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Scleroderma bowel

Naoko Okiyama, Yoshihide Asano, Yasuhito Hamaguchi, Masatoshi Jinnin, Sei-Ichiro Motegi, Haruka Koizumi, Minoru Hasegawa, Osamu Ishikawa, Shinichi Sato, Kazuhiko Takehara, Toshiyuki Yamamoto, Manabu Fujimoto, Hironobu Ihn
Localized scleroderma (LoS) involves dermal but not internal inflammation and fibrosis. Cosmetic changes often impact quality of life (QOL), however, impairment of activities of daily living (ADL) in LoS patients has not been investigated. To determine what factor(s) are associated with ADL in adult patients with LoS, we performed a retrospective observational study in 177 Japanese adult LoS patients using a novel LoS disability score based on Barthel's indices of ADL: feeding, bathing, grooming, dressing, bowels, bladder, toilet use, transfers, mobility and stairs...
January 25, 2018: Journal of Dermatology
Evan Calabrese, Peter Jm Ceponis, Bruce J Derrick, Richard E Moon
Pneumatosis intestinalis (PI), or the presence of air in the bowel wall, is a rare disorder that is associated with a variety of underlying diseases, including connective tissue disorders. PI presents on a spectrum from asymptomatic to bowel obstruction and acute abdomen. In general, treatment of PI consists of treating the underlying disease. Both normobaric and hyperbaric oxygen have been used to treat PI directly. Here we report a symptomatic scleroderma-related case of PI that responded clinically to hyperbaric oxygen therapy...
May 30, 2017: BMJ Case Reports
Mary-Louise C Greer
Magnetic resonance enterography (MRE) now plays a central role in diagnosing pediatric inflammatory bowel disease (IBD), and its role in other intestinal pathologies such as scleroderma is gradually expanding. MRE helps distinguish between Crohn disease and ulcerative colitis, defining extent and severity. Standard MRE protocols can be optimized in children and adolescents to be diagnostic and well tolerated, both of which are important with increasing use of serial MRE in pediatric IBD for monitoring treatment response and evaluating complications...
May 2016: Pediatric Radiology
Kuang-Hui Yu, Chang-Fu Kuo, Lu Hsiang Huang, Wen-Kuan Huang, Lai-Chu See
The aim of this study was to determine whether inflammation is related to cancer development, and whether the incidence of cancer is increased and occurs in a site-specific manner in patients with systemic autoimmune rheumatic diseases (SARDs).This study included a nationwide dynamic cohort of patients with various newly diagnosed SARDs from 1997 to 2010 with follow-up until 2012.This study included 75,123 patients with SARDs. During 562,870 person-years of follow-up, 2844 patients developed cancer. Between 1997 and 2010, the highest number of newly diagnosed SARDs cases were rheumatoid arthritis (n = 35,182), followed by systemic lupus erythematosus (SLE, n = 15,623), Sjögren syndrome (n = 11,998), Kawasaki disease (n = 3469), inflammatory bowel disease (n = 2853), scleroderma (n = 1814), Behçet disease (n = 1620), dermatomyositis (n = 1119), polymyositis (n = 811), and vasculitis other than Kawasaki disease (n = 644)...
May 2016: Medicine (Baltimore)
Martyn Dibb, Mattias Soop, Antje Teubner, Jon Shaffer, Arun Abraham, Gordon Carlson, Simon Lal
BACKGROUND: Home parenteral nutrition (HPN) is the mainstay of treatment for patients with Type 3 intestinal failure (IF), however long term data on mortality and nutritional outcomes are limited. OBJECTIVES: To assess the long-term survival and requirements for ongoing HPN in patients receiving treatment at a UK national referral centre for intestinal failure. METHODS: Patients with IF who received HPN for more than 3 months at this Intestinal Failure Unit between 1978 and 2011 had their clinical records reviewed...
April 2017: Clinical Nutrition: Official Journal of the European Society of Parenteral and Enteral Nutrition
A Menys, S Butt, A Emmanuel, A A Plumb, A Fikree, C Knowles, D Atkinson, N Zarate, S Halligan, S A Taylor
BACKGROUND: Chronic intestinal pseudo-obstruction (CIPO) is characterized by dilatation of the bowel lumen and abnormal motility. In this study, we aimed to quantify small bowel dysmotility in CIPO using a validated pan-intestinal motility assessment technique based on motion capture magnetic resonance imaging (MRI) compared to normal controls. In addition, we explored if motility responses of CIPO patients to neostigmine challenge differed from healthy volunteers. METHODS: Twenty healthy volunteers (mean age 28, range 22-48) and 11 CIPO patients (mean age 47, range 19-90) underwent MRI enterography to capture global small bowel motility...
