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1.
BackgroundSystemic amyloidosis is a rare but life-threatening complication of inflammatory bowel disease (IBD), most cases being reported among Crohn's disease (CD) patients. The only two available retrospective studies showed a prevalence ranging from 0.9% to 3% among CD patients.AimsTo evaluate the prevalence of secondary systemic amyloidosis in a large IBD cohort of a referral centre, and to describe its clinical characteristics and outcome.MethodsPatients diagnosed with amyloidosis were identified among 1006 IBD patients included in the IBD database of our centre, and their medical records were carefully reviewed.ResultsAmong a total of 1006 IBD patients, 5 cases of amyloidosis were identified, all of them with CD, resulting in a prevalence of 0.5% for IBD and 1% for CD. Two patients died after developing renal failure. Two patients were treated with anti-TNF agents, showing a clinical improvement of their amyloidosis.ConclusionsSecondary amyloidosis occurs mainly in long-lasting, complicated, Crohn's disease and seems to be as prevalent among IBD patients as previously reported.  相似文献   

2.
继发性淀粉样变的临床特点   总被引:4,自引:0,他引:4  
目的:研究继发性淀粉样变的临床特点、治疗与预后。方法:对诊为继发性淀粉样变的全部住院病例进行回顾性临床分析。结果:在8例继发性淀粉样变病人中,其原发病分别为结缔组织病4例,多发性骨髓瘤3例,慢性乙型肝炎1例。由结缔组织病至继发淀粉样变平均6.2年,肾脏为其主要累及器官,表现为蛋白尿(4例),1例发生肾功能衰竭,以免疫抑制剂及/或激素治疗后,患者平均生存期长于10年。多发性骨髓瘤继发淀粉样变的时间较短,以舌组织及心肌受累为主,化疗效果差,病人的生存期平均不超过2年。而继发于慢性肝炎后的淀粉样变主要表现为胸腔积液与肝脾大,病情相对平缓。结论:淀粉样变可以继发于结缔组织病、多发性骨髓瘤或慢性炎性疾病,他们的起病时间、受累脏器以及临床表现差异很大,继发于多发性骨髓瘤的淀粉样变预后差,但结缔组织病继发的淀粉样变经积极有效的治疗可以明显改善预后。  相似文献   

3.
Airway diseases are the most commonly described lung manifestations of inflammatory bowel disease(IBD). However,the similarities in disease pathogenesis and the sharing of important environmental risk factors and genetic susceptibility suggest that there is a complex interplay between IBD and airway diseases. Recent evidence of IBD occurrence among patients with airway diseases and the higher than estimated prevalence of subclinical airway injuries among IBD patients support the hypothesis of a two-way association. Future research efforts should be directed toward further exploration of this association,as airway diseases are highly prevalent conditions with a substantial public health impact.  相似文献   

4.
Objective: To identify risk factors for serum amyloid-A (AA) amyloidosis in patients living in Germany.

Methods: Clinical and genetic data were obtained from 71 patients with AA amyloidosis. SAA1 genotypes were analyzed in 231 individuals. Control groups comprised 45 patients with long-standing inflammatory diseases without AA amyloidosis and 56 age-matched patients without any inflammatory disease.

Results: The most frequent underlying diseases of AA amyloidosis were familial Mediterranean fever (FMF) (n?=?24, 34%) and inflammatory rheumatic diseases (n?=?30, 42%). Patients without any known underlying disease (n?=?11, 16%) were considered as having idiopathic AA amyloidosis. Patients with FMF were significantly younger at disease onset and younger at diagnosis of AA amyloidosis compared with patients with rheumatic diseases. Patients with idiopathic AA amyloidosis were older than patients with definite rheumatic diseases. Patients with FMF and high penetrance MEFV gene mutations had a relative risk of 1.73 for AA amyloidosis. Patients with FMF or a rheumatic disease and the SAA1 α/α genotype had a relative risk of 4.86 and 2.53, respectively, for developing an AA amyloidosis. The prevalence of this risk genotype was 36% in German patients without an inflammatory disease, 92% in German patients with AA amyloidosis and 100% in German patients with idiopathic AA amyloidosis.

