Plummer-Vinson syndrome in primary Sjögren syndrome: a case-based review.

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.

Síndrome de Plummer-Vinson / Plummer-Vinson syndrome

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)

Síndrome de Plummer-Vinson: a propósito de un caso / Plummer-Vinson syndrome: a case study / Síndrome de Plummer-Vinson: relato de caso

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.

Plummer-vinson syndrome associated with lower esophageal ring (Schatzki's Ring)

We report a case of Plummer-Vinson syndrome (PVS) and lower esophageal ring with a small sliding hiatal hernia. PVS is a rare entity formed by the combination of dysphagia, cervical esophageal web and iron deficiency anemia. It occurs mainly in middle-aged women1,2,3. A lower esophageal ring and a small sliding hiatal hernia were also observed in this case. We documented clinical manifestations of iron deficiency anemia through images and esophageal abnormalities through barium esophagogram. (AU)

Síndrome de Plummer-Vinson: relato de caso / Plummer-Vinson syndrome: case report

A síndrome de Plummer-Vinson é uma condição rara, caracterizada pela presença de anemia ferropriva, disfagia cervical e membranas esofágicas. É mais prevalente em mulheres de 40 a 70 anos, de países escandinavos e anglo-saxônicos, de etiologia incerta, contudo há duas teorias que podem ser relacionadas à síndrome: a teoria da deficiência de ferro e a teoria da disfagia primária. Adeficiência em ferro pode provocar mudanças no trato digestório superior, provocan do anemia hipocrômica e microcítica. Já a disfagia colabora com a mudança dos hábitos alimentares dos pacientes, o que resulta em deficiência de ferro e outras vitaminas. Mesmo rara, a Síndrome de Plummer-Vinson necessita ser lembrada durante a formulação de hipóteses diagnósticas, visto que está relacionada como fator de risco para carcinomas espinocelulares do trato gastrointestinal superior.

The Plummer-Vinson syndrome is a rare condition characterized by the presence of iron deficiency anemia, cervical dysphagia and esophageal membranes. It is more prevalent in women aged 40-70 from Scandinavian and Anglo-Saxon countries, and is of unknown etiology, but there are two theories that can be related to the syndrome: the iron deficiency theory and the theory of primary dysphagia. Iron deficiency can cause changes in the upper digestive tract, causing hypochromic and microcytic anemia. Existing dysphagia combines with the changing eating habits of patients, resulting in deficiency in iron and other vitamins. Although rare, Plummer-Vinson syndrome needs to be remembered during the formulation of diagnostic hypotheses, since it is listed as a risk fator for squamous cell carcinoma of the upper gastrointestinal tract.

PLUMMER VINSON SYNDROME--is it common in males?

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.

[Plummer-Vinson syndrome: a case report]. / Síndrome de Plummer-Vinson: relato de caso.

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.

Síndrome de Plummer-Vinson: relato de caso / Plummer-Vinson syndrome: a case report

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.

[Plummer-Vinson syndrome: report of a case and review of literature]. / Síndrome de Plummer-Vinson: reporte de caso y revisión de la literatura.

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.

Síndrome de Plummer-Vinson: reporte de caso y revisión de literatura / Plummer-Vinson Syndrome: case report and review of literature

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.

Síndrome de plummer vinson: relato de caso / Plummer vinson syndrome: case report

A síndrome de Plummer-Vinson ou Patterson-Kelly é doença rara caracterizada pela tríade disfagia intermitente, ferropenia e membrana esofágica. São poucos os dados na literatura sobre sua prevalência. Embora seja atualmente uma síndrome rara, seu reconhecimento é importante, pois identifica um fator de risco para câncer de trato gastrointestinal alto. Aqui descrevemos um caso desta síndrome com revisão de literatura

A possible link between iron deficiency and gastrointestinal carcinogenesis.

There is definitive evidence that iron overload induces oxidative stress and DNA damage, which can enhance carcinogenic risk. However, other evidence suggests that iron deficiency and anemia also increase oxidative stress and DNA damage, which might increase carcinogenesis risk, especially in the gastrointestinal (GI) tract. The aim of this review is to provide essential background information for the accurate interpretation of future research on iron deficiency and increased GI cancer risk. Based on clinical, epidemiological, and experimental evidence, we discuss how iron deficiency might contribute to increased cancer risk through the impairment of several iron-dependent metabolic functions that are related to genome protection and maintenance (e.g., immune responses against cancer-initiated cells, metabolism of toxic compounds, and redox regulation of DNA biosynthesis and repair). Some epidemiological studies have indicated increased risk of GI tumors among individuals with low iron intake or low somatic iron stores, and in vivo data from rodent cancer models indicates the early progression of GI tumors during iron deficiency. Given the preliminary but consistent evidence relating iron deficiency to cancer risk and the fact that iron deficiency affects about one third of the world's population, further studies are needed to define the extent to which iron deficiency might increase GI cancer risk.

Síndrome de Plummer-Vinson: uma rara associação na talassemia / Plumer-Vinson syndrome: a rare association with thalassemia

A síndrome de Plummer-Vinson é caracterizada por disfagia cervical, deficiência de ferro e presença de membrana esofágica. Neste estudo, relatam-se dois casos dessa síndrome em irmãos adolescentes. Eles não obtiveram aumento dos níveis hematimétricos após reposição com ferro oral, o que, associado à eletroforese de hemoglobinas, sustentou o diagnóstico de talassemia concomitante. Devido ao quadro dos filhos, os pais foram também submetidos à eletroforese de hemoglobinas cujo diagnóstico do pai foi talassemia alfa/beta menor e da mãe, talassemia alfa menor. Os irmãos tiveram disfagia refratária e necessitaram de dilatação endoscópica. Ambos necessitaram de terapia com ferro venoso com melhora dos níveis hematimétricos.

