ABSTRACT
This study aimed to describe a patient with Sjögren syndrome who developed Plummer-Vinson syndrome, and to review the literature and describe shared aspects of this rare association. A systematic screening of articles was conducted in PubMed/MEDLINE, LILACS, SciELO, Scopus, Web of Science, and Cochrane, dating 1940 to 2020. All the articles included the association between Sjögren syndrome and Plummer-Vinson syndrome. No language restriction was applied. The following terms were used: "Sjögren syndrome" or "sicca syndrome" and "Plummer-Vinson syndrome" or "Paterson-Kelly syndrome." We performed our analysis by adding our present case, with a total of 4 cases. Three out of four were female (75%), age varied from 56 to 58 years old. In 2 cases, Sjögren syndrome preceded Plummer-Vinson syndrome diagnosis, and in 1 report, Plummer-Vinson syndrome appeared before Sjögren syndrome. Disease duration varied from 7 to 20 years. In two cases, autoantibodies were available, and antinuclear antibodies and anti-Ro/SS-A were positive in both, and anti-La/SS-B in one of them was associated with anti-dsDNA; however, no data regarding lupus was available in the article. Treatment involved iron supplementation in 3/3. Two out of three received parenteral iron supplementation, and in these two cases, mechanical esophageal dilatation was needless. In the other case, an additional endoscopic esophageal dilatation was necessary to receive the oral iron supplement. All 3 cases had a good outcome. This case illustrates a patient with Sjögren syndrome who developed the rare Plummer-Vinson syndrome. In Sjögren syndrome, the presence of iron-deficiency anemia, dysphagia, and weight loss should alert the physician to search for associated Plummer-Vinson syndrome.
Subject(s)
Anemia, Iron-Deficiency , Deglutition Disorders , Plummer-Vinson Syndrome , Sjogren's Syndrome , Anemia, Iron-Deficiency/complications , Deglutition Disorders/complications , Deglutition Disorders/diagnosis , Female , Humans , Iron , Male , Middle Aged , Plummer-Vinson Syndrome/complications , Plummer-Vinson Syndrome/diagnosis , Plummer-Vinson Syndrome/therapy , Sjogren's Syndrome/complications , Sjogren's Syndrome/diagnosisABSTRACT
Introducción: El síndrome de Plummer-Vinson es una entidad rara caracterizada por la tríada anemia ferropénica, disfagia y membrana esofágica. Descrito en la segunda década del siglo pasado, en la actualidad la mayoría de los datos que se obtienen provienen de presentaciones de casos o pequeñas series de estudios prospectivos. Objetivo: Hacer la revisión de la bibliografía disponible a propósito de un caso portador de síndrome de Plummer-Vinson. Caso clínico: Paciente femenina de 35 años de edad con anemia desde la adolescencia remitida por presentar disfagia de 8 años de evolución. Se realizaron complementarios de laboratorio donde se constata anemia ferropénica y estudio contrastado del tracto digestivo superior que reveló imagen sugestiva de membrana en esófago cervical. Conclusiones: A pesar de su baja frecuencia en la actualidad debemos mantenernos alertas ante la aparición de síntomas sugestivos del síndrome de Plummer-Vinson fundamentalmente en mujeres con cuadros de anemia(AU)
Introduction: Plummer-Vinson syndrome is a rare entity characterized by the triad of iron deficiency anemia, dysphagia and esophageal membrane. Described in the second decade of the last century, today most of the data obtained comes from case presentations or small series of prospective studies. Objective: To review the available bibliography regarding a case with Plummer-Vinson syndrome. Clinical case: 35-year-old female patient with anemia since adolescence, referred to surgery clinic for presenting dysphagia of 8 years of evolution. Additional laboratory tests were carried out where iron deficiency anemia was found. A contrasted study of the upper digestive tract revealed a suggestive image of a membrane in the cervical esophagus. Conclusions: Despite its low frequency we must be alert to the appearance of symptoms suggestive of Plummer-Vinson syndrome, mainly in women with anemia. Its association with esophageal cancer indicates this(AU)
Subject(s)
Humans , Female , Adult , Deglutition Disorders/etiology , Plummer-Vinson Syndrome/diagnosis , Anemia, Iron-Deficiency/etiology , Ferrous Compounds/therapeutic use , Prospective Studies , Folic Acid/therapeutic useABSTRACT
Presentamos el caso de una paciente de sexo femenino de 40 años que consultó por astenia, adinamia, mareos y disnea progresiva de diez meses de evolución agregando en los últimos cuatro meses disfagia intermitente para sólidos. La radiografía esofágica con bario evidenció la presencia de una estenosis subcricoidea con buen pasaje distal y la videogastroscopía demostró inmediatamente por debajo del cricofaríngeo una membrana fibrosa estenosante. Se realizó tratamiento con hierro vía oral y reiteradas sesiones de dilatación con balón que lograron la resolución de los síntomas. El síndrome de Plummer-Vinson es una entidad poco frecuente caracterizada por la tríada anemia ferropénica, disfagia y membrana esofágica alta.