Conclusions: Risk factors for AA amyloidosis are the presence of a hereditary autoinflammatory or chronic rheumatic disease, elevated C-reactive protein and SAA serum levels, a long delay of a sufficient therapy, an advanced age and the SAA1α/α genotype.  相似文献   

5.
The prevalence of inflammatory bowel disease (IBD) continues to rise around the globe. Although the percentage of pediatric IBD patients seems to be increasing, rates are surprisingly heterogeneous among different populations. Although the pathogenesis of IBD is believed to be multifactorial, a genetic predisposition may be especially relevant in pediatric-onset IBD. Phenotypic characteristics can also be significantly different when comparing pediatric and adult-onset IBD. Patients that develop the disease at a younger age usually present with more extensive and more aggressive disease and develop complications faster when compared to those that develop it during adulthood. Children with IBD are found to have frequent mood disorders and have a higher risk of developing socio-economic hardship, failing to meet development milestones. Therefore, IBD management should always involve a multidisciplinary team that is not limited to medical providers. Most institutions do not have an established transition protocol and lack the resources and training for transition care. Although there is no consensus on an optimal timing to transition the patient's care to an adult team, it is usually accepted they should be eligible for adult care when most of the key transition points have been met. Management strategies should be tailored to each patient's developmental level and environment. A successful transition can improve the long-term outcomes such as sustained remission, medication adherence, mental health and social and academic performance, while decreasing healthcare utilization. Every institution that manages pediatric IBD patients should have a well-established transition protocol in order to make sure to maintain continuity of care.  相似文献   

6.
Patients with inflammatory bowel disease(IBD)may have an increased risk of venous thrombosis(VTE).PubMed,ISI Web of Knowledge and Scopus were searched to identify studies investigating the risk of VTE and the prevalence of acquired and genetic VTE risk factors and prothrombotic abnormalities in IBD.Overall,IBD patients have a two-to fourfold increased risk of VTE compared with healthy controls,with an overall incidence rate of 1%-8%.The majority of studies did not show significant differences in the risk of VTE between Crohn’s disease and ulcerative colitis.Several acquired factors are responsible for the increased risk of VTEin IBD:inflammatory activity,hospitalisation,surgery,pregnancy,disease phenotype(e.g.,fistulising disease,colonic involvement and extensive involvement)and drug therapy(mainly steroids).There is also convincing evidence from basic science and from clinical and epidemiological studies that IBD is associated with several prothrombotic abnormalities,including initiation of the coagulation system,downregulation of natural anticoagulant mechanisms,impairment of fibrinolysis,increased platelet count and reactivity and dysfunction of the endothelium.Classical genetic alterations are not generally found more often in IBD patients than in nonIBD patients,suggesting that genetics does not explain the greater risk of VTE in these patients.IBD VTE may have clinical specificities,namely an earlier first episode of VTE in life,high recurrence rate,decreased efficacy of some drugs in preventing further episodes and poor prognosis.Clinicians should be aware of these risks,and adequate prophylactic actions should be taken in patients who have disease activity,are hospitalised,are submitted to surgery or are undergoing treatment.  相似文献   

7.

Objectives

To evaluate the prevalence of osteopenia and osteoporosis in patients with inflammatory bowel disease (IBD) and to study the factors involved in their pathogenesis.

Methods

One hundred consecutive patients with IBD (57 women, mean age 41 years) were included in this study. Data were collected about their life habits, disease characteristics of medication use (mainly corticosteroids). Bone turnover markers were analyzed and the presence of osteoporosis or osteopenia was assessed with total hip and lumbar spine bone densitometry (DXA).

Results

Osteopenia percentages ranged from 37% (t-score measured by lumbar spine DXA) to 39% (hip DXA t-score). The prevalence of osteoporosis ranged from 2% (t-score measured by hip DXA) to 15% (lumbar spine DXA t-score). In the multivariate analysis, diagnosis of Crohn's disease (vs. ulcerative colitis; odds ratio 2.9, 95% CI 1-8.7) and the number of flares controlled by the cumulative dose of steroids (number of flares ≥3: odds ratio 8.7; 95%CI 1.6-45) were associated with a higher risk of osteopenia/osteoporosis. None of the analytical parameters significantly correlated with bone mineral density values.

