation of the portal vein, placing special emphasis on the disease extent, progression, and outcome after stratification into different etiologic groups. RESULTS: There were 20 males and 15 females with a mean age of 40.4 years. The etiologies of these patients could be classified into four groups including hematologic disease (n=12, 34.2%), malignant disease (n=8, 22.9%), non-malignant disease (n=6, 17.2%), and idiopathic (n=9, 25.7%). The hematologic disease group had 9 patients with myeloproliferative disease and 3 patients with protein C or protein S deficiency. The hematologic disease group survived well after medication or surgical management. Hepatocellular carcinoma (n=6) was the leading cause in the malignant diseases group, which had a significantly poorer survival rate than the rest (p<0.05). CONCLUSIONS: Cavernous transformation of the portal vein is a long-term sequele of portal vein thrombosis in which the etiology determines the outcome. More aggressive management including long-term anticoagulation in patients with myeloproliferative disorder or underlying prothrombotic states and endoscopic therapy in controlling variceal bleeding may be a more effective treatment for these patients.     

Diabetes mellitus, the renin-angiotensin-aldosterone system, and the heart

With diabetes mellitus reaching epidemic proportions, mainly secondary to obesity, the impact of cardiovascular disease due to this combination makes it a dominant public health problem during the first quarter of the 21st century. The complex interaction that results in diabetic heart disease is created by overlapping mechanisms. There is a propensity to develop premature, diffuse atherosclerotic coronary disease, which is associated with adverse short- and long-term morbidity and mortality. There are structural and functional abnormalities of the microvasculature, autonomic dysfunction, and intrinsic failure of myocardial contraction (so-called diabetic cardiomyopathy). These changes are amplified by arterial hypertension and kidney disease. In this review, we consider the role of the renin-angiotensin-aldosterone system and how it is a crucial driver of most of the pathophysiologic mechanisms behind diabetic heart disease and why in the past 5 years blocking this system in diabetic patients has emerged as a critical therapeutic intervention.     

Comment on the use of self-reporting instruments to assess patients with rheumatoid arthritis: the longitudinal association between the DAS28 and the VAS general health

OBJECTIVE: Recently, the use of patient self-reporting instruments instead of clinical, objective measurements to assess rheumatoid arthritis (RA) patients was proposed. This assumes a constant association between disease activity and the self-reporting instruments. The objective was to explore the association (in time) between disease activity and patient perception of general health, disease activity, pain, and functional disability in patients with RA. METHODS: Data of 624 newly diagnosed RA patients who completed 3 years of followup were analyzed. Cross-sectional linear regression models and longitudinal regression models were estimated, with a visual analog scale (VAS) measuring general health (VAS-GH; 0 = best, 100 = worst) as a dependent variable and the Disease Activity Score (DAS28) without the VAS-GH as an independent variable. Other dependent variables were VAS disease activity, pain, and the Health Assessment Questionnaire. RESULTS: The DAS28 and VAS-GH were significantly associated in RA patients (P < 0.001). However, the explained variance was low (6.7%). From diagnosis to 3 years after the diagnosis, the intercept decreased given the same regression coefficient. The longitudinal regression model showed that the VAS-GH improved during disease course independent of a change in DAS28. Analyses on the other outcome parameters showed similar results. CONCLUSION: Patients' perception of health can be different with equal disease activity, depending on the moment in the disease course. Furthermore, our results indicate that self-reported measures on functionality, disease activity, and general health cannot substitute for objective measures of disease activity in RA in longitudinal studies; subsequently, both need to be measured.     

Diabetic Cardiovascular Disease: Getting to the Heart of the Matter

Diabetes is a major risk factor for heart disease, and heart disease is responsible for substantial morbidity and mortality among people living with diabetes. The diabetic metabolic milieu predisposes to aggressive obstructive coronary artery disease that causes heart attacks, heart failure, and death. Furthermore, diabetes can be associated with heart failure, independent of underlying coronary artery disease, hypertension, or valve abnormalities. The pathogenesis of the vascular and myocardial complications of diabetes is, as yet, incompletely understood. Although a number of medical and surgical approaches can improve outcomes in diabetic patients with cardiovascular disease, much remains to be learned in order to optimize approaches to these critical complications.     

