Budd-Chiari syndrome in a patient with ulcerative colitis and no inherited coagulopathy

We report a case of 27 year old female patient who was admitted to the hospital with an acute flare up of ulcerative colitis.The patient presented with complaints of persistent abdominal pain and bloody diarrhea despite aggressive therapy for her ulcerative colitis.A CT scan of the abdomen on admission revealed hepatic vein thrombosis,suggesting a diagnosis of Budd-Chiari syndrome.Significantly,an associated thrombosis of the inferior mesenteric vein was also detected.Based on imaging data and clinical assessment,the patient was started on anticoagulation therapy and an extensive work-up for hypercoagulability was initiated.Up to the time of publication,no significant findings suggesting this patient has an underlying coagulation disorder have been found.Based on our search of PUBMED,this report is one of only five reported adult cases of Budd-Chiari Syndrome associated with ulcerative colitis in the English literature in living patients without evidence of a co-existing coagulation disorder.This case highlights the potential for thrombosis at unusual sites in ulcerative colitis patients even in the absence of classical coagulation abnormalities.In addition to the case presented,we provide a brief review of previously reported cases of Budd-Chiari Syndrome occurring in patients with inflammatory bowel disease.     

Tenosynovitis of the ankles as onset of sarcoidosis in a patient with ulcerative colitis

Arthritis and tenosynovitis are frequently reported as complications of inflammatory bowel diseases. About 10% of patients with ulcerative colitis presents articular inflammation, usually in the phases of activity of intestinal disease. Tenosynovitis is also a frequent complication of ulcerative colitis. We describe here a case of tenosynovitis of both ankles occurring in a patient affected by ulcerative colitis not in active phase. Chest X-ray and TC showed hilar lymph node enlargement and transbronchial biopsy confirmed the diagnosis of sarcoidosis. In this disease tenosynovitis is very rare, unlike arthritis that is rather common. In conclusion we observed a case of ankle bilateral tenosynovitis as onset manifestation of sarcoidosis.     

An open-label trial of the PPARγ ligand rosiglitazone for active ulcerative colitis

OBJECTIVES: Previous research has demonstrated that ligands for the gamma subtype of peroxisome proliferator-activated receptors (PPARs) reduce inflammation in two different murine models of colitis. This study was designed to examine the potential efficacy of rosiglitazone, a ligand for the gamma subtype of PPARs, as a therapy for active ulcerative colitis. METHODS: Fifteen patients with mild to moderately active ulcerative colitis despite therapy with 5-aminosalicylic acid compounds were enrolled in an open-label study of rosiglitazone (4 mg b.i.d. p.o.) for 12 wk. Thirteen of 15 patients were receiving concomitant therapy with corticosteroids and/or immunomodulator medications. Disease activity was measured with the Disease Activity Index. RESULTS: After 12 wk of therapy, four patients (27%) had achieved clinical remission, of whom three (20%) also had an endoscopic remission. Four additional patients (27%) had a clinical response without achieving remission. Two patients were hospitalized with worsened disease activity, and one patient was withdrawn for nephrotic syndrome. CONCLUSIONS: These data suggest that ligands for the gamma subtype of PPARs may represent a novel therapy for ulcerative colitis. A double blind, placebo-controlled, randomized trial is warranted.     

Appendiceal orifice inflammation as a skip lesion in ulcerative colitis: an analysis in relation to medical therapy and disease extent

BACKGROUND: Although several reports have claimed that the appendix can be involved as a skip lesion in ulcerative colitis, they do not exclude the possibility that this skip lesion occurs as a result of medical therapy. Also, little is known about the relation between the presence of appendiceal orifice inflammation and the extent of the disease. METHODS: The presence of appendiceal orifice inflammation was prospectively assessed both endoscopically and histologically in 94 patients with active ulcerative colitis, the extent of whose disease had not been beyond the hepatic flexure. To evaluate the effect of prior medical therapy on the prevalence of appendiceal orifice inflammation, all cases were divided into two groups. Group A consisted of 66 patients who had been treated before inclusion; group B was composed of 28 patients newly diagnosed at inclusion. RESULTS: Appendiceal orifice inflammation was diagnosed in 24 (26%) of 94 patients with active subtotal ulcerative colitis, with no statistical difference observed between group A (23%) and group B (32%). In all 94 patients, the frequency of appendiceal orifice inflammation decreased significantly as the extent of disease increased, i.e., 37% in proctitis (n = 49), 17% in left-sided colitis (n = 36), and 0% in extensive colitis (n = 9) (p < 0.05). CONCLUSIONS: Appendiceal orifice inflammation as a skip lesion of ulcerative colitis is not rare, is more frequently observed in patients with less extensive disease, and is not the result of patchy improvement due to medical therapy.     