Conclusions

The prevalence of osteopenia/osteoporosis is higher in patients with IBD (mainly those with Crohn's disease) than in the general population. Changes in bone metabolism seem to be more closely related to the inflammatory activity of IBD than to the steroid dose per se. Bone turnover markers did not correlate with the presence of osteopenia and osteoporosis.  相似文献   

8.

Objective

To determine the prevalence of immune-mediated inflammatory diseases (IMID) in a cohort of patients with inflammatory bowel disease (IBD) enrolled in hospital gastroenterology outpatients units for the AQUILES study, a prospective 2-year follow-up study.

Material and methods

We included patients ≥ 18 years old with a prior or new diagnosis of IBD (Crohn disease [CD], ulcerative colitis [UC] or indeterminate colitis). Diagnoses were collected in a cross-sectional manner from the clinical records at enrollment of a new patient in the study.

Results

We included 526 patients (mean age 40.2 years; 47.3% men, 52.7% women), 300 with CD (57.0%), 218 with UC (41.4%) and 8 with indeterminate colitis. Other types of IMID were present in 71 patients (prevalence: 13.5%, 95% CI: 10.8-16.7): 47 were spondyloarthropathies (prevalence: 8.9%); 18 psoriasis (3.4%); 5 pyoderma gangrenosum (1.0%), and 11 uveitis (2.1%). The prevalence of IMID was higher in patients with CD than in those with UC (17.0% [95% CI: 13.2-21.7] vs 9.2% [95% CI: 6.0-13.8], p = 0.011). In the multivariate analysis, the variables associated with the presence of IMID were diagnosis of CD (OR = 1.8 [95% CI: 1.1-3.2]) and duration of IBD ≥ 4 years (OR = 2.1 [95% CI: 1.1-4.1] in those with disease duration 4-8 years, and OR = 2.1 [95% CI: 1.2-3.9] in those with ≥ 8 years vs. < 4 years).

Conclusions

In the cohort of patients with IBD in the AQUILES study, 13.5% had another IMID, with a higher prevalence in patients with CD and > 4 years since disease onset.  相似文献   

9.
The risk of thromboembolism (TE) is increased in patients with inflammatory bowel disease (IBD), mainly due to an increased risk of venous TE (VTE). The risk of arterial TE (ATE) is less pronounced, but an increased risk of cardiovascular diseases needs to be addressed in IBD patients. IBD predisposes to arterial and venous thrombosis through similar prothrombotic mechanisms, including triggering activation of coagulation, in part mediated by impairment of the intestinal barrier and released bacterial components. VTE in IBD has clinical specificities, i.e., an earlier first episode in life, high rates during both active and remission stages, higher recurrence rates, and poor prognosis. The increased likelihood of VTE in IBD patients may be related to surgery, the use of medications such as corticosteroids or tofacitinib, whereas infliximab is antithrombotic. Long-term complications of VTE can include post-thrombotic syndrome and high recurrence rate during post-hospital discharge. A global clot lysis assay may be useful in identifying patients with IBD who are at risk for TE. Many VTEs occur in IBD outpatients; therefore, outpatient prophylaxis in high-risk patients is recommended. It is crucial to continue focusing on prevention and adequate treatment of VTE in patients with IBD.  相似文献   

10.
In this cross-sectional study, our aim was to analyze association of ambulatory blood pressure monitoring (ABPM) values with pulse wave velocity (PWV) in inflammatory bowel disease (IBD) patients as well as the prevalence and characteristics of white coat hypertension (WCH) in this group of patients with chronic inflammation and high prevalence of anxiety. We enrolled 120 consecutive IBD patients (77 Crohn´s disease; 43 ulcerative colitis) who were not treated with antihypertensive drugs without cardiovascular, cerebrovascular and renal morbidity. Office blood pressure, ABPM, and PWV were measured with Omrom M6, SpaceLab 90207, and Arteriograph, respectively. The prevalence of true normotension, sustained hypertension and WCH was analyzed in IBD patients. WCH was found in 27.5% patients. IBD-WCH patients had significantly lower prevalence of traditional risk factors than general WCH subjects. PWV and augmentation index (AIx) values were higher in WCH than in true normotensive patients. When adjusted for age and duration of IBD, only PWV was a positive predictor of WCH, and patients with higher PWV and longer disease duration had OR´s for WCH of 0.69 and 2.50, respectively. IBD patients had significantly higher prevalence of WCH and higher PWV values than healthy control patients. WCH is highly prevalent in IBD patients but IBD-WCH patients have lower frequency of traditional cardiovascular risk factors than general WCH population. Our results suggest that WCH could be considered as another clinical characteristic of IBD which is associated with increased arterial stiffness and those patients should be monitored more closely.  相似文献   

