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Background: Intestinal Behçet's syndrome is a major cause of morbidity and mortality in Behçet's syndrome. Objectives: Current treatment challenges remain in refractory intestinal Behçet's syndrome, when patients failed first and second-line therapies. Design: We reported the efficacy and safety profiles of tofacitinib in patients with moderate-severe intestinal Behçet's syndrome in a retrospective single-center study. Methods: Treatment with glucocorticoids, immunosuppressors, or even anti-TNFα monoclonal antibodies (mAbs) had previously failed. Primary outcomes were clinical remission or low disease activity and endoscopic healing. Results: We included 13 patients; 11 were administered tofacitinib 5 mg twice daily, and 2 took tofacitinib 5 mg once daily. Nine patients achieved clinical remission after a mean treatment duration of 10.1 ± 7.0 months, and the other four had low disease activity. Follow-up endoscopy was available in 11 patients: 5 had achieved mucosal healing; the other 4 achieved marked mucosal improvement. Prednisone dosage was significantly reduced, from 30 (interquartile range: 20–30) mg/d to 2.5 (interquartile range: 0–12.5) mg/ d (p < 0.001). No serious adverse event was observed. Conclusion: Tofacitinib could be an efficacious and generally well-tolerated option in patients with intestinal Behçet's syndrome refractory to conventional agents, even anti-TNFα mAbs. [ABSTRACT FROM AUTHOR]

Gastrointestinal involvement in Takayasu Arteritis (TA) requires invasive or expensive diagnostic studies. Instead a fecal biomarker can be used as an initial screening test. In this context, S100A12 is promising as an established biomarker in intestinal inflammation and its role in TA pathogenesis. As such we aimed to test the feasibility of fecal S100A12 as a means of the fecal biomarker in gastrointestinal involvement in TA in this pilot study. Our study population consisted of 30 TA patients and 14 control patients with non-inflammatory arthralgia. Patients with inflammatory or infectious gastrointestinal tract diseases, or used oral antibiotics or NSAIDs for the 3 weeks were excluded. Vasculitis involvements were determined with cross-sectional radiologic studies. TA disease activity was evaluated per Indian Takayasu's Activity Score (2010) criteria and vascular involvements were classified according to Numano classification. ELISA test was used to determine fecal S100A12 levels. Fecal S100A12 levels were higher in TA patients when compared to the controls (37.9 ng/ml vs. 12.5 ng/ml p = 0.038). ESR and CRP levels were also higher in the TA group, however not correlated with fecal S100A12. Among TA patients, fecal S100A12 levels were higher inactive ones with ITAS2010 > 1 (72.9 ng/ml vs. 16.7 ng/ml p = 0.016) correlated with total ITAS2010 scores. (R = 0.52 p = 0.003). TA patients with abdominal symptoms had higher fecal S100A12 levels when compared to the remaining TA population (327.8 ng/ml vs. 28.0 ng/ml p = 0.003). However, fecal S100A12 levels in patients with or without mesenteric vessel involvement did not differ. Fecal S100A12 shows promise as a fecal biomarker to screen intestinal ischemia and inflammatory bowel disease in TA patients. [ABSTRACT FROM AUTHOR]

• Burkitt's lymphoma, an aggressive non-Hodgkin lymphoma, is extremely rare during pregnancy. • Lymphoma of the small intestine is often overlooked in the early stages of the disease. • We described a multidisciplinary approach, cesarean section with surgical intestinal exploration. Burkitt's lymphoma (BL), an aggressive subtype of non-Hodgkin lymphoma (NHL), is extremely rare during pregnancy. In the case of bowel localization, diagnosis can be very difficult. Moreover, signs and symptoms of the primary small intestine lymphoma are nonspecific, mostly attributable to the "mass effect" of the tumor. The most frequent symptom is abdominal cramp-like pain, associated with nausea and vomiting. We report a rare case of a 37-year-old pregnant woman, at the 33rd week of gestation, with an abdominal-pelvic mass of uncertain nature. Surgical strategy consisted of a two-step procedure, which involved a cesarean section and typing of the mass: extemporaneous examination hypothesized intestinal lymphoma. The definitive histological examination confirmed the diagnosis of rare case of BL in pregnancy. The clinical case reported, representing a rare occurrence of BL in pregnancy, was associated with difficult interpretation and complex management. Lymphoma of the small intestine is often overlooked in the early stages of the disease, due to the fact that symptoms are non-specific and consequently underestimated. In our case, based on gestational age, it was possible to perform a multidisciplinary approach, a cesarean section with surgical intestinal exploration, achieving at the same time delivery of the child and a definitive diagnosis of BL with intestinal involvement. The involvement of multiple professionals is undoubtedly the best way to deal with the above referred to situation, with the main point being to keep in mind the possibility of this type of occurrence. [ABSTRACT FROM AUTHOR]

