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Prevalence of esophageal abnormalities detected in contrasted image of the esophagus in systemic sclerosis and profile of associated autoantibodies Poster No.: C-0562 Congress: ECR 2014 Type: Scientific Exhibit Authors: A. K. Sakuma , A. Amaral e Castro , T. Larocca Skare , W. Haese 1 2 2 2 2 2 1 2 Barros , R. Lírio Bortoncello , F. Morandini ; Paris/FR, Curitiba/ BR Keywords: Swallowing disorders, Obstruction / Occlusion, Epidemiology, Statistics, Laboratory tests, Barium meal, Conventional radiography, Thorax, Mediastinum, Gastrointestinal tract DOI: 10.1594/ecr2014/C-0562 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. 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Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. www.myESR.org Page 1 of 19 Aims and objectives Introduction Systemic sclerosis (SS) is an idiopathic multisystem disease characterized by an abnomality of small blood vessels, skin and visceral fibrosis. There are specific diagnostic criteria for this condition, as well as classification criteria for activity and severity. The socalled CREST syndrome (calcinosis, Raynaud, esophagus, sclerodactyly, telangiectasia) can lead to esophageal abnomalities with significant morbidity for the affected patient [1,2]. Fig. 1: CREST syndrome References: Department of Rheumathology, Hospital Universitário Evangélico de Curitiba/ Brazil 2013 The esophagus is the most affected region of the gastrointestinal tract in SS. Its pathogenesis is not yet fully understood, although it is recognized atrophy, fibrosis and obliteration of vascularization and innervation of the muscles of the body, resulting in dyskinesia of its body, associated perhaps, with lower esophageal sphincter dysfunction. The SS is the prototype disorder of esophageal dyskinesia. Manometric evidence of organ dysfunction can be found in about 80% of affected patients. The desease characteristically affects the smooth muscle of the distal esophagus, although in some cases the proximal skeletal muscles may be involved [1,3,4]. Page 2 of 19 Page 3 of 19 Fig. 2: Double contrast barium study, showing a significant esophageal dilatation. References: Department of Radiology, Hospital Universitário Evangélico de Curitiba/ Brazil 2013 The decreased peristalsis of the distal esophagus and their possible association with gastroesophageal reflux (GER), delays esophageal clearance, allowing a prolonged exposure to refluxed liquid, possibly resulting in severe esophagitis and other deseases related to GER. The treatment of these specific conditions, without compromising organ function, is important. For example, there is the video laparoscopic [5] anti- reflux surgery. Page 4 of 19 Fig. 3: Double contrast barium study, showing distal esophageal dilatation and signs of esophagitis. References: Department of Radiology, Hospital Universitário Evangélico de Curitiba/ Brazil 2013 It is estimated that 80% or more of patients with SS have some detectable autoantibody. These autoantibodies may provide information regarding the diagnosis of the disease. Page 5 of 19 Moreover, many of them are related to clinical characteristics of each patient , including esophagic changes [6,7,8]. Esophageal manometry is the gold standard test for the diagnosis of esophageal motor disorders, while endoscopy is used to study the changes of the mucosa, related to the GER [1]. As for imaging, today there is a large focus toward the CT scans and MRIs. Despite this, the barium examination remains the primary modality for radiological evaluation of patients with dysphagia, GER and other clinical findings of esophageal diseases. Esophagographies with double contrast are particularly useful for the detection of GER and its complications. Fluoroscopy, part of these routine examinations, is very useful to study the motility of the esophagus and detecting related disorders. Radiographic findings in the SS esophagography may include weak or absent peristalsis in the distal esophagus, delayed esophageal emptying, rigid dilated esophagus that does not colapses while emptying, and GER [9]. Barium x-ray examinations like the esophagographies and esophagogastroduodenal seriographies, are exams with undeniable importance in the study of anatomy and upper gastrointestinal diseases, as well as being a group of tests considered as cheap, quick, simple and with minimal risk to the patient [10]. The primary diagnostic approach of patients with SS with or without esophageal symptoms can be taken with esophagogastroduodenal seriographies. The present study analyses the prevalence of esophageal abnormalities in patients with SS evidenced by esophagogastroduodenal seriography with esophageal symptoms, comparing them with patients without esophageal symptoms. It also compares the clinical and autoantibodies profile of both groups. Images for this section: Page 6 of 19 Fig. 1: CREST syndrome Page 7 of 19 Page 8 of 19 Fig. 2: Double contrast barium study, showing a significant esophageal dilatation. Fig. 3: Double contrast barium study, showing distal esophageal dilatation and signs of esophagitis. Page 9 of 19 Methods and materials 31 patients with sclerodermia, according to the Preliminary qualifying Criteria of the American College of Rheumatology, were included. It was collected data about signs and symptoms of the sclerodermia through medical records review. Moreover, it was applied the International dysphagia specific questionary and taken the seriography, following the institution`s exam protocol. Significance level for statistic analysis was 0,05. Results 31 patients were studied. 