11.
Familial Mediterranean fever (FMF) is a hereditary disease, the most threatening complication of which is systemic amyloidosis. The thyroid gland may be asymptomatically involved in most patients with systemic amyloidosis secondary to FMF. However, clinically detectable thyroid goitre is quite rare, and until now only nine cases of thyroid goitre secondary to amyloid deposition in FMF have been reported. Of 1,100 FMF patients regularly followed up at our centre, thyroid goitre due to the accumulation of amyloid substance could be detected in only three (0.27%). In this report, we summarise the clinical and laboratory features of these patients. All three patients were euthyroid. Total thyroidectomy was performed for compressive symptoms in one patient and for aesthetic purposes in the other two. In countries with a high prevalence of FMF, such as Turkey, secondary amyloidosis of the thyroid gland should be borne in mind in long-standing FMF patients.  相似文献   

12.
Idiopathic inflammatory bowel diseases(IBD), Crohn's disease(CD) and ulcerative colitis(UC), are multifactorial diseases that are manifested after disruption of a genetic predisposed individual and its intestinal microflora through an environmental stimulus. Urbanization and industrialization are associated with IBD. Epidemiological data, clinical observations and family/immigrants studies indicate the significance of environmental influence in the development of IBD. Some environmental factors have a different effect on the subtypes of IBD. Smoking and appendectomy is negatively associated with UC, but they are aggravating factors for CD. A westernized high fat diet, full of refined carbohydrates is strongly associated with the development of IBD, contrary to a high in fruit, vegetables and polyunsaturated fatty acid-3 diet that is protective against these diseases. High intake of nonsteroidal antiinflammatory drug and oral contraceptive pills as well as the inadequacy of vitamin D leads to an increased risk for IBD and a more malignant course of disease. Moreover, other factors such as air pollution, psychological factors, sleep disturbances and exercise influence the development and the course of IBD. Epigenetic mechanism like DNA methylation, histone modification and altered expression of miR NAS could explain the connection between genes and environmental factors in triggering the development of IBD.  相似文献   

13.
OBJECTIVE: To explore the nature of the interrelationship between inflammatory disease of the spine/joints, skin, eye, and bowel [i.e., ankylosing spondylitis (AS), psoriasis, iritis, inflammatory bowel disease (IBD)]. METHODS: The study used 4 approaches: (1) analysis of the prevalence of secondary disorders within the AS individual (chi-square and matched pair analysis); (2) study of the temporal relationship between the onset of the different conditions; (3) evaluation of the prevalence of disease among first degree relatives; and (4) influence of secondary disorders on outcome of AS. RESULTS: 1. Among 3287 patients with AS, more than expected had either spondylitis associated with multiple co-disorders or pure AS (with no co-diseases); fewer than expected had AS plus a single co-disease (chi-square = 32.2, p < 0.001). In a matched pair analysis, patients with AS and a secondary disorder were more likely to have an additional concomitant disease, e.g., IBD-AS (n = 335) patients had a higher prevalence of iritis [45.4% vs 36.7%; OR 1.4 (1.1-2.0)] or psoriasis [23.9% vs 14.3%; OR 1.9 (1.3-2.8)] than controls. 2. Among our database subjects, the symptomatic onset of the spinal disease precedes or is contemporaneous with gut, skin, and eye involvement (matched pair t test, p < 0.001). 3. Patients with multiple disorders predict the highest prevalence of co-diseases (i.e., psoriasis, IBD, iritis, or AS) within family members, followed by those AS patients with only IBD, psoriasis, or iritis in descending order. 4. Both psoriasis and IBD increase severity in terrms of function and disease activity of AS in the patient. Radiological change is greatest for those AS subjects with iritis. CONCLUSION: There is a striking overlap within patients and family members of rheumatological, dermatological, and gastroenterological diseases. The susceptibility genes of these co-disorders appear to overlap with each other and with AS: 1. A patient with 2 inflammatory conditions is at an increased risk of developing an additional related inflammatory disorder. 2. Those with enteropathic spondylarthritis would appear to carry the greatest genetic load in terms of first degree relatives developing inflammatory conditions (including psoriasis and iritis that are not seen in the index IBD-AS patient). 3. The secondary disorders do not precede AS (arguing against psoriasis and IBD allowing for an environmental conduit to pathogenic triggers in AS). The susceptibility factors for these inflammatory conditions may be additive or have a synergistic effect on each other. There is evidence for a shared gene hypothesis.  相似文献   