Colonic malacoplakia and ulcerative colitis: report of a case

Malacoplakia is a form of chronic granulomatous inflammatory reaction that rarely affects the digestive tract and has exceptionally been reported in association with ulcerative colitis. We report a new case in a 58-year-old woman suffering from ulcerative colitis. As colitis worsened, the patient received systemic steroid therapy but symptoms did not improve. As colonic perforation was suspected, a sub-total colectomy was performed. Histopathological study revealed a diffuse infiltration of the colonic mucosa by sheets of large macrophages with eosinophilic granular cytoplasm and characteristic cytoplasmic inclusions (Michaelis-Gutmann bodies) together with active and chronic lesions of ulcerative colitis. Malacoplakia gradually disappeared under antibiotics and did not recur whereas ulcerative colitis remained active. In our case, as in three similar published cases associated with ulcerative colitis or Crohn's disease, malacoplakia was probably triggered by steroid therapy and was not clinically suspected. This particular and potentially severe inflammation must be recognized and treated in order to prevent worsening of the associated bowel disease.     

Hypokalemic nephropathy after pelvic pouch procedure and protective loop ileostomy

Proctocolectomy with ileal pouch-anal anastomosis and temporary ileostomy has been established as a curative operation in severe ulcerative colitis during the last 2 decades. Electrolyte imbalances during the first postoperative weeks until ileostomy closure have been reported previously. Here we report about a 70-year-old male patient with a 38 year-history of severe ulcerative colitis who developed slowly progressive renal failure after proctocolectomy with ileal pouch-anal anastomosis and temporary ileostomy. He was referred to our centre with a serum creatinine of 818 micromol/L, hypokalemia of 2.83 mmol/L and metabolic alkalosis as a patient with suspected end-stage renal disease in order to perform shunt surgery and start chronic hemodialysis. However, hypokalemia and metabolic alkalosis are not typical for end-stage renal disease, and renal biopsy showed typical signs of hypokalemic nephropathy. Our patient almost completely recovered after ileostomy closure.This case clearly shows that temporary ileostomy in patients who underwent proctocolectomy, e. g. for ulcerative colitis, is associated with a risk of hypokalemic nephropathy. The appropriate and definite therapy is a surgical one, i. e. ileostomy closure. Monitoring metabolic changes after proctocolectomy and ileostomy, especially during the defunctionalized stage when temporary ileostomy is still present, is essential.     

A case of familial Mediterranean fever and polyarteritis nodosa complicated by spontaneous perirenal and subcapsular hepatic hemorrhage requiring multiple arterial embolizations

The association of familial Mediterranean fever (FMF) and polyarteritis nodosa (PAN) has been well established. These patients have been reported to have an overall better prognosis than other PAN patients. Herein we report a patient with FMF and PAN who died of sepsis following a severe course of recurrent bleeding episodes which required multiple embolization attempts. The 39-year-old Turkish male presented with abdominal pain of 1-month duration. He had been diagnosed with FMF at the age of 24. On admission, he had pallor with general ill appearance. Rebound tenderness was obtained in the right upper abdominal quadrant. He had mild anemia, leukocytosis, thrombocytosis, and hypoalbuminemia. On the 2nd day of his admission, he developed hypotension with a rapid decline in hemoglobin level. Abdominal angiography showed multiple aneurysms in the branches of renal arteries, superior mesenteric artery, and hepatic arterial system including left renal infarct, suggesting PAN. He was put on high-dose steroids and oral cyclophosphamide. Despite medical treatment, he developed intense abdominal pain, hypotension, tachycardia, and a rapid fall in hemoglobin on four occasions. Active bleeding sites were embolized in two different angiography sessions. Although the patient experienced no more recurrent bleeding, he died of multiorgan dysfunction syndrome resulting from sepsis 6 weeks after admission. Polyarteritis nodosa associated with FMF may follow a grave course despite immunosuppressive therapy. Arterial embolization should be considered in the presence of bleeding aneurysms in addition to immunosuppressive therapy.     