We present the case of a woman with a severe clinical history of antiphospholipid syndrome and persistent positivity for lupus anticoagulant, IgG anticardiolipin and IgG anti-β2Glycoprotein I antibodies. An acute clinical onset characterized by severe abdominal pain immediately followed by circulatory shock and histological colonic small vessel thrombosis pattern pointed to a diagnosis of ischemic colitis. The subsequent rapid onset of pulmonary alveolitis and heart failure associated to subendocardial hypoperfusion led to a diagnosis of definite catastrophic antiphospholipid syndrome (CAPS). Conventional triple therapy together with a broad-spectrum preventive antibiotic therapy were quickly initiated, and the outcome was favorable. We evaluated the patients with ischemic colitis in CAPS described in the literature between 1992 and May 2019 and our CAPS case. In accordance with the "two-hit" hypothesis and on the basis of the patients' data, we would like to speculate that the colonic wall necrosis related to ischemic colitis damaged the intestinal barrier causing loss of resistance to bacteria and leading to endotoxemia and bacteremia with bacteria translocation through the circulatory stream to the lungs and heart. The bacteria acted as the priming factor which favored the binding of β2Glycoprotein I to the endothelium vessels in the colon, lungs, and heart following activation of anti-β2Glycoprotein I antibodies which attached to the domain I of β2Glycoprotein I. This was followed by complement activation which triggered the thrombotic and cytokine storm. If further clinical studies confirm this hypothesis, the treatment of CAPS could be more targeted and effective. [ABSTRACT FROM AUTHOR]

Abstract Background Prokinetics are used to treat enteric dismotility symptoms in systemic sclerosis (SSc) patients, but they often lack adequate efficacy. The most effective prokinetics belonging to the serotonin (5-HT4) receptor agonists class were withdrawn due to cardiac toxicity in relation to modest 5-HT4 receptor affinity. Prucalopride is a high-affinity 5-HT4 receptor agonist with no major cardiac issues, for which the efficacy in SSc has not yet been assessed. Methods Forty patients with self-reported mild to moderately severe enteric symptoms were enrolled in a cross-over 2 × 2 study. Subjects were randomized 1:1 to prucalopride 2 mg/day or no treatment for one month and vice versa after a 2-week washout period. Before and after each sequence the patients compiled the University of California Los Angeles gastrointestinal tract (UCLA GIT) 2.0 questionnaire and the numbers of complete intestinal movements were recorded. Oro-cecal transit time (OCTT) was evaluated by lactulose breath test in a subgroup of patients. Data were evaluated by mixed linear models corrected for the number of laxatives used during the study periods. Results There were 29 subjects who completed the study; 7 subjects withdrew due to side-effects and 4 subjects were not compliant with the study procedures. As compared to dummy treatment, prucalopride was associated with more intestinal evacuations (p