14.
The association between inflammatory bowel disease (IBD) and primary sclerosing cholangitis should be considered a distinct clinical entity. This association involves genetic abnormalities, epidemiological factors (more common in men, with no a geographical pattern) and, commonly, subclinical inflammation, predominance of the right colon (endoscopic and histological), backwash ileitis and rectal sparing. Furthermore, there is an increased risk of colorectal cancer and cholangiocarcinoma. The aim of this review is to show how IBD influences the progression of this entity, transplantation requirements and recurrence. We also discuss the current evidence on the use of biological therapy in this group of patients.  相似文献   

15.
Inflammatory bowel disease(IBD) is the result of a combination of environmental,genetic and immunologic factors that trigger an uncontrolled immune response within the intestine,which results in inflammation among genetically predisposed individuals. Several studies have reported that the prevalence of classic cardiovascular risk factors is lower among subjects with IBD than in the general population,including obesity,dyslipidaemia,diabetes and hypertension. Therefore,given the risk profile of IBD subjects,the expected cardiovascular morbidity and mortality should be lower in these patients than in the general population. However,this is not the case because the standardized mortality ratio is not reduced and the risk of coronary heart disease is increased in patients with IBD. It is reasonable to hypothesize that other factors not considered in the classical stratification of cardiovascular risk may be involved in these subjects. Therefore,IBD may be a useful model with which to evaluate the effects of chronic low-grade inflammation in the development of cardiovascular diseases. Arterial stiffness is both a marker of subclinical target organ damage and a cardiovascular risk factor. In diseases characterized by chronic systemic inflammation,there is evidence that the inflammation affects arterial properties and induces both endothelial dysfunction and arterial stiffening. It has been reported that decreasing inflammation viaanti tumor necrosis factor alpha therapy decreases arterial stiffness and restores endothelial function in patients with chronic inflammatory disorders. Consistent with these results,several recent studies have been conducted to determine whether arterial properties are altered among patients with IBD. In this review,we discuss the evidence pertaining to arterial structure and function and present the available data regarding arterial stiffness and endothelial function in patients with IBD.  相似文献   

16.
17.
The past 18 months have seen many studies of the prevalence, pathogenesis, and treatment of the extraintestinal manifestations of inflammatory bowel disease (IBD). Inhibitors of tumor necrosis factor alpha have shown effectiveness in randomized trials for the treatment of spondyloarthropathies and ocular manifestations. Open-label studies suggest that these agents may be effective for pyoderma gangrenosum as well. The epidemiology of primary sclerosing cholangitis (PSC), and its relationship to IBD, is becoming clearer. Colorectal neoplasia in PSC remains an important clinical problem. Osteoporosis occurs more commonly in IBD, but the relative importance of corticosteroid use versus underlying chronic bowel inflammation as risk factors remains controversial. Chromoendoscopy may be an important means to improve detection of colorectal neoplasia in IBD. Observational studies suggest that prolonged use of aminosalicylates is associated with decreased risk of neoplasia, but data are conflicting. A randomized trial of ursodeoxycholic acid in PSC showed decreased risk of colorectal neoplasia in patients receiving the drug relative to those on placebo.  相似文献   