The study presents the case of a 40 year old female patient who consulted for asthenia, adynamia, dizziness and progressive dyspnea with 10 months of evolution, evidencing intermittent solid dysphagia in the last 4 months. Barium X-rays of the esophagus evidenced subcricoid stenosis with good distal passage and the gastroscopy immediately showed a stenosing fibrous membrane under the cricopharyngeal. Iron oral treatment was initiated and several sessions with a balloon catheter which solved the symptoms. Plummer-inson syndrome is a rare entity characterized by iron deficiency anemia, dysphagia and a high esophageal membrane.
Apresentamos o caso de uma paciente de sexo feminino de 40 anos que consultou por astenia, adinamia, tontura e dispneia progressiva com 10 meses de evolução e disfagia intermitente para sólidos nos últimos 4 meses. A radiografia esofágica com bário mostrou a presença de estenose abaixo da cartilagem cricoide com boa passagem distal e a endoscopia digestiva alta evidenciou membrana fibrosa estenosante logo abaixo do cricofaríngeo. Realizou-se tratamento com ferro por via oral e várias sessões de dilatação com balão com eliminação dos sintomas. A síndrome de Plummer-Vinson é uma doença rara caracterizada pela tríade ferropenia, disfagia e membrana esofágica alta.
Subject(s)
Humans , Plummer-Vinson Syndrome/diagnosis , Plummer-Vinson Syndrome/therapyABSTRACT
BACKGROUND: Plummer-Vinson syndrome is characterized by a triad of dysphagia, iron deficiency anemia and esophageal web. Most of the patients are middle aged women in the fourth to seventh decade of life. OBJECTIVE: Very few cases of Plummer-Vinson syndrome affecting males have been reported. Here we report a series of male patients found to be suffering from Plummer-Vinson syndrome. METHODS: Five males presented to us with dysphagia and fatigue of various durations, from April to August, 2012. RESULTS: These patients were found to have iron deficiency anemia and esophageal web on subsequent investigations and were treated successfully with oral iron therapy. CONCLUSION: Plummer-Vinson syndrome is common in both males and females and can be successfully treated with oral iron therapy. The patients have an increased risk of developing gastrointestinal malignancies and should be thoroughly evaluated for the same.
Subject(s)
Plummer-Vinson Syndrome/diagnosis , Adult , Anemia, Iron-Deficiency/complications , Deglutition Disorders/physiopathology , Esophagoscopy , Esophagus/physiopathology , Humans , Iron/therapeutic use , Male , Men's Health , Middle Aged , Plummer-Vinson Syndrome/drug therapy , Plummer-Vinson Syndrome/etiology , Rare DiseasesABSTRACT
We present a case of Plummer-Vinson syndrome, which is a rare condition nowadays. The diagnosis was made after years of the disease, many doctors having attended the patient. The treatment consisted of oral supplementation of iron and endoscopic dilatations. The patient is asymptomatic.
Subject(s)
Plummer-Vinson Syndrome , Adult , Female , Humans , Plummer-Vinson Syndrome/diagnosisABSTRACT
We present a case of Plummer-Vinson syndrome, which is a rare condition nowadays. The diagnosis was made after years of the disease, many doctors having attended the patient. The treatment consisted of oral supplementation of iron and endoscopic dilatations. The patient is asymptomatic.
Subject(s)
Adult , Female , Humans , Plummer-Vinson Syndrome , Plummer-Vinson Syndrome/diagnosisABSTRACT
A 39-year-old woman was admitted to our hospital with an eight-month history of dyspnea on exertion, weakness and increasing fatigue. She reported repeated episodes of menometrorrhagia and underwent a myomectomy. She is not a vegetarian. Her menstrual bleeding: 3-5 days per month. Two months ago, she complained of burning sensation of the tongue upon swallowing food and noted brittle nails. She tolerated soft foods. On physical examination, she was pale; her nails were very thin, fragile and somewhat concave. Her oral examination showed angular stomatitis, depapillated tongue and glossitis. The clinical diagnosis was anemia and dysphagia. Laboratory tests were: Hb: 7.0g/dL, MCV: 57.42fL, MCH: 15.82 pg; leukocytes: 4,980; reticulocytes: 2.18%, reticulocyte index: 0.1%, serum iron: 21ug/dl, total iron binding capacity (TIBC): 286, transferrin saturation: 7% and serum ferritin: 27ng/ml. The peripheral blood smear showed anisocytosis and hypochromic microcytic cells. Thevenon test was negative. Abdominal ultrasound: uterine myoma. A barium swallow X-ray showed a 2-mm linear filling defect between the 4th and 5th cervical vertebrae in the anteroposterior and lateral view; it protruded from the anterior wall and reduced esophageal lumen by 60%. In the endoscopy, we found a fibrous web in the cricopharyngeal area. Serial dilatations were performed over a guidewire using Savary-Gilliard dilators with diameter up to 14 mm, improving dysphagia. She was treated with transfusional therapy and parenteral iron. She was discharged with ferrous sulfate and folic acid. The Plummer-Vinson syndrome, Paterson-Brown-Kelly or sideropenic dysphagia is characterized by dysphagia, irondeficiency anemia and upper esophageal web. The syndrome is described as very rare.
