Extraintestinal Manifestation Of Ulcerative Colitis Research Articles

Ulcerative Colitis Associated with Isolated Unilateral Hypoglossal Nerve Palsy

We herein describe a rare case of ulcerative colitis associated with unilateral hypoglossal nerve palsy. A 64-year-old woman developed severe active ulcerative colitis and was treated with prednisolone. The dose of oral prednisolone was reduced to 7.5 mg/day, following which the patient noticed slight dysphagia and a speech disturbance. She was diagnosed with unilateral hypoglossal nerve palsy, which was thought to be caused by mononeuritis. She was treated with intravenous methylprednisolone at a dose of 500 mg/day, which improved the neuropathy. When the neuropathy occurred, the patient was in a mildly active stage of ulcerative colitis. We concluded that the mononeuritis observed in the present case was likely an extraintestinal manifestation of ulcerative colitis.

Risk Factors Associated with Refractory Pouchitis Following Ileal Pouch Anal-Anastomosis

Purpose: Patients with severe medically refractory ulcerative colitis commonly undergo total proctocolectomy with ileal pouch-anal anastomosis (IPAA). This surgery occurs in 1, 2 or 3 stages. Up to 50% of patients develop pouchitis, diagnosed based on various clinical, endoscopic, and histological findings. Multiple risk factors for pouchitis have been suggested, including bacterial dysbiosis, presence of pANCA titers, extra-intestinal manifestations of ulcerative colitis, use of preoperative steroids and NSAIDs. While many different therapies have been studied, antibiotics are the mainstay of treatment. Approximately 10% of patients with pouchitis develop antibiotic refractory pouchitis and require immunomodulators, biologics, and/or surgery. There are no randomized trials in the literature and maintenance of remission in refractory pouchitis remains a challenge. The aim of this study was to identify risk factors leading to the development of refractory pouchitis. Methods: A retrospective analysis was performed of patients who underwent IPAA from 2003-2010 at a single tertiary care center. The variables of pre-operative diagnosis, age, gender, Charlson comorbidity index score, and diagnosis and treatment of pouchitis were assessed. Patients were excluded if they had less than one year follow-up documented in the chart or a surgical complication leading to diagnosis of failed IPAA. Results: A total of 112 patients were included in the study (91 UC, 1 CD, 9 IC, 11 FAP). The mean age at diagnosis was 26.8 years, 59 patients were male (52.7%), and the mean Charlson score was 0.69. Pouchitis was diagnosed in 67 patients (59.8%) and all received antibiotics for initial management. Of those patients with pouchitis, 46 (68.7%) were also given probiotics, 4 (5.9%) received immunomodulators (azathioprine, methotrexate, or 6-mercaptopurine), and 8 (11.9%) received biologics (infliximab, adalimumab, or certolizumab). A total of 6 patients (8.9%) required management with pouch excision or revision. Univariate analysis identified a trend towards significance for number of stages (p=0.08) and post-operative diagnosis change to Crohn's Disease (p=0.08) for predicting such refractory disease. Conclusion: We hypothesized that certain patients may be predisposed to refractory pouchitis. While more data is needed to prove significance, the patients with more aggressive distal disease necessitating a 3 stage procedure may be at higher risk for subsequent pouchitis. This also may influence the conversion to phenotypically identified Crohn's disease. Additionally, operative techniques for the IPAA itself need to be investigated. Given the difficulties of management, further studies are needed to identify patients at risk for development of refractory pouchitis.

Extraintestinal manifestations of inflammatory bowel diseases and their management

Extraintestinal manifestations of ulcerative colitis and Crohn's disease are symptoms of varied organ injuries due to systemic inflammatory process. Bowel disease seems to be primary but upon genetic disposition disturbed immune regulation may result in inflammation of other organs that may appear simultaneously, precede bowel disease or follow it in the quiescent phase. As far as possible extraintestinal manifestations have to be distinguished from the complications of the intestinal inflammation and also from side effects of drugs for its treatment. Involvement of bones and joints, skin, eyes, liver and biliary tract is more common but disease of blood coagulation system, lung, kidneys and nervous system may also appear. Treatment of extraintestinal manifestations is influenced by their association with the activity of the bowel disease. Beyond some specific interventions, to achieve improvement of manifestations independent from activity long lasting anti-inflammatory therapy or immunomodulation is almost always needed. Clinical evidence of different levels from reports support the favorable effect of biologic therapy in different extraintestinal manifestations of inflammatory bowel diseases as well. Care and follow-up of patients is a multidisciplinary task.