Cutaneous sarcoidosis in a patient with ulcerative colitis on infliximab

The advance of anti-tumour necrosis factor (TNF) therapy had dramatically changed the treatment algorithm of inflammatory bowel disease (IBD). This had significantly improved the quality of life for patients with Crohn's disease (CD) and ulcerative colitis (UC).¹ However, side-effects of anti-TNF treatment were unavoidable with paradoxical inflammation (for example leucocytoclastic vasculitis and psoriasis) being well-known phenomena of anti-TNF therapy.² We report a case of infliximab induced cutaneous sarcoidosis in a patient with ulcerative colitis and review the literature.     

Ulcerative colitis and sensorineural hearing loss: is there a relationship?

A number of systemic complications are recognized in patients with ulcerative colitis. We describe a patient with this disease who developed severe bilateral sensorineural hearing loss and vestibular dysfunction. Her hearing deficit improved dramatically with corticosteroids and cyclophosphamide therapy. The association between these two conditions has not previously been reported.     

Do technetium-99m hexamethylpropylene amine oxime-labeled leukocytes truly reflect the mucosal inflammation in patients with ulcerative colitis?

Twenty-five patients with ulcerative colitis and nine controls with macroscopically non-inflamed colon were investigated with technetium-99m hexamethylpropylene amine oxime-labeled leukocyte scintigraphy and colonoscopy with biopsies. The interval between leukocyte scintigraphy and colonoscopy was < or = 14 days in all patients with ulcerative colitis and < or = 30 days in eight of nine controls. Scintigrams were obtained at approximately 45 min and 4 h after injection of labeled leukocytes. One nuclear physician, one internist, and one pathologist graded blindly and independently of each other the degree of active inflammation in seven different colonic segments for each patient, using 4-grade scales for scans and macroscopically and histologically viewed inflammation, respectively. A positive correlation between endoscopic and histologic grading of all colonic segments and scan gradings for all subjects and for ulcerative colitis patients separately was found (all, p < 0.001). By means of kappa statistics, the inter-observer agreement between scintigraphic grading at 45 min and endoscopy was, for all subjects, 0.32 (95% confidence interval (CI), 0.20-0.44; p < 0.001) and, for patients with ulcerative colitis, 0.19 (CI, 0.07-0.31; p < 0.001). When 17 patients who had complete colonoscopies were divided into those with total, extensive, or distal colitis, leukocyte scintigraphy underestimated the extension of active inflammation. A simple scintigraphic scoring system reflects the colonic inflammation viewed endoscopically and histologically in patients with ulcerative colitis but underestimates the presence of active inflammation in individual colonic segments.     

类固醇激素依赖型溃疡性结肠炎缓解期IL-23表达的研究

目的 探讨IL-23在缓解期类固醇激素依赖型溃疡性结肠炎患者结肠组织中表达的病理意义.方法 采用Western blot分析及免疫组化SABC法检测15例缓解期激素依赖型溃疡性结肠炎患者炎症修复区结肠组织IL-23的蛋白表达情况,予统计学软件统计分析,并以30例缓解期的一般溃疡性结肠炎患者(15例SASP维持治疗,15例强的松维持治疗)炎症修复区结肠组织及10例正常结肠黏膜组织为对照组.结果 与正常对照组比较,SASP维持治疗及强的松维持治疗缓解期一般溃疡性结肠炎患者炎症修复区结肠组织IL-23的蛋白表达均轻度升高(P>0.05),而缓解期激素依赖型溃疡性结肠炎患者炎症修复区结肠组织IL-23的蛋白表达显著高于一般溃疡性结肠炎组(P<0.01).结论 IL-23的过度表达可能在溃疡性结肠炎类固醇激素依赖发病机制中起关键作用.     