Subject(s)
Plummer-Vinson Syndrome/diagnosis , Adult , Female , HumansABSTRACT
Presentamos a una mujer de 39 años que acude a nuestro hospital por disnea de esfuerzo, adinamia y cansancio progresivo desde hace 8 meses. Antecedente quirúrgico de miomectomia por menometrorragias frecuentes. Niega ser vegetariana. Régimen catamenial: 3-5 días cada mes. Hace 2 meses se añade ôardorõ en lengua al comer. Nota uñas quebradizas. Toleraba alimentos blandos. Al examen físico había moderada palidez; las uñas eran muy delgadas, frágiles y algo cóncavas. En la boca se encontró estomatitis angular, lengua depapilada y glositis. El diagnóstico clínico era síndrome anémico y disfagia. Exámenes auxiliares: Hb: 7.0g/dL; VCM: 57.42fL; HCM: 15.82pg; leucocitos: 4,980; reticulocitos: 2.18%, índice reticulocitario: 0.1%, hierro sérico: 21ug/dl, transferrina (TIBC): 286, saturación de transferrina: 7%, ferritina sérica: 27ng/ml. La lámina periférica demostró anisocitosis, hipocromía y microcitosis. Thevenon en heces negativo. Ecografía abdominal: mioma uterino. La radiografía esofágica con bario demostró una imagen lineal por defecto de relleno de 2 mm a altura entre vértebras C4 y C5 en vista anteroposterior y lateral; se extendía a cara anterior y reducía la luz esofágica en 60%. Durante la endoscopía, evidenciamos una membrana fibrosa estenosante en la región cricofaríngea. Multiples dilataciones fueron realizadas progresivamente mediante una guía con bujías dilatadoras Savary-Gilliard hasta 14 mm, mejorando la disfagia. Recibió terapia transfusional y hierro parenteral. Salió de alta con sulfato ferroso y ácido fólico. El síndrome de Plummer-Vinson, Paterson-Brown-Kelly o disfagia sideropénica es definido por disfagia, anemia ferropénica y membrana esofágica alta. El síndrome es descrito como muy raro.
A 39-year-old woman was admitted to our hospital with an eight-month history of dyspnea on exertion, weakness and increasing fatigue. She reported repeated episodes of menome trorrhsgis snd underwent a myomectomy. She is not a vegetarian. Her menstrual bleeding: 3-5 days per month. Two months ago, she complained of burning sensation of the tonge upon swallowing food and noted brittle nails. She tolerated soft foods. On physical examination, she was pale; her nails were very thin, fragile and somewhat concave. Her oral examination showed angular stomatitis, depapillated togue and glossitis. The clinical diagnosis was anemia and dysphagia. Laboratory tests were: Hb: 7.0g/dL, MCV: 57.42fL, MCH: 15.82 pg; leukocytes:4,980; reticulocytes:2.18%, reticulocyte index:0.1%, serum iron:21ug/dl, total iron binding capacity (TIBC):286, transferrin saturation: 7% and serum ferritin: 27ng/ml. The peripheral blood smear showed anisocytosis and hypochromic microcytic cells. Thevenon test was negative. Abdominal ultrasound: uterine myoma. A barium swallow X-ray showed a 2-mm linear filling defect between the 4th and 5th cervical vertebrae in the anteroposterior and lateral view; it protruded from the anterior wall and reduced esophageal lumen by 60%. In the endoscopy, we found a fibrous web in the cricopharyngeal area. Serial dilatations were performed over a guidewire using Savary-Gilliard dilators with diameter up to 14 mm, improving dysphagia. She was treated with transfusional therapy and parenteral iron. She was discharged with ferrous sulfate and folic acid. The Plummer-Vinson syndrome, Paterson-Brown-Kelly or sideropenic dysphagia is characterized by dysphagia, iron-deficiency anemia and upper esophaegal web. The syndrome is described as very rare.