Vasculitis in the geriatric population

Vasculitis represents an uncommon but important group of disease entities that may affect older patients. The most common systemic vasculitic disease in humans is giant cell arteritis, a disease process seen almost exclusively in patients over the age of 50. Vasculitic disease in geriatric patients presents unique challenges with regard to diagnosis and treatment. A thorough understanding of the vasculitic disease entities that may affect older patients as well as their diagnosis and management is essential in minimizing disease and treatment related morbidity and mortality.     

Vasculitis in the geriatric population

Vasculitis represents an uncommon but important group of disease entities that may affect older patients. The most common vasculitic disease in humans in giant cell arteritis, a disease process seen almost exclusively in patients older than 50 years in age. Vasculitic disease in geriatric patients presents unique challenges with regard to diagnosis and treatment. A thorough understanding of the vasculitic disease entities that may affect older patients as well as their diagnosis and management is essential in minimizing disease and treatment-related morbidity and mortality.     

Evolutionary changes in the pathologic diagnosis after the ileoanal pouch procedure

PURPOSE: Inadequate initial differentiation between ulcerative colitis and Crohn's disease may lead to a diagnosis of indeterminate colitis. Construction of an ileoanal pouch in these patients may result in significant morbidity and pouch failure when the ultimate diagnosis is Crohn's disease. METHOD: We prospectively studied 543 patients with idiopathic inflammatory bowel disease to determine whether a patient's pathologic diagnosis changed with time and how it affected outcome. RESULTS: Preoperative diagnosis was ulcerative colitis in 499 patients, indeterminate colitis in 42 patients, and Crohn's disease in 2 patients. Prior colectomy was performed in 58 percent of patients with ulcerative colitis and in all patients with indeterminate colitis and Crohn's disease. Postoperatively, the diagnosis changed in 20 patients with ulcerative colitis (13 to indeterminate colitis, 7 to Crohn's disease). Another two patients with indeterminate colitis showed evidence of Crohn's disease in the resected rectal specimen. As patients were followed up, an additional 13 patients were found to have Crohn's disease (5 indeterminate colitis, 8 ulcerative colitis). With the current diagnosis, perineal complications and pouch failure occurred, respectively, in 23 and in 2 percent of patients with ulcerative colitis, in 44 and in 12 percent of patients with indeterminate colitis, and in 63 and in 37 percent of patients with Crohn's disease. Pathologic diagnosis was altered in 35 patients (6 percent) overall, with a 12-fold increase in the diagnosis of Crohn's disease. Only 3 percent of patients with ulcerative colitis compared with 13 percent of patients with indeterminate colitis had a change in diagnosis to Crohn's disease (P =0.006; Fisher's exact test). CONCLUSION: Pouch-related complications, eventual pouch failure, and discovery of underlying Crohn's disease occurred in a significant number of patients with a diagnosis of indeterminate colitis. Until more accurate diagnostic differentiation is available, caution is advised in recommending the ileoanal pouch procedure to patients with indeterminate colitis.Read at the meeting of The American Society of Colon and Rectal Surgeons, Seattle, Washington, June 9 to 14, 1996.     

New knowledge of the pathogenesis of Crohn's disease

Crohns disease is a complex chronic inflammatory disease of the gastrointestinal tract with multifactorial pathogenesis. Over the recent years, there has been rather a sharp increase in the incidence of Crohn's disease and, even though this disease had been known for some time, the cause remains unknown. Studies exploring genetic basis of Crohn's disease have provided new knowledge of the pathogenesis of this disease, suggesting that this may be associated with a failure of mechanisms behind symbiosis of gut microflora and intestinal mucosal immune system. Crohn's disease seems to be caused by inadequate immune response to intestinal flora in genetically predisposed individuals. Crohn's disease has been linked to a number of genes. Many of them are related to the modulation of non-specific immune response, defects of which are considered to be key in Crohn's disease pathogenesis. The aim of this review paper is to summarize the new knowledge on the pathogenesis of Crohn's disease at the level of polymorphisms of the NOD2, ATG16L1 genes and the IL23-Th17-lymfocytes signalling pathway genes and to consider further research directions in this disease.