类固醇激素依赖型溃疡性结肠炎缓解期IL-23表达的研究

目的 探讨IL-23在缓解期类固醇激素依赖型溃疡性结肠炎患者结肠组织中表达的病理意义.方法 采用Western blot分析及免疫组化SABC法检测15例缓解期激素依赖型溃疡性结肠炎患者炎症修复区结肠组织IL-23的蛋白表达情况,予统计学软件统计分析,并以30例缓解期的一般溃疡性结肠炎患者(15例SASP维持治疗,15例强的松维持治疗)炎症修复区结肠组织及10例正常结肠黏膜组织为对照组.结果 与正常对照组比较,SASP维持治疗及强的松维持治疗缓解期一般溃疡性结肠炎患者炎症修复区结肠组织IL-23的蛋白表达均轻度升高(P>0.05),而缓解期激素依赖型溃疡性结肠炎患者炎症修复区结肠组织IL-23的蛋白表达显著高于一般溃疡性结肠炎组(P<0.01).结论 IL-23的过度表达可能在溃疡性结肠炎类固醇激素依赖发病机制中起关键作用.     

类固醇激素依赖型溃疡性结肠炎缓解期IL-23表达的研究

目的 探讨IL-23在缓解期类固醇激素依赖型溃疡性结肠炎患者结肠组织中表达的病理意义.方法 采用Western blot分析及免疫组化SABC法检测15例缓解期激素依赖型溃疡性结肠炎患者炎症修复区结肠组织IL-23的蛋白表达情况,予统计学软件统计分析,并以30例缓解期的一般溃疡性结肠炎患者（15例SASP维持治疗,15例强的松维持治疗）炎症修复区结肠组织及10例正常结肠黏膜组织为对照组.结果 与正常对照组比较,SASP维持治疗及强的松维持治疗缓解期一般溃疡性结肠炎患者炎症修复区结肠组织IL-23的蛋白表达均轻度升高（P〉0.05）,而缓解期激素依赖型溃疡性结肠炎患者炎症修复区结肠组织IL-23的蛋白表达显著高于一般溃疡性结肠炎组（P〈0.01）.结论 IL-23的过度表达可能在溃疡性结肠炎类固醇激素依赖发病机制中起关键作用.     

Clinical experiences of five cases with ulcerative colitis and recto- or anovaginal fistula]

Clinical aspects, treatment and outcome of five patients with ulcerative colitis recto- or anovaginal fistula and were studied retrospectively. All patients had total colitis (relapse and remission type) and more than a 5 year history of ulcerative colitis. They all had anorectal complications, such as periproctal abscess, stenosis of fistula. Four patients had total colectomy with an ileal pouch anal canal anastomosis for intractability or dysplasia. One was treated conservatively. Complete closure of fistula was obtained in two patients;in one patient rectum was resected below the fistula and in one patient defect of the vaginal posterior wall was reconstructed by using a gluteal fold flap following colectomy. Recto- or anovaginal fistula complicating ulcerative colitis is rare but may occur in the patients with severe rectal inflammation and they can be managed by restorative proctocolectomy with an ileal pouch anal or anal canal anastomosis.     

Do Technetium-99m Hexamethylpropylene Amine Oxime-Labeled Leukocytes Truly Reflect the Mucosal Inflammation in Patients with Ulcerative Colitis?