Subject(s)
Humans , Adult , Female , Anemia, Iron-Deficiency , Esophageal Spasm, Diffuse , Plummer-Vinson Syndrome/diagnosis , Deglutition DisordersABSTRACT
As membranas esofágicas, quando localizadas no esôfago cervical e associadas a quadro de anemia ferropriva, alterações na mucosa bucal, faríngea e gástrica, constituem a síndrome de Plummer-Vinson. O objetivo deste trabalho é apresentar os aspectos clínicos de dois pacientes do sexo feminino, com 51 e 66 anos de idade, com disfagia havia seis e sete anos, anemia, glossite e coiloníquia. O exame físico em ambos mostrou desnutrição e anemia. Estudos radiológicos e endoscópicos demonstraram estreitamento no esôfago proximal. O estudo manométrico: aperistalse com contrações de baixa amplitude em uma paciente e exame normal na outra. Dilatações foram realizadas nas pacientes com sondas de Savary-Guillard, sendo que uma necessitou de nova dilatação com oliva de Eder-Puestow. Esses resultados dão suporte ao conceito de que a dilatação endoscópica é a melhor opção para o tratamento da síndrome de Plummer-Vinson
Subject(s)
Humans , Female , Middle Aged , Deglutition Disorders , Plummer-Vinson Syndrome/diagnosis , Endoscopy, Digestive System , Plummer-Vinson Syndrome/therapyABSTRACT
El síndrome de Plummer-Vinson se caracteriza por la presencia de anemia ferropénica,disfagia y una menbran esofágica alta.Esta entidad clínica es poco frecuente en la población pediátrica,por lo cual presentamos a los cuatro pacientes en quienes se estableció este diagnóstico entre los años 1995 y 2000,quienes consultaron por disfagia y trastornos tróficos en piel y en mucosas del tracto digestivo
Subject(s)
Humans , Infant , Child, Preschool , Anemia, Iron-Deficiency , Plummer-Vinson Syndrome/diagnosis , Plummer-Vinson Syndrome/therapy , Deglutition Disorders/diagnosis , Deglutition Disorders/therapy , PediatricsABSTRACT
El síndrome de Plummer-Vinson se caracteriza por la presencia de anemia ferropénica,disfagia y una menbran esofágica alta.Esta entidad clínica es poco frecuente en la población pediátrica,por lo cual presentamos a los cuatro pacientes en quienes se estableció este diagnóstico entre los años 1995 y 2000,quienes consultaron por disfagia y trastornos tróficos en piel y en mucosas del tracto digestivo
Subject(s)
Humans , Infant , Child, Preschool , Plummer-Vinson Syndrome/diagnosis , Plummer-Vinson Syndrome/therapy , Deglutition Disorders/diagnosis , Deglutition Disorders/therapy , Anemia, Iron-Deficiency , PediatricsABSTRACT
A síndrome de Plummer-Vinson é uma entidade clínica caracterizada por disfagia, membranas esofágicas e anemia ferropriva. Esta síndrome está associada com aumento no risco de câncer no trato alimentar superior. Os autores relatam um caso, revisam a literatura e apresentam uma discussão sobre a etiopatia, diagnóstico, tratamento e prognóstico dessa afecção
Subject(s)
Humans , Plummer-Vinson Syndrome/diagnosis , Plummer-Vinson Syndrome/physiopathology , Plummer-Vinson Syndrome/therapy , Deglutition Disorders/physiopathologyABSTRACT
El síndrome Plummer-Vinson es un cuadro infrecuente caracterizado por disfagia debida a un diafragma membranoso de la hipofaringe o del esófago cervical y manifestaciones sideropénicas cutáneas, mucosas (orales, esofágicas y gástricas) y hematológicas. El síndrome se presenta casi exclusivamente en mujeres (85 a 90 porciento)
Subject(s)
Humans , Plummer-Vinson Syndrome/diagnosis , Plummer-Vinson Syndrome/therapyABSTRACT
Esophageal webs are an uncommon group among esophageal diseases. We report our findings in ten patients with membranes in the upper esophagus; seven of them were women. The median age was 48.5 years (range 24 to 73 years). Dysphagia was the main symptom in all patients and anemia was found in six cases. Radiologic and endoscopic studies provided the diagnosis. All patients underwent endoscopic dilatation and/or debridement and good results with this management were achieved. A review of literature of this condition and a discussion about the diagnosis, treatment and etiopathogenesis are presented.
Subject(s)
Esophageal Diseases/complications , Plummer-Vinson Syndrome/complications , Adult , Aged , Anemia, Hypochromic/complications , Endoscopy, Digestive System , Female , Humans , Male , Middle Aged , Plummer-Vinson Syndrome/diagnosis , Plummer-Vinson Syndrome/therapyABSTRACT
El síndrome de Plummer vinson es una entidad rara, más frecuente en los países escandinavos, pero es necessario pensar en él cuando se presenta la asociación disfagia y anemia microcítica, hipocrómica, ferropénica ya que la endoscopia bajo visión directa permite el diagnóstico y la terpéutica al destruir la membrana durante el mismo procedimiento