18.
BACKGROUND/AIMS: The aim of the present study was to investigate the familial occurrence of autoimmune diseases in a large group of patients with primary sclerosing cholangitis (PSC). METHODS: All patients with PSC treated at Huddinge University Hospital between 1984 and 1999 were included (n=145). For every patient with PSC and inflammatory bowel disease (IBD) (n=126) we randomly selected a control patient with IBD (n=126), matched for age, sex and type of IBD. A questionnaire comprising information about autoimmune diseases among first-degree relatives was answered by all patients and controls. RESULTS: We identified 22 index cases with PSC from 21 families with a first-degree relative with either chronic liver disease and/or IBD. Five patients with PSC had a first-degree relative with PSC (3.4%). The prevalence of PSC among first-degree relatives was 0.7% (5/717). In siblings the prevalence was 1.5% (4/269). The prevalence of first-degree relatives with autoimmune diseases outside the liver was similar in PSC patients and controls. CONCLUSIONS: First-degree relatives of patients with PSC have a PSC prevalence of 0.7%. This represents a nearly 100-fold increased risk of developing PSC compared with the general population, supporting the hypothesis that genetic factors are of importance for development of PSC.  相似文献   

19.
Inflammatory bowel diseases(IBD), conventionally consist of Crohn's disease(CD) and ulcerative colitis. They occur in individuals with high risk genotype for the disease in the setting of appropriate environmental factors. The pathogenesis of IBD involves a dysregulated autoimmune response to gut dysbiosis, which in turn is triggered due to exposure to various inciting environmental factors. But there is no clearly defined etiology of IBD and this type of disease is termed as "idiopathic IBD", "classic IBD", or "primary IBD". We reviewed the current medical literature and found that certain etiological factors may be responsible for the development of IBD or IBD-like conditions, and we consider this form of de novo IBD as "secondary IBD". Currently known factors that are potentially responsible for giving rise to secondary IBD are medications; bowel altering surgeries and transplantation of organs, stem cells or fecal microbiome. Medications associated with the development of secondary IBD include; immunomodulators, anti-tumor necrosis factor alpha agents, anti-interleukin agents, interferons, immune stimulating agents and checkpoint inhibitors. Colectomy can in some cases give rise to de novo CD, pouchitis of the ileal pouch, or postcolectomy enteritis syndrome. After solid organ transplantation or hematopoietic stem cell transplantation, the recipient may develop de novo IBD or IBD flare. Fecal microbiota transplantation has been widely used to treat patients suffering from recurrent Clostridium difficile infection but can also causes IBD flares.  相似文献   

20.

Introduction

Inflammatory bowel disease (IBD) is an independent risk factor for thromboembolic phenomena (TEP). We evaluated the prevalence and the possible risk factors associated with developing TEP in patients with IBD in our center.

Material and methods

Data were retrospectively collected from January 1995 to December 2011 from 23 patients. A total of 61% were diagnosed with Crohn's disease (CD) and 39% with ulcerative colitis (UC) according to routine criteria.

Results

When the Montreal classification was used, 58% of the patients with CD had an inflammatory pattern (B1), 25% a stenosing pattern (B2) and 17% a fistulizing pattern (B3). Half the patients had ileocolic involvement (L3), one-third had colonic involvement (L2) and the remainder had ileal involvement (L1). Among patients with UC according to the Montreal classification, 78% had extensive colitis (E3), 11% had left colonic involvement (E2) and 11% had proctocolitis (E1). During the event, almost half the patients with UC had severe inflammatory activity (S3; 44%), 33% had mild-moderate activity (S1: 22%, S2: 11%) and only 22% were in remission (S0). Overall, at the time of the TEP, 48% of the patients had mild-moderate activity and 22% had severe activity. Likewise, 44% were hospitalized at the time of the event. In UC, an increase in the prevalence of TEP was found in admitted patients (66%). None of the patients had a family history of TEP, two patients (9%) had associated thrombophilia and 26% were active smokers. There were no TEP during pregnancy. Only one patient was taking contraceptive pills when the event occurred. The most frequent forms of TEP were deep vein thrombosis of the legs (55%) followed by pulmonary thromboembolism (25%).

Conclusions

TEP are relatively frequent in patients with IBD, with a strong impact on morbidity and mortality. In our series, risk factors for these events were more extensive involvement (any of the groups) and severe inflammatory activity. No significant association between classical risk factors such as the use of contraceptives, pregnancy, coagulation disorders or smoking and the risk of TEP were found.  相似文献   

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