March 2016: Neurogastroenterology and Motility: the Official Journal of the European Gastrointestinal Motility Society
Christopher P Denton
Systemic sclerosis (scleroderma) leads to morbidity and mortality through a combination of inflammation, fibrosis and vascular damage leading to internal organ complications affecting the heart, lung, kidneys and bowel. More than half of those diagnosed ultimately die from the disease. Current treatments focus on broad spectrum immunosuppression or organ-based therapy for complication such as lung fibrosis, pulmonary or systemic hypertension. Targeting peptide mediators such as endothelin-1 have already led to licensed effective therapies for SSc vasculopathy...
July 2015: Clinical and Experimental Rheumatology
Kristina N Katz, Kevin Skole, Jean M Fede
Scleroderma is an autoimmune disease that can affect multiple organ systems, including the gastrointestinal tract. Intussusception, the telescoping of the bowel that can lead to intestinal obstruction, is an uncommon phenomenon in adults. We report the first case of sigmoid intussusception in a patient with scleroderma.
October 2014: ACG Case Reports Journal
Alireza Ghanadan, Amene Saghazadeh, Issa Jahanzad, Nima Rezaei
Because the most common term used in conversations considering autoimmunity is autoantibodies, it is well-expected that the indirect immunofluorescence assay, which detects antibodies directed against various antigens, is one of our most impressive techniques for investigating autoimmune diseases (AIDs). Roughly speaking, the current literature corroborates that this immunopathologic investigation means that autoantibodies detection makes a considerable contribution to both diagnostic and prognostic aspects of AIDs in the clinical setting...
May 2015: Expert Review of Clinical Immunology
K Heelan, A L Mahar, S Walsh, N H Shear
BACKGROUND: Pemphigus is a rare autoimmune blistering disease, reported to be associated with other coexisting and autoimmune diseases, including thyroid diseases, rheumatoid arthritis, alopecia areata, vitiligo, systemic lupus erythematosus, scleroderma and rare entities such as myasthenia gravis. AIM: To identify and describe patients with pemphigus with a diagnosed comorbidity, and to quantify the risk of additional comorbidities. METHODS: This was a cross-sectional study of patients with pemphigus treated at a tertiary referral centre...
August 2015: Clinical and Experimental Dermatology
Fernando Vázquez-Villa, Marcos García-Ocaña, José A Galván, Jorge García-Martínez, Carmen García-Pravia, Primitiva Menéndez-Rodríguez, Carmen González-del Rey, Luis Barneo-Serra, Juan R de Los Toyos
The COL11A1 human gene codes for the α1 chain of procollagen 11A1 and mature collagen 11A1, an extracellular minor fibrillar collagen. Under regular conditions, this gene and its derived products are mainly expressed by chondrocytes and mesenchymal stem cells as well as osteoblasts. Normal epithelial cells and quiescent fibroblasts from diverse locations do not express them. Mesenchyme-derived tumors and related conditions, such as scleroderma and keloids, are positive for COL11A1/(pro)collagen 11A1 expression, as well as high-grade human gliomas/glioblastomas...
April 2015: Tumour Biology: the Journal of the International Society for Oncodevelopmental Biology and Medicine
John Nay, Christine O Menias, Vincent M Mellnick, Dennis M Balfe
Systemic diseases have many different presentations, including imaging findings in the bowel. Recognizing the imaging findings in these diseases is important in making the correct diagnosis. Although certain imaging features overlap, knowledge of specific findings along with the clinical presentation aid in narrowing the differential or in making an imaging diagnosis. The pictorial review will focus on the gastrointestinal manifestations of systemic diseases, including amyloidosis, angiotensin converter enzyme inhibitor-induced angioedema, celiac sprue, distal intestinal obstruction syndrome, graft-versus-host disease, hemolytic uremic syndrome, hemophilia, Henoch-Schönlein purpura, intestinal lymphangiectasia, mastocytosis, scleroderma, systemic lupus erythematosus, Wegener's granulomatosis, and Whipple's disease...
August 2015: Abdominal Imaging
Heather M Mascio, Christie A Joya, Richard A Plasse, Thomas P Baker, Michael F Flessner, Robert Nee
Oxalate nephropathy is an uncommon cause of acute kidney injury. Far rarer is its association with scleroderma, with only one other published case report in the literature. We report a case of a 75-year-old African-American female with a history of systemic scleroderma manifested by chronic pseudo-obstruction and small intestinal bacterial overgrowth (SIBO) treated with rifaximin, who presented with acute kidney injury with normal blood pressure. A renal biopsy demonstrated extensive acute tubular injury with numerous intratubular birefringent crystals, consistent with oxalate nephropathy...
August 2015: Clinical Nephrology
Muhammad Rashad, Mark Valdez, Linh Turong, Aarti Chawla, Ganesh Sivagini
SESSION TITLE: Infectious Disease Cases IISESSION TYPE: Affiliate Case Report SlidePRESENTED ON: Wednesday, October 29, 2014 at 11:00 AM - 12:15 PMINTRODUCTION: Infectious Infectious complications are common after organ trans- plantation because of the profound immunosuppressed status. The incidence of invasive mycoses is 5% to 42%, depending on type of organ transplanted. Here we present a case of invasive and rapidly progressive scedopsirum infection in a lung transplant patient. CASE PRESENTATION: A 53-year-old man underwent bilateral lung transplantation for scleroderma 8 years prior to admission...