Twenty-five patients with ulcerative colitis and nine controls with macroscopically non-inflamed colon were investigated with technetium-99m hexamethylpropylene amine oxime-labeled leukocyte scintigraphy and colonoscopy with biopsies. The interval between leukocyte scintigraphy and colonoscopy was 14 days in all patients with ulcerative colitis and 30 days in eight of nine controls. Scintigrams were obtained at approximately 45 min and 4 h after injection of labeled leukocytes. One nuclear physician, one internist, and one pathologist graded blindly and independently of each other the degree of active inflammation in seven different colonic segments for each patient, using 4-grade scales for scans and macroscopically and histologically viewed inflammation, respectively. A positive correlation between endoscopic and histologic grading of all colonic segments and scan gradings for all subjects and for ulcerative colitis patients separately was found (all, p < 0.001). By means of kappa statistics, the inter-observer agreement between scintigraphic grading at 45 min and endoscopy was, for all subjects, 0.32 (95% confidence interval (CI), 0.20–0.44; p < 0.001) and, for patients with ulcerative colitis, 0.19 (CI, 0.07–0.31; p < 0.001). When 17 patients who had complete colonoscopies were divided into those with total, extensive, or distal colitis, leukocyte scintigraphy underestimated the extension of active inflammation. A simple scintigraphic scoring system reflects the colonic inflammation viewed endo-scopically and histologically in patients with ulcerative colitis but underestimates the presence of active inflammation in individual colonic segments.     

New keys to maintenance treatment in ulcerative colitis

Maintenance treatment in ulcerative colitis often fails to prevent flares and long term complications. The first key to maintenance is to use effective therapy, even when patients become asymptomatic. The second key is to communicate the importance of adherence to patients, and to help them achieve long term adherence. Simplified dosing schedules are of some benefit, but the bond between patient and doctor, and the patient's belief in the efficacy of the therapy are essential. Decreased co-pays (a fixed amount paid by patients seeking care that is not reimbursed my medical insurance) have been associated with increased adherence, and incentives for patients may be a cost-effective approach to improving adherence. While the most substantial data on the association between adherence and clinical outcomes is in 5-ASAs, non-adherence can also limit the efficacy of thiopurines and biologics. The third key to maintenance treatment is monitoring and maintaining control of inflammation. Decreased histologic and endoscopic damage to the colon has been associated with decreased risk of colon cancer. The most cost-effective way to monitor smoldering inflammation is not known, but endoscopy, structured symptom indices, and biomarkers may be valuable approaches. The fourth key to maintenance treatment is optimizing immunomodulator therapy with thiopurines, and possibly methotrexate in the future. The fifth key to maintenance treatment in ulcerative colitis is maintaining biologic efficacy by avoiding low trough levels and being vigilant for subclinical inflammation and symptom recurrence at the end of dose intervals. Combination therapy with immunomodulators improves trough levels in Crohn's, and may prove to have benefits for the maintenance of biologic efficacy in ulcerative colitis.     

Atypital localisation of pyoderma gangraenosum in patient with ulcerative colitis

The authors present a case of 61-year-old man with ulcerative colitis and with extraintestinal manifestation of the disease in the form of pyoderma gangraenosum. Multiple skin defects, which developed in atypical localisation (extensive affection of facial and hairy parts of the head) in patient with chronically active form of ulcerative colitis were complicated with bacterial contamination of methicilin-resistant strains of Staphylococcus aureus. After application of the parenteral feeding, corticotherapy and targeted antibiotic therapy the subjective and objective status of the patient markedly improved, stool frequency was reduced, admixture of blood in the stool disappeared, temperatures fell back and there was a decrease in activity of non-specific bowel inflammation in laboratory findings. However endoscopic examination of the intestine confirmed the finding of chronically active ulcerative colitis with ulcerations and bridging polyps. Patient was indicated to total colectomy, but he refused it.     