Abstract: Purpose: The aim of this study was to test the predictive value of interleukin (IL) 8 in the assessment of intestinal involvement in necrotizing enterocolitis (NEC). Methods: Forty infants with surgically treated NEC were classified into 3 groups based on intestinal involvement during laparotomy: focal (n = 11), multifocal (n = 16), and panintestinal (n = 13). Preoperatively obtained serum levels of IL-8, C-reactive protein, white blood cell count, and platelet count were correlated with intestinal involvement using logistic regression models. Results: Interleukin 8 correlated significantly with intestinal involvement in infants with surgically treated NEC (odds ratio, 1.74; confidence interval, 1.27-2.39; P < .001). An exploratory IL-8 cutoff value of 449 pg/mL provided a specificity of 81.8% and sensitivity of 82.8% to discriminate focal from multifocal and panintestinal disease. An IL-8 cutoff value of 1388 pg/mL provided a specificity of 77.8% and a sensitivity of 76.9% to discriminate panintestinal disease from focal and multifocal disease. Conclusions: To our knowledge, this is the first study to demonstrate a significant correlation of IL-8 with intestinal involvement in advanced NEC in a large patient population. Our results indicate that IL-8 may be a promising biomarker for assessing intestinal involvement in infants with advanced NEC. [Copyright &y& Elsevier]

The aim of this study was to determine the clinical characteristics of Behcet’s disease (BD) complicated with malignancy in Korea. Of 1,769 patients with BD in our hospital, 32 patients (1.8%, 21 in solid cancer, 11 in hematologic malignancy) developed cancer. In 10 of the 32 subjects (31.3%), malignancy was diagnosed before or concomitantly with BD. Twelve cases (37.5%) occurred within the first 2 years of disease and 9 cases (28.1%) occurred 5 years after the diagnosis of BD. Myelodysplastic syndrome (MDS) was the most common disease ( n = 7) followed by thyroid cancer ( n = 4), breast cancer, cervix cancer, stomach cancer, rectal cancer, hepatoma, aplastic anemia ( n = 3, each), renal cell cancer, endometrial cancer, lymphoma ( n = 1, each). There were no significant differences in the clinical characteristic between patients with or without malignancy. Intestinal involvement were more frequent in patients with malignancy than those without, but was not statistically significant ( p = 0.083). Our results demonstrate that MDS and thyroid cancer are the most common hematologic disease and solid cancer associated with BD, respectively. Further studies will be required to ascertain the pathogenic link between BD and malignancy and the prevalence of malignancy in BD. [ABSTRACT FROM AUTHOR]

Background: The aim of this study was threefold; to define the enteroclysis (EC) findings of intestinal involvement in Behcet disease (BD), to compare these findings with those seen in Crohn disease (CD), and to determine the relation between the duration of BD and severity of the EC findings. Methods: From 1997 to 2000, 17 BD and 50 CD cases were examined by EC examination. EC was performed with a 13-F balloon catheter via transnasal entubation. Mucosal and mural changes were evaluated. Statistical analysis was performed with the Mann-Whitney U test to determine the relation between duration of BD and severity of the EC findings. P ≤ 0.05 was considered statistically significant. Results: In 12 (70.58%) of 17 BD cases, EC demonstrated intestinal pathologic findings such as ulcerations, fold thickening, granular pattern, pseudopolyp formation, and bowel wall thickening. No statistical correlation between the severity of EC findings and the duration of BD was found. Conclusion: EC should be the radiologic method to evaluate the intestinal pathology in symptomatic BD patients. The main EC finding in BD was ulceration mostly in the aphthous form. The EC findings in BD, even in longstanding cases, are usually mild when compared with those seen in CD. Correspondence to: U. Korman --> [ABSTRACT FROM AUTHOR]

There is a need for intravenous therapy for severe forms of tuberculosis in those who are HIV-positive, diabetic, have cystic fibrosis, extensive gastrointestinal involvement, haemodynamic instability with difficulty to take and absorb oral medications, which results in a lack of appropriate therapeutic effect during treatment, such as not creating the optimal blood concentration of the substance. We report an HIV-infected patient presenting with severe disseminated tuberculosis with extensive intestinal involvement which was successfully treated with an initial combination regimen of injectable and oral anti-tuberculosis agents. We discuss and emphasize the importance of an initial regimen with injectable and oral agents for treating severe forms of tuberculosis. [ABSTRACT FROM AUTHOR]