Plummer-Vinson syndrome is characterized by cervical dysphagia, iron deficiency and the presence of esophageal membranes. We report two cases of this syndrome present in adolescent brothers with associated thalassemia. After oral iron therapy, their hematimetric levels showed no increase, which associated with the results of hemoglobin electrophoresis, sustained the diagnosis of thalassemia. Due to the condition of the children, the parents were submitted to hemoglobin electrophoresis examinations; the father was diagnosed as having minor alpha/beta thalassemia and the mother as minor beta thalassemia. Both patients suffered from refractory dysphagia and required endoscopic dilatation. They both underwent venous iron therapy, which improved the hematimetric levels.

Síndrome de Plummer-Vinson: quatro diferentes apresentações clínicas / The Plummer-Vinson´s Syndrome

Introdução: A síndrome de Plummer - Vinson, também conhecida como Síndrome de Patterson Kelly, écaracterizada pela presença de membranas esofágicas apresentando-se, como descrito nos quatro casos, deformas variadas, mas tendo como principal característica a disfagia conseqüente da presença de anéis esofágicos mais comumente no esôfago superior. Objetivos: Relatar quatro casos da Síndrome de Plummer-Vinson com apresentações clínicas diversasdiagnosticados no Serviço de gastroenterologia do Hospital São José de Criciúma-SC e discutir os principaistópicos da abordagem diagnóstica e terapêutica dessa enfermidade. Métodos: Os casos foram relatados com base nos prontuários médicos dos pacientes obtidos nos arquivos do Hospital São José de Criciúma-SC com diagnóstico de Síndrome de Plummer-Vinson. Discussão: Os autores discutem os principais pontos a respeito do quadro clínico, diagnóstico e tratamentodos pacientes com o diagnóstico de Síndrome de Plummer-Vinson.

Introduction: The Plummer-Vinson´s Syndrome, also known as Patterson Kelly´s Syndrome, is caracterized by esophageal membranes presence, asdescribed in the four cases in varied ways, but having as main characteristic a dysphagia coming from esophageal rings presence more common in the upper part of the esophagus. Objectives: To present four Plummer-Vinson´sSyndrome cases with several clinical presentations diagnosed at the Gastroenterology Department at SãoJosé Hospital in Criciúma-SC, and discuss the man topics of the therapeutic and diagnostic approach of such illness.Methods: The cases were presented based on the patientsæ medical record had in the files of São José Hospital, Criciúma-SC, with Plummer-Vinson´ssyndrome diagnosis. Discussion: The researchers discuss about the mainpoints concercining the clinical finding diagnosis and the patientesæ treatment with the Plummer-Vinsonæs syndromediagnosis.

Disfagia sideropênica (síndrome de plummer – vinson ou patterson – kelly): relato de caso e revisão da literatura / Sideropenic dysphagia (Plummer-Vinson or Patterson-Kelly Syndrome): a case report and literature review

A presença de membrana esofágica adquirida, na área pós-cricóide, associada ou não com anemia ferropriva é denominada síndrome de Plummer-Vinson ou de Patterson-Kelly. A disfagia associada a SPV é tipicamente intermitente e limitada aos alimentos sólidos. Os enfermos podem apresentar queixa de engasgo ou até episódios aspirativos. A patogênese da síndrome envolve a deficiência nutricional com dieta deficiente em frutas frescas, vegetais e carnes. A presença de membranas esofágicas é associada ao risco elevado de câncer esofágico ou faríngeo, com necessidade de endoscopia digestiva alta periódica para a detecção de alterações pré-neoplásicas na faringe ou no esôfago. Os autores descrevem doente portadora de síndrome de Plummer-Vinson com disfagia para alimentos sólidos, anemia ferropriva, carência nutricional crônica e membranas na luz do esôfago cervical. O tratamento habitual é a reposição oral ou parenteral de ferro associada à dilatação esofágica e/ou rompimento da membrana do esôfago por via endoscópica e à correção das deficiências nutricionais. Essa terapêutica foi adotada com sucesso na enferma desse relato que, após seis meses de seguimento, permanece assintomática, alimentando-se adequadamente, sem perda ponderal ou anemia.

Síndrome de Plummer-Vinson / Plummer-Vinson syndrome

Introdução: A deficiência de ferro consiste na insuficiência nutricional mais freqüente do mundo, sendo a anemia a forma mais grave dessa deficiência. Objetivo: Discutir o diagnóstico diferencial de anemia por deficiência de ferro refratária à ferroterapia oral. Método: Descrição de caso clínico. Resultado: Paciente de 7 anos de idade com anemia por deficiência de ferro refratária à ferroterapia oral que foi encaminhada ao gastroenterologista. Os exames laboratoriais para avaliar a função digestivo-absortiva resultaram normais. A endoscopia digestiva alta revelou estreitamento anelar a cerca de 2 cm do esfíncter esofágico superior com cerca de 0,7 cm de diâmetro impedindo a progressão do fibroscópio. O exame contrastado de esôfago, estômago e duodeno mostrou estreitamento anelar em terço superior de esôfago, sendo feita a hipótese diagnóstica de síndrome de Plummer-Vinson. Foi realizada a dilatação esofágica e a paciente foi submetida à ferroterapia oral. Conclusão: As causas da deficiência de ferro resistente à ferroterapia são diversas, sendo a síndrome de Plummer-Vinson uma dessas causas.

Síndrome de Plummer-Vinson: relato de dois casos / Plummer-Vinson syndrome: report of two cases

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

Síndrome de plummer: vinson en Pediatría / Plummer vinson: syndrome in children

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