Clinical characteristics of ulcerative colitis associated with primary sclerosing cholangitis in Korea

Primary sclerosing cholangitis (PSC) is well known as one of the extraintestinal manifestations of ulcerative colitis (UC). However, the prevalence of PSC in UC patients together with the clinical characteristics and outcomes of UC associated with PSC (UC-PSC) are not clear in Asians. We retrospectively reviewed the medical records of UC patients who were registered and followed up at the Asan Medical Center. The prevalence of PSC in UC and the clinical features of UC-PSC compared with matched UC without PSC were investigated. A total of 1849 patients diagnosed with UC between July 1977 and September 2009 were reviewed. Among these, 21 UC-PSC patients (1.1%) were identified. The cumulative probability of PSC after diagnosis of UC was 0.71% after 1-5 years, 1.42% after 10 years, 2.59% after 15 years, and 3.35% after 20-25 years. Compared with 63 matched UC alone patients, UC-PSC showed pancolitis (95.2%), rectal sparing (38.1%), and backwash ileitis (42.9%) more frequently (P < 0.001). During follow-up, three of 21 UC-PSC patients (14.3%) were diagnosed with colorectal neoplasia and three patients (14.3%) died of PSC-associated complications. UC-PSC patients were associated with increased probability of colorectal neoplasia development (P = 0.036) and a trend toward increased mortality compared with the UC alone group. The prevalence of PSC in Korean UC patients appears to be lower than that of Western patients. Similar to Caucasians, UC-PSC shows unique colonoscopic features and is associated with more frequent colorectal neoplasia development and poor prognosis in Korea.

A rare extraintestinal manifestation of ulcerative colitis: Tracheobronchitis associated with ulcerative colitis

We describe a 44 year-old woman who presented with dyspnea and cough 5 years after initial diagnosis of ulcerative colitis. The evaluation of patient showed findings of large airway inflammation with tracheal wall thickening and mucosal irregularities. The etiology was investigated and was diagnosed as tracheobronchitis that is a rare extraintestinal manifestation of ulcerative colitis. She was treated with systemic corticostreoids and rapid clinical improvement was detected.

W1238 Investigation of Crohn's Disease Associated Loci in Ulcerative Colitis Points Towards Common Susceptibility Genes in the TH17 Pathway and Innate Inmunity

and Systemic Lupus Erythematosus (SLE). Based on the functional differences described for Hp1 and Hp2 and its immunomodulatory role, we studied the Hp locus in Ulcerative Colitis (UC) and Crohn's disease (CD). Methods and patients. We genotyped these alleles in an UC cohort (n=755), an exploratory cohort of CD patients (CD1, n=429), a second independent CD cohort (CD2=632), and in 452 healthy controls. Hp genotypes were determined using touch-down polymerase chain reaction (PCR). Distribution of alleles was in HardyWeinberg equilibrium. Chi-square test was performed to evaluate the differences between groups (SPSS 16.0). P values less than 0.05 were considered statistically significant. Results. The Hp2 allele frequency was significantly higher in UC patients ( p=0.006) and in CD1(p<0.0001) and CD2 (p=0.016), all compared to the same control group (table 1). Also the distribution of the genotypes was significantly different between UC and controls (p= 0.0046), and between CD1 (p<0.00003) and CD2 (p<0.018) and controls, mainly through an overrepresentation of Hp 2-2 in both UC and CD patients. Furthermore, the differences between CD combined group (1+2) and controls were also significant regarding allele freq (p <0.0004) and genotypes distribution (p<0.0001). We found no significant association between haptoglobin genotypes and smoking status at diagnosis, familial disease, disease extension, type of medical treatment, need of surgery, dysplasia or extraintestinal manifestations in UC and CD patients. Conclusion. Polymorphisms in the Hp gene play a role in susceptibility to IBD. The higher frequency of the functional allele 2 in IBD patients suggests a decrease in the Hp anti-inflammatory capacity. Hp 2-2, as in other immune diseases like RA and SLE, is more frequent in CD and UC, and may provoke an imbalance in Th1/ Th2 response. Table 1. Allele frequency and genotypes in controls, UC and CD.