Paradoxical Response to Heparin in 10 Patients with Ulcerative Colitis

Objectives : A patient with ulcerative colitis refractory to standard therapy was treated with heparin for a deep vein thromhosis. Paradoxically, rectal bleeding did not increase; instead, his colitis rapidly went into remission. The same effect occurred when this patient was later treated for a pulmonary embolism. On the basis of these observations and reports of a hypercoagulable state in ulcerative colitis, heparin was tested as a therapeutic agent in nine additional patients. Methods : Nine of the 10 patients had ulcerative colitis poorly controlled on sulfasalazine and prednisolone. Two had associated thromboembolic disease, and one was on no medication. Patients were started on heparin in hospital, taught to self-inject subcutaneously, and discharged to continue on 10.000 U of unfractionated heparin twice daily. Current doses of sulfasalazine were maintained; prednisolone was tapered and stopped. Patients were carefully monitored for adverse side-effects. Sections of colonic mucosa from nine patients were examined for intravascular thrombosis of the mucosal blood vessels. Results : Nine patients became asymptomatic (normal stool frequency, no rectal bleeding) on combined heparin and sulfasulazine therapy; one patient had a partial improvement in symptoms. Highly significant statistical differences between pre- and posttreatment mean scores were found for all disease parameters. Intravascular fibrin thrombi were identified in sections from six of nine patients. No serious complications were associated with this use of heparin. Conclusions : The heparin-linked remission of ulcerative colitis, observed by chance in our first patient, was followed by simitar responses in eight of nine further patients. This suggests that, used as described, heparin may have a role in treating refractory ulcerative colitis.     

Fatal toxic megacolon due to cytomegalovirosis in a patient with ulcerative colitis: case report and review

BACKGROUND: The toxic megacolon is a rare and severe complication of ulcerative colitis. In general it complicates patients with active colitis that are resistant to clinical treatment and, in some cases, the developing factor is unknown. Cytomegalovirus infection in humans in general is a subclinical condition. However, in patients with immunodeficiency the primary infection or the reactivation of latent infection could have enormous clinical effects. One of these effects is the toxic megacolon. AIM: To report a case of fatal toxic megac lon due to cytomegalovirosis in a patient with ulcerative colitis. PATIENT: A male patient, 38 years old, with complaints of diarrhea and weight loss. The diagnosis of ulcerative colitis was made and a vigorous immunossupressive therapy was performed. RESULTS: Due to the evolution to a toxic megacolon the patient was submitted to colectomy. In the post-operatory period there were severe clinical complications and the patient died. The pathological study of the colon revealed active ulcerative colitis, associated with confluent ulcerations and numerous cells with cytomegalic nuclear inclusions. CONCLUSION: The cytomegalovirosis must be considered as one of the causal agent of toxic megacolon in ulcerative colitis.     

Immunoglobulin G (IgG), IgG1, and IgG2 determinations from endoscopic biopsy specimens in control, Crohn's disease, and ulcerative colitis subjects.

Acute exacerbations of chronic inflammatory bowel disease (ulcerative colitis and Crohn's disease) are characterised by an increase in immunoglobulin G (IgG) positive cells in the mucosa, whereas uninflamed mucosa of inflammatory bowel disease patients displays only moderately increased or normal numbers of these cells. Previous data suggest that acute exacerbations of ulcerative colitis and Crohn's disease can be distinguished by different IgG subclass expression of mucosal immunocytes and a different IgG subclass production pattern of lamina propria lymphocytes. A procedure to obtain enough intestinal mononuclear cells from biopsy specimens to measure in vitro IgG and IgG1 production in control subjects and various patient groups has been established. IgG2 could be measured in Crohn's disease and ulcerative colitis only, as the concentrations in control subjects were below the sensitivity of the ELISA method. We found that IgG and IgG1 production correlated with the degree of local inflammation in both diseases, even in slightly inflamed mucosa, compared with control subjects. The proportion of IgG1 subclass was significantly increased in severely inflamed mucosa of both ulcerative colitis and Crohn's disease patients. A major difference between Crohn's disease and ulcerative colitis mucosa is apparent in mild or no inflammation. In Crohn's disease mucosa in remission, the IgG1/IgG ratio is comparable with that in controls, yet ulcerative colitis mucosa still displays significantly increased proportions of IgG1. In addition, the IgG2/IgG ratio is 0.12 in ulcerative colitis and 0.19 in Crohn's disease patients. The results show the dependence of local IgG and IgG1 production on the degree of inflammation and that an increase in subclass IgG1 in ulcerative colitis is present at all stages, including remission. These findings support the hypothesis that different immunoregulatory mechanisms are involved in Crohn's disease and ulcerative colitis. Environmental stimuli or genetic background may be responsible for the observed differences.