90,7% females and 80,6% caucasians, with average age of 50 years. The main clinical type was limited form (64,5%). The main clinical finding was Raynaud`s phenomenon (96%). Table 1: Clinical findings References: Department of Rheumathology, Hospital Universitário Evangélico de Curitiba/ Brazil 2013 NAF was positive in 93% of the patients and the most prevalent autoantibody was the anticentromere (34,6%). Page 10 of 19 Table 2: Autoantibodies profile References: Department of Rheumathology, Hospital Universitário Evangélico de Curitiba/ Brazil 2013 80% of the patients shown at least one alteration on the esophagogastroduodenal seriography, being the esophageal caliber variation, the most usual (54%). Table 3: Alterations on esophagogastroduodenal seriography References: Department of Rheumathology, Hospital Universitário Evangélico de Curitiba/ Brazil 2013 Page 11 of 19 Page 12 of 19 Fig. 4: Alteration on the esophageal caliber References: Department of Radiology, Hospital Universitário Evangélico de Curitiba/ Brazil 2013 No significant difference between both groups was found, as symptoms comparison, dysphagia questionary points, seriography results and laboratory profile are concerned. Images for this section: Table 1: Clinical findings Table 2: Autoantibodies profile Page 13 of 19 Table 3: Alterations on esophagogastroduodenal seriography Page 14 of 19 Page 15 of 19 Fig. 4: Alteration on the esophageal caliber Page 16 of 19 Conclusion No significant differences in clinical or laboratorial profile was found. Neither in the dysphagia questionary points or in the alterations in the seriography exam for both groups. Personal information Alexandre Sakuma, Student. Department of Radiology, Faculty of Medicine, Faculdade Evangélica do Paraná, Curitiba, Brazil; [email protected] Adham do Amaral e Castro, M.D. Department of Radiology, Hospital Universitário Evangélico de Curitiba, Curitiba, Brazil; [email protected] Thelma Larocca Skare, M.D., Ph.D. Department of Rheumatology, Hospital Universitário Evangélico de Curitiba, Curitiba, Brazil; Wagner Haese Barros, M.D. Department of Radiology, Hospital Universitário Evangélico de Curitiba, Curitiba, Brazil; Rafael Lírio Bortoncello, M.D. Department of Radiology, Hospital Universitário Evangélico de Curitiba, Curitiba, Brazil; Fernando Morandini, M.D. Department of Radiology, Hospital Universitário Evangélico de Curitiba, Curitiba, Brazil; Page 17 of 19 References 1. Calderaro DC. Estudo de alterações esofágicas, achados sorológicos, aspectos demográficos e uso de medicamentos em pacientes acometidos por esclerose sistêmica [dissertação de mestrado]. Belo Horizonte: Faculdade de Medicina da Universidade Federal de Minas Gerais; 2009. 2. Freire EAM, Ciconelli RM, Sampaio-Barros PD. Análise dos critérios Diagnósticos, de Classificação, Atividade e Gravidade de Doença na Esclerose Sistêmica. Rev Bras Reumatol 2004; 44(1): 40-45. 3. Roberts CGP, Hummers LK, Ravich WJ, Wigley FM, Hutchins GM. A case-control study of the pathology of oesophageal disease in systemic sclerosis (scleroderma). Gut 2006; 55: 1967-1703. 4. Spechler SJ, Castell DO. Classification of oesophageal motility abnormalities. Gut 2001; 49: 145-151. 5. Lopes LR, Cunha AG, Andreollo NA, Brandalise NA. Tratamento cirúrgico do refluxo gastroesofágico na esclerodermia. Rev Col Bras Cir 2001; 28(2): 133-137. 6. Andrade LEC, Leser PG. Auto-Anticorpos na Esclerose Sistêmica (ES). Rev Bras Reumatol 2004; 44(3): 215-223. 7. Müller CS, Paiva ES, Azevedo VF, Radominski SC, Lima Filho JHC. Perfil de autoanticorpos e correlação clínica em um grupo de pacientes com esclerose sistêmica na região sul do Brasil. Rev Bras Reumatol 2011; 51(4): 314-324. 8. Skare TL, Luciano AC, Fonseca AE, Azevedo PM. Autoanticorpos em esclerodermia e sua associação ao perfil clínico da doença; estudo em 66 pacientes do sul do Brasil. An Bras Dermatol 2011; 86(6): 1075-1081. 9. Levine MS, Rubesin SE. Diseases of the Esophagus: Diagnosis with Esophagography. Radiology 2005; 237(2): 414-427. 10. Nacif MS, Jauregui GF, Rocha VB, Barcia A, Boechat AP, Mendonça Neto A, Silva Neto S, Osteme EMC, Mello RAF, Santos AASMD. Análise crítica da seriografia do Page 18 of 19 esôfago, estômago e duodeno em um serviço de radiologia de um hospital geral. Radiol Bras 2004; 37(6): 425-429. Page 19 of 19
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