Pericardial Tamponade in a Patient with Inactive Ulcerative Colitis

Cardiac tamponade is a rare extraintestinal manifestation of ulcerative colitis. We present a case report of tamponade occurring four years after curative proctocolectomy and in the absence of any medical therapy for ulcerative colitis. Options for medical management of this condition are also discussed.

Joint Extraintestinal Manifestations in Ulcerative Colitis

An extraintestinal manifestation (EIM) very often occurs in ulcerative colitis (UC) patients. EIM modifies the natural course of UC and decreases the quality of life in these patients. The aim of this study was to analyze clinical and laboratory findings in UC patients with joint EIM. 319 UC patients were examined. Among them were 131 (41.1%) patients with distal UC, 102 (32.0%) suffered from left-sided UC and 86 (26.9%) had pancolitis. 95 (29.8%) UC patients had joint EIM. Arthritis correlated with extensive forms of UC and was more often determined in patients with left-sided UC and pancolitis. Arthralgia was a prevalent symptom of joint EIM in patients with distal UC. Colon microbiocenosis and the mucosal barrier in UC patients were analyzed. The cytokine status with privileged cytokine profile changes was investigated. In all UC patients, dysbiosis with a decreasing quantity of bifidobacteria, lactobacilli and зscherichia coli was found, but an increase of facultative flora was also found. At the same time, an association of facultative flora in UC patients with arthritis was observed. In these patients, Staphylococcus, Klebsiella and Proteus were found more often in stool cultures. These associations correlated with a modification of the colonocytes’ cell receptor maturity of mucus, a condition with a decreased staining intensity by lectins. A cytokine imbalance with an increase of proinflammatory and a decrease of anti-inflammatory cytokines was found in all UC patients. The privileged cytokine profile changes in UC patients with joint EIM were analyzed. Maximal increases of IL-1 and TNF with decreases of IL-10 in plasma in patients with joint EIM were observed.

A case of tracheobronchitis in ulcerative colitis: a review of literature

Tracheobronchitis is a rare extraintestinal manifestation of ulcerative colitis (UC) and is often mis-diagnosed and treated as asthma. We report a case of tracheobromchitis in a patient with long-standing UC, who had been treated for asthma for many years with a poor response to standard asthma management. Only very few cases are reported and in the majority of them tracheobronchitis developed after colectomy. Subsequent investigations of unremitting respiratory symptoms in our patient revealed tracheobronchitis. His UC remained quiescent for more than 20 years on medical treatment having never required a bowel resection. On thoroughly investigating the aetiology for his pulmonary pathology, it was thought to be an extraintestinal manifestation of UC, which makes this case noteworthy. Pulmonary complications can develop many years after the diagnosis of inflammatory bowel disease, without any sign of disease activity. In such cases awareness and a high index of suspicion are important to ensure early diagnosis and treatment, and further investigations to detect large airway disease is warranted. The pulmonary manifestations tend to respond well to steroid therapy, which may prevent or ameliorate permanent lung damage.

Predictors of Pouchitis after Ileal Pouch-Anal Anastomosis: A Retrospective Review