October 1, 2014: Chest
Mariangelí Arroyo-Ávila, Luis M Vilá
Mesenteric panniculitis pertains to a group of uncommon disorders named sclerosing mesenteritis that present with different levels of inflammation and fibrosis of the small bowel mesentery. It is associated with abdominal surgeries, trauma, malignancies, infections and connective tissue diseases. To the best of our knowledge, no cases of sclerosing mesenteritis have been reported in patients with systemic sclerosis. We present a case of a 61-year-old woman who had incidental CT findings of mesenteric panniculitis...
October 17, 2014: BMJ Case Reports
R Knobler, G Berlin, P Calzavara-Pinton, H Greinix, P Jaksch, L Laroche, J Ludvigsson, P Quaglino, W Reinisch, J Scarisbrick, T Schwarz, P Wolf, P Arenberger, C Assaf, M Bagot, M Barr, A Bohbot, L Bruckner-Tuderman, B Dreno, A Enk, L French, R Gniadecki, H Gollnick, M Hertl, C Jantschitsch, A Jung, U Just, C-D Klemke, U Lippert, T Luger, E Papadavid, H Pehamberger, A Ranki, R Stadler, W Sterry, I H Wolf, M Worm, J Zic, C C Zouboulis, U Hillen
BACKGROUND: After the first investigational study on the use of extracorporeal photopheresis for the treatment of cutaneous T-cell lymphoma was published in 1983 with its subsequent recognition by the FDA for its refractory forms, the technology has shown significant promise in the treatment of other severe and refractory conditions in a multi-disciplinary setting. Among the major studied conditions are graft versus host disease after allogeneic bone marrow transplantation, systemic sclerosis, solid organ transplant rejection and inflammatory bowel disease...
January 2014: Journal of the European Academy of Dermatology and Venereology: JEADV
Yohei Ichimura, Yoshihide Asano, Kaname Akamata, Takehiro Takahashi, Shinji Noda, Takashi Taniguchi, Tetsuo Toyama, Naohiko Aozasa, Hayakazu Sumida, Yoshihiro Kuwano, Koichi Yanaba, Yayoi Tada, Makoto Sugaya, Shinichi Sato, Takafumi Kadono
CXCL5 is a member of CXC chemokines with neutrophilic chemoattractant and pro-angiogenic properties, which has been implicated in the pathological angiogenesis of rheumatoid arthritis and inflammatory bowel diseases. Since aberrant angiogenesis is also involved in the developmental process of systemic sclerosis (SSc), we herein measured serum CXCL5 levels in 63 SSc and 18 healthy subjects and investigated their clinical significance and the mechanism explaining altered expression of CXCL5 in SSc. Serum CXCL5 levels were significantly lower in SSc patients than in healthy subjects...
May 2014: Archives of Dermatological Research
Thomas Thomaidis, Martin Goetz, Sebastian Paul Gregor, Arthur Hoffman, Elias Kouroumalis, Markus Moehler, Peter Robert Galle, Andreas Schwarting, Ralf Kiesslich
AIM: To assess the esophageal motility in patients with irritable bowel syndrome (IBS) and to compare those with patients with autoimmune disorders. METHODS: 15 patients with IBS, 22 with systemic lupus erythematosus (SLE) and 19 with systemic sclerosis (SSc) were prospectively selected from a total of 115 patients at a single university centre and esophageal motility was analysed using standard manometry (Mui Scientific PIP-4-8SS). All patients underwent esophago-gastro-duodenoscopy before entering the study so that only patients with normal endoscopic findings were included in the current study...
October 14, 2013: World Journal of Gastroenterology: WJG
Puneet Devgun, Hal Hassan
Pneumatosis cystoides intestinalis is a rare gastrointestinal complication in the course of connective tissue diseases, especially in scleroderma, that can lead to pneumoperitoneum or obstruction. Findings on plain radiography may reveal radiolucent linear or bubbly circular air bubbles in the bowel wall, with or without free gas accumulation in the peritoneal cavity. Treatment of pneumatosis cystoides intestinalis ranges from supportive care to laparotomy.
2013: Case Reports in Radiology
Erik Corona, Rong Chen, Martin Sikora, Alexander A Morgan, Chirag J Patel, Aditya Ramesh, Carlos D Bustamante, Atul J Butte
Genetic diversity across different human populations can enhance understanding of the genetic basis of disease. We calculated the genetic risk of 102 diseases in 1,043 unrelated individuals across 51 populations of the Human Genome Diversity Panel. We found that genetic risk for type 2 diabetes and pancreatic cancer decreased as humans migrated toward East Asia. In addition, biliary liver cirrhosis, alopecia areata, bladder cancer, inflammatory bowel disease, membranous nephropathy, systemic lupus erythematosus, systemic sclerosis, ulcerative colitis, and vitiligo have undergone genetic risk differentiation...
May 2013: PLoS Genetics
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