Abstract: Gastrointestinal involvement is a rare event in patients with B-cell chronic lymphocytic leukemia (B-CLL) and is usually associated to lymphomatous transformation. However, in autopsy studies the reported incidence of microscopic infiltration can reach up to 50% of cases. Seven B-CLL patients in advanced stage/progressive disease were evaluated by colonoscopy because of continuous diarrhea. Five out of seven patients (71%) presented histological evidence of colonic infiltration. Persistent diarrhea in patients with progressive/advanced B-CLL can be a clinical sign of intestinal infiltration and justifies endoscopic examinations. [Copyright &y& Elsevier]

Malignant atrophic papulosis (Degos' disease) is a very rare condition characterized by atrophic papular skin lesions and variable association of systemic involvement. We describe a 33-year-old man who presented with a widespread skin eruption consistent with malignant atrophic papulosis. During the course of the disease he even developed penile ulcerations, a symptom that has been reported only a few times previously. He subsequently died of multiple perforations of the small bowel 2.5 years after onset of the disease. Laboratory investigations revealed a mutation of factor V Leiden and the presence of lupus anticoagulant, but no anti-cardiolipin antibodies. The patient was treated with narrow-band ultraviolet (UV)B, prednisolone and, later, aspirin, pentoxifyllin and warfarin. Despite this very intensive anticoagulant and anti-platelet therapy, the treatment had no effect on the skin lesions and could not prevent systemic involvement. [ABSTRACT FROM AUTHOR]

Toxic epidermal necrolysis (TEN) is a severe adverse drug reaction, which is characterized by erythema, blisters, and/or erosions of the mucous membranes and skin, but intestinal involvement is rare. In contrast, pneumatosis cystoides intestinalis (PCI) is a rare condition associated with a wide variety of underlying diseases, but to date no patient has presented with PCI associated with TEN. A 55-year-old man was admitted to intensive care unit for treatment of TEN caused by phenobarbital. On day 8 after admission, he presented with progressive abdominal distention and hypotension. Computed tomography (CT) showed gas in the superior mesenteric vein and air filled cysts in the walls of the small intestine. He was suspected of having septic shock due to PCI. As there were no indications of bowel ischemia or necrosis, the patient was managed conservatively with antibiotics and oxygen therapy. On day 10 after admission, he was weaned off catecholamines, with CT on day 11 showing complete resolution of gas in the superior mesenteric vein and air filled cysts. To our knowledge, this article describes the first patient presenting with PCI associated with TEN.

Copy number variation has been associated with various autoimmune diseases. We investigated the copy number (CN) of the DEFA1 gene encoding α-defensin-1 in samples from Korean individuals with Behcet's disease (BD) compared to healthy controls (HC). We recruited 55 BD patients and 35 HC. A duplex Taqman® real-time PCR assay was used to assess CN. Most samples (31.1%) had a CN of 5 with a mean CN of 5.4 ± 0.2. There was no significant difference in the CN of the DEFA1 gene between BD patients and HC. A high DEFA1 gene CN was significantly associated with intestinal involvement in BD patients. Variable DEFA1 gene CNs were observed in both BD patients and HC and a high DEFA1 gene CN may be associated with susceptibility to intestinal involvement in BD.

High-mobility group box 1 (HMGB1) protein has been demonstrated to play an important role in chronic inflammatory diseases including rheumatoid arthritis, and systemic lupus erythematosus. This study investigated the association between extracellular HMGB1 expression and disease activity, and clinical features of Behçet's disease (BD). Extracellular HMGB1 expression in the sera of 42 BD patients was measured and was compared to that of 22 age- and sex-matched healthy controls. HMGB1 expression was significantly increased in BD patients compared to healthy controls (78.70 ± 20.22 vs 10.79 ± 1.90 ng/mL, P = 0.002). In addition, HMGB1 expression was significantly elevated in BD patients with intestinal involvement compared to those without (179.61 ± 67.95 vs 61.89 ± 19.81 ng/mL, P = 0.04). No significant association was observed between HMGB1 concentration and other clinical manifestations, or disease activity. It is suggested that extracellular HMGB1 may play an important role in the pathogenesis of BD.