The primary end point of this study was to determine the risk factors that predict chronic pouchitis in those patients having ileal pouch-anal anastomosis. A total of 237 patients with ulcerative colitis and undergoing ileal pouch-anal anastomosis by one surgeon at Oregon Health & Science University from 1993 to 2003 were evaluated. Data were gathered via retrospective chart reviews and by a questionnaire administered by telephone in 2004. Patients were excluded if there was less than one-year follow-up documented in the chart or they could not be contacted by telephone (n = 62), postoperative diagnosis of Crohn's disease (n = 3), failed ileoanal procedure (n = 1), and one-stage ileal pouch-anal anastomosis (n = 3), leaving 167 patients for evaluation. Patients were defined as having chronic pouchitis (> 3 episodes of pouchitis) or no pouchitis (< or = 3 episodes of pouchitis). Potential risk factors included number of operations used to perform ileal pouch-anal anastomosis, fulminant ulcerative colitis with two-stage operation, duration of diverting ileostomy after pouch formation, primary sclerosing cholangitis, other extraintestinal manifestations of ulcerative colitis, preoperative liver function tests, duration of ulcerative colitis, and the occurrence of postoperative complications. Initial univariate analysis was performed on all risk factors. Multivariate analysis was performed on all univariate risk factors with P values < 0.2. The prevalence of chronic pouchitis in our population was 46 percent. The following variables were identified during univariate analysis and entered into a multivariate model: preoperative serum albumin (P = 0.07), PSC (P = 0.126), duration of diverting ileostomy (P = 0.111), fulminant ulcerative colitis with two-stage operation, (P = 0.051), the presence of postoperative complications (P = 0.031), and the type of postoperative complications (anastomotic complications, P = 0.013). Patients who did not undergo diverting ileostomy at the time of their ileal pouch-anal anastomosis trended toward a lower likelihood of developing chronic pouchitis (P = 0.06). Multivariate analysis showed that patients with postoperative complications (53 percent, P = 0.042), specifically anastomotic complications, were more likely to develop chronic pouchitis (P = 0.005). Eight percent of patients had primary sclerosing cholangitis and 11 percent of patients had at least one extraintestinal manifestation of ulcerative colitis. Patients with primary sclerosing cholangitis were not more likely to develop chronic pouchitis (P = 0.168). Patients with extraintestinal manifestations also were not more likely to develop chronic pouchitis (P = 0.273). Chronic pouchitis is a frequent complication after ileal pouch-anal anastomosis. In this study patients with primary sclerosing cholangitis or other extraintestinal manifestations of ulcerative colitis were not more likely to develop chronic pouchitis. Patients with postoperative complications, specifically anastomotic complications after ileal pouch-anal anastomosis, were more likely to develop chronic pouchitis and may benefit from early strategies to prevent pouchitis.

Rare Extra-Intestinal Manifestation of Ulcerative Colitis

Rare Extra-Intestinal Manifestation of Ulcerative Colitis

Subretinal fibrosis and uveitis syndrome associated with ulcerative colitis

We report a case of subretinal fibrosis and panuveitis syndrome as an extraintestinal manifestation of ulcerative colitis. We present an interventional case report of a 40-year-old female patient with inactive ulcerative colitis referred to our center for refractory uveitis in her right eye. She was diagnosed with subretinal fibrosis and uveitis syndrome after work-up ruled out tuberculosis, bartonellosis, Lyme disease, and sarcoidosis. Right eye multifocal choroiditis and panuveitis syndrome with subretinal fibrosis was diagnosed, and posterior pole subretinal fibrosis caused a 90% loss of visual efficiency. She had concurrent dermatological manifestations but no gastrointestinal symptoms. One year of immunosuppressive therapy with methotrexate (7.5 mg/week) controlled ocular inflammation. Two colitis episodes, with no visual involvement, were recorded during follow-up. Her final, right-eye, best-corrected visual acuity remained stable at 0.160 due to scarring sequela after 7 years of follow-up. Subretinal fibrosis and uveitis syndrome can occur as a sight-threatening extraintestinal manifestation of ulcerative colitis.

Retrospective survey of 452 patients with inflammatory bowel disease in Wuhan City, central China

Inflammatory bowel disease (IBD) had been uncommon in China until about 1990, but since then, it has been seen in the clinical setting more and more. The prevalence and phenotype of IBD in the Chinese population is not well known. The present study investigates the trend of prevalence in ulcerative colitis (UC) and Crohn's disease (CD) in Wuhan City, central China, and evaluates clinical features, extraintestinal manifestations, and the treatment of IBD in the last 14 years. Three hundred and eighty-nine patients with UC and 63 patients with CD were retrospectively collected from 5 central hospitals in Wuhan City, in which high-quality endoscopic and histological diagnoses were available from 1990 to 2003. UC and CD were diagnosed based on clinical, experimental, radiological, endoscopic, and histological examinations according to the internationally accepted Lennard-Jones criteria. The trend toward prevalence of UC and CD increased between 1990 and 2003 in Wuhan City. There was no change in the sex and age distribution comparing 1990 to 1996 with 1997 to 2003 both in UC and CD. However, the number of individuals with higher education and a professional occupation during 1997 to 2003 was significantly higher than that during the period 1990 to 1996 in patients with UC (OR 2.1, 95% CI 1.27-3.35, P = 0.004; OR 2.2, 95% CI 1.31-3.61, P = 0.003). The mean age of patients with CD was significantly younger than that of UC at the time of diagnosis (32.6 +/- 12.5 vs. 42 +/- 14.5, P < 0.0001). The ratio of male to female patients was 1.53:1 in UC and 2.32:1 in CD, respectively. The mean duration of onset of the disease to diagnosis was 1.4 years in UC and 1.1 years in CD. The extra intestinal manifestations of UC and CD were 5.7% and 19%, respectively, and complications of UC and CD were 6.4% and 50.8%, respectively. Only 3% of UC patients required surgery, whereas 27% of CD patients underwent surgical procedures (P < 0.001). The prevalence of IBD has increased in Wuhan City, central China, but is not as high as in Western countries. The disease in Wuhan City has often been associated with young adult professional males with a high level of education. The clinical presentation of UC was often mild and had few extra intestinal manifestations.

Concomitant Aseptic Subcutaneous Abscess and Immunoglobulin M Nephropathy—Rare Extraintestinal Manifestations in Ulcerative Colitis

Concomitant Aseptic Subcutaneous Abscess and Immunoglobulin M Nephropathy—Rare Extraintestinal Manifestations in Ulcerative Colitis

Acute deafness as an extraintestinal manifestation of ulcerative colitis

Autoimmune sensorineural hearing loss is a syndrome with an immunological mechanism occasionally associated with diverse autoimmune diseases. We describe a case of sensorineural hearing loss in a patient with ulcerative colitis. Several pathogenic mechanisms have been invoked. Treatment is steroid therapy and should be initiated when symptoms appear to restore hearing.

Predictors of Pouchitis Following Ileal Pouch Anal Anastamosis

Purpose: Primary endpoint is to determine risk factors that predict chronic pouchitis in those patients having ileal pouch anal anastamosis (IPAA.) Methods: 237 patients with ulcerative colitis (UC) and undergoing IPAA by one surgeon at Oregon Health & Science University from 1993–2003 were evaluated. Data were gathered via retrospective chart reviews and by a questionnaire administered by telephone in 2004. Patients were excluded if there was less than one year follow-up documented in the chart or they could not be contacted by telephone (n = 63), post-operative diagnosis of Crohn's disease (n = 3), failed ileoanal procedure (n = 1), and 1-stage IPAA (n = 3.) Patients were defined as having chronic pouchitis (CP = >3 episodes of pouchitis) or having no pouchitis (≤3 episodes of pouchitis.) Potential risk factors included number of operations used to perform IPAA, fulminant UC with 2-stage operation, duration of diverting ileostomy following pouch formation, primary sclerosing cholangitis (PSC), other extra-intestinal manifestations (EIMs) of UC, pre-operative liver function tests, duration of UC, and the occurrence of post-operative complications. Initial univariate analysis was performed on all risk factors. Multivariate analysis was performed on all univariate risk factors with p values < 0.2. Results: The prevalence of CP in our population was 46%. The following variables were identified during univariate analysis and entered into a multivariate model: pre-operative serum albumin (p = .07), PSC (p = 0.126), duration of diverting ileostomy (p = 0.111), fulminant UC with 2-stage operation, (p = .051), and occurrence of post-operative complications (p = .007). Patients who did not undergo diverting ileostomy at the time of their IPAA trended towards a lower likelihood of developing CP (p = 0.06.) Multivariate analysis showed that patients with post-operative complications (53%) were more likely to develop CP (p = .009.) 8% of patients had PSC and 11% of patients had at least one EIM of UC. Patients with PSC and EIMs were not more likely to develop CP (p = 0.273, p = 0.126.) Conclusions: Chronic pouchitis (CP) is a frequent complication following IPAA. In this study patients with PSC or other EIMs of UC were not more likely to develop CP. Patients with post-operative complications following IPAA were more likely to develop CP and may benefit from early strategies to prevent pouchitis.

Extraintestinal manifestations in ulcerative colitis

Extraintestinal manifestations in ulcerative colitis

Biopsychosocial Determinants of Health-Related Quality of Life After Ileal Pouch Anal Anastomosis for Ulcerative Colitis*

In patients with ileal pouch anal anastomosis (IPAA) the influences of psychosocial variables and of extraintestinal manifestations of ulcerative colitis (UC) on health-related quality of life (HRQOL) have not been studied so far. 61 patients with UC (age 52.7+/-13.9 years; 47% female) completed the German version of the Inflammatory Bowel Disease Questionnaire (IBDQ-D), the Short Form Health Survey (SF - 36), the German version of the Hospital Anxiety and Depression Scale (HADS-D) and the Giessener Symptom List (GBB 24). Independent of their current clinical activity 37 patients underwent endoscopies. Pouchitis was defined by the Pouch Disease Activity Score (PDAI) > or = 7. Where possible, IPAA-patients were compared with the data for the German general population and with a clinical sample of patients with UC and no IPAA. Patients with IPAA complained more about fatigue and arthralgia and a reduced physical and mental health (SF-36) than the German general population (P < 0.01). The IBDQ-total score could be predicted (adjusted R2 = 29.1, P < 0.01) by the number of operations due to IPAA-related complications (beta = -18.8) and HADS-D-Anxiety scores > or = 11 (beta = -29.1). The IBDQ-subscale score "Bowel" could be predicted (adjusted R2 = 13.7, P = 0.04) by PDAI > or = 7 (beta = -9.2) and the subscale score "Systemic" (adjusted R2 = 13.3, P = 0.04) by the number of extraintestinal manifestations (beta = -1.9). HRQOL of patients with UC and IPAA is determined not only by IPAA-related factors but also by anxiety and extraintestinal manifestations with associated musculoskeletal pain.

Cigarette smoking and appendectomy are risk factors for extraintestinal manifestations in ulcerative colitis.

Two common factors, cigarette smoking and appendectomy, have been found to play a role in ulcerative colitis (UC). Data on their role in the development of extraintestinal manifestations (EIM) are scarce. The relationship between cigarette smoking, appendectomy, and EIM was examined in a prospective study involving 535 (M/F = 319/216) consecutive UC patients followed up for 18 yr. We considered the major EIM: seronegative spondyloarthropathy, pyoderma gangrenosum/erythema nodosum, acute anterior uveitis, and primary sclerosing cholangitis. We excluded patients with a history of EIM or those colectomized before study entry, ex-smokers, and those who started to smoke during the course of UC. In UC patients, seronegative spondyloarthropathy and dermatologic complications were found increased in smokers (p < 0.0001; p = 0.001) or in subjects with appendectomy (p = 0.0003; p = 0.02), while acute anterior uveitis and primary sclerosing cholangitis did not differ. The Kaplan-Meier analysis showed 18-yr rates for EIM of 71% in smokers and 45% in nonsmokers (log-rank test, p = 0.0001), and of 85% in patients with appendectomy and 48% in those without (p = 0.0001). Cox proportional-hazard model showed that cigarette smoking and appendectomy are independent factors promoting EIM. In smokers with appendectomy the adjusted hazard ratio (3.197, 95% CI 1.529-6.684) was higher than in patients with appendectomy alone (2.617, 95% CI 1.542-4.442) or smoking alone (1.947, 95% CI 1.317-2.879). In UC patients, appendectomy and cigarette smoking are prognostic factors for the development of EIM. The unfavorable effect of cigarette smoking on EIM is additive to that of appendectomy.

Cigarette smoking and appendectomy are risk factors for extraintestinal manifestations in ulcerative colitis

Cigarette smoking and appendectomy are risk factors for extraintestinal manifestations